Uncommon benign and extremely vascular tumour Up to 05 of head and neck tumours Occurring almost exclusively in males Average age of onset - 15 years old
JNA Facts and Statistics Intracranial Extension between 10-20 Recurrence Rates as high as 50
Origin - Posterolateral nasal wall at the sphenopalatine foramen
Through this foramen tumours from fossa of rosenmuller invades intracranial structures III IV V VI cranial nerves are located near to this foramen which are commonly involved when tumours invade intracranial structures via the foramen lacerum
PATHOGENESIS
Pathology
Macroscopic ndash Well-defined spongy lobulated tumours with nodules covered by
nasopharyngeal mucosa (squamous epithelium) Nodularity increases with age Color varies from pink (part seen in nasopharynx) to white or grey
(extrapharyngeal areas) On section tumour is reticulated whorled or spongy in appearance
lacking a true capsule Edges are sharply demarcated and easily distinguishable from the
surrounding tissues
Pathology conthellipMicroscopic ndash Consists of proliferating irregular vascular chan nels within a fibrous stroma Vascular component is more in young tumours and as age increases
collagen content increases Fibrous tissue increases towards periphery and vascular element tends to
be more central
Pathology conthellip
Tumour blood vessels typically lack smooth muscle and elastic fibres this is the reason for sustained bleeding
Cellular infiltration with plasma cells lymphocytes polymorphs eosinophils can be present
Stromal compartment consists spindle or stellate plump cells that give rise to varying amounts of collagen which makes some tumours very hard or firm
Mucous glands can be seen in superficial parts of the tumour underneath the epithelial covering
Pathogenesis
This tumour is almost exclusively found in adolescent boys so there is much speculation and indirect evidence that sex-hormone receptors play some part in its development
Theories associated with its aetiopathogenesis - Ringertz theory (1938) ndash JNA always arose from the periosteum of the skull
base Som amp Neffson (1940) ndash inequalities in the growth of bones forming skull base
resulted in hypertrophy of the underlying periosteum in response to hormonal influence
Bensch amp Ewing (1941) ndash tumour probably arose from embryonic fibro cartilage between basi-occiput and basi-sphenoid
Brunner (1942) ndash suggested origin from conjoined pharyngobasilar and buccopharyngeal fascia
Pathogenesis conthellip
Marten et al (1948) ndash proposed a hormonal theory suggesting that these tumours resulted from deficiency of androgens or overactivity of estrogens and that the hormonal stimulation is responsible for angiomatous components seen in JNA
Sternberg (1954) ndash proposed that JNA could be a type of hemangioma like a cutaneous hemangioma seen in children which regresses with age
Osborn (1959) ndash it could be due to either a hamartoma or residual fetal erectile tissue which were subjected to hormonal influence
Girgis amp fahmy (1973) ndash observed cell nests of undifferentiated epitheloid cells or ldquozellballenrdquo at the growing edge of angiofibromas and so considered it as a paraganglioma
Pathogenesis conthellip
The most accepted theory is that JNAs originate from sex steroid ndash stimulated hamartomatous tissue located in the turbinate cartilage The proposed hormonal influence explains why some JNAs involute after puberty
Recent immunocytochemical techniques shown that androgen receptors are present in at least 75 percent of tumours these
receptors are present in both the vascular and stromal elements progesterone receptors were found in some In contrast oestrogen receptors have not been demonstrated
Pathogenesis conthellip
Other factors that play a role in the growth of this tumour are The angiogenic growth factor (vascular endothelial growth factor (VEGF))
has been found localized on both endothelial and stromal cells indicating both cell types play a role in tumour development
Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67
But both of them do not have any relation to its degree of aggressiveness Overexpression of insulin-like growth factor II (IGFII)
found in a large number of juvenile angio fibromas IGFII gene is situated on the chromosome 11q associated with a tendency to recurrence and poorer prognosis
Pathogenesis conthellip
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Uncommon benign and extremely vascular tumour Up to 05 of head and neck tumours Occurring almost exclusively in males Average age of onset - 15 years old
JNA Facts and Statistics Intracranial Extension between 10-20 Recurrence Rates as high as 50
Origin - Posterolateral nasal wall at the sphenopalatine foramen
Through this foramen tumours from fossa of rosenmuller invades intracranial structures III IV V VI cranial nerves are located near to this foramen which are commonly involved when tumours invade intracranial structures via the foramen lacerum
PATHOGENESIS
Pathology
Macroscopic ndash Well-defined spongy lobulated tumours with nodules covered by
nasopharyngeal mucosa (squamous epithelium) Nodularity increases with age Color varies from pink (part seen in nasopharynx) to white or grey
(extrapharyngeal areas) On section tumour is reticulated whorled or spongy in appearance
lacking a true capsule Edges are sharply demarcated and easily distinguishable from the
surrounding tissues
Pathology conthellipMicroscopic ndash Consists of proliferating irregular vascular chan nels within a fibrous stroma Vascular component is more in young tumours and as age increases
collagen content increases Fibrous tissue increases towards periphery and vascular element tends to
be more central
Pathology conthellip
Tumour blood vessels typically lack smooth muscle and elastic fibres this is the reason for sustained bleeding
Cellular infiltration with plasma cells lymphocytes polymorphs eosinophils can be present
Stromal compartment consists spindle or stellate plump cells that give rise to varying amounts of collagen which makes some tumours very hard or firm
Mucous glands can be seen in superficial parts of the tumour underneath the epithelial covering
Pathogenesis
This tumour is almost exclusively found in adolescent boys so there is much speculation and indirect evidence that sex-hormone receptors play some part in its development
Theories associated with its aetiopathogenesis - Ringertz theory (1938) ndash JNA always arose from the periosteum of the skull
base Som amp Neffson (1940) ndash inequalities in the growth of bones forming skull base
resulted in hypertrophy of the underlying periosteum in response to hormonal influence
Bensch amp Ewing (1941) ndash tumour probably arose from embryonic fibro cartilage between basi-occiput and basi-sphenoid
Brunner (1942) ndash suggested origin from conjoined pharyngobasilar and buccopharyngeal fascia
Pathogenesis conthellip
Marten et al (1948) ndash proposed a hormonal theory suggesting that these tumours resulted from deficiency of androgens or overactivity of estrogens and that the hormonal stimulation is responsible for angiomatous components seen in JNA
Sternberg (1954) ndash proposed that JNA could be a type of hemangioma like a cutaneous hemangioma seen in children which regresses with age
Osborn (1959) ndash it could be due to either a hamartoma or residual fetal erectile tissue which were subjected to hormonal influence
Girgis amp fahmy (1973) ndash observed cell nests of undifferentiated epitheloid cells or ldquozellballenrdquo at the growing edge of angiofibromas and so considered it as a paraganglioma
Pathogenesis conthellip
The most accepted theory is that JNAs originate from sex steroid ndash stimulated hamartomatous tissue located in the turbinate cartilage The proposed hormonal influence explains why some JNAs involute after puberty
Recent immunocytochemical techniques shown that androgen receptors are present in at least 75 percent of tumours these
receptors are present in both the vascular and stromal elements progesterone receptors were found in some In contrast oestrogen receptors have not been demonstrated
Pathogenesis conthellip
Other factors that play a role in the growth of this tumour are The angiogenic growth factor (vascular endothelial growth factor (VEGF))
has been found localized on both endothelial and stromal cells indicating both cell types play a role in tumour development
Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67
But both of them do not have any relation to its degree of aggressiveness Overexpression of insulin-like growth factor II (IGFII)
found in a large number of juvenile angio fibromas IGFII gene is situated on the chromosome 11q associated with a tendency to recurrence and poorer prognosis
Pathogenesis conthellip
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Through this foramen tumours from fossa of rosenmuller invades intracranial structures III IV V VI cranial nerves are located near to this foramen which are commonly involved when tumours invade intracranial structures via the foramen lacerum
PATHOGENESIS
Pathology
Macroscopic ndash Well-defined spongy lobulated tumours with nodules covered by
nasopharyngeal mucosa (squamous epithelium) Nodularity increases with age Color varies from pink (part seen in nasopharynx) to white or grey
(extrapharyngeal areas) On section tumour is reticulated whorled or spongy in appearance
lacking a true capsule Edges are sharply demarcated and easily distinguishable from the
surrounding tissues
Pathology conthellipMicroscopic ndash Consists of proliferating irregular vascular chan nels within a fibrous stroma Vascular component is more in young tumours and as age increases
collagen content increases Fibrous tissue increases towards periphery and vascular element tends to
be more central
Pathology conthellip
Tumour blood vessels typically lack smooth muscle and elastic fibres this is the reason for sustained bleeding
Cellular infiltration with plasma cells lymphocytes polymorphs eosinophils can be present
Stromal compartment consists spindle or stellate plump cells that give rise to varying amounts of collagen which makes some tumours very hard or firm
Mucous glands can be seen in superficial parts of the tumour underneath the epithelial covering
Pathogenesis
This tumour is almost exclusively found in adolescent boys so there is much speculation and indirect evidence that sex-hormone receptors play some part in its development
Theories associated with its aetiopathogenesis - Ringertz theory (1938) ndash JNA always arose from the periosteum of the skull
base Som amp Neffson (1940) ndash inequalities in the growth of bones forming skull base
resulted in hypertrophy of the underlying periosteum in response to hormonal influence
Bensch amp Ewing (1941) ndash tumour probably arose from embryonic fibro cartilage between basi-occiput and basi-sphenoid
Brunner (1942) ndash suggested origin from conjoined pharyngobasilar and buccopharyngeal fascia
Pathogenesis conthellip
Marten et al (1948) ndash proposed a hormonal theory suggesting that these tumours resulted from deficiency of androgens or overactivity of estrogens and that the hormonal stimulation is responsible for angiomatous components seen in JNA
Sternberg (1954) ndash proposed that JNA could be a type of hemangioma like a cutaneous hemangioma seen in children which regresses with age
Osborn (1959) ndash it could be due to either a hamartoma or residual fetal erectile tissue which were subjected to hormonal influence
Girgis amp fahmy (1973) ndash observed cell nests of undifferentiated epitheloid cells or ldquozellballenrdquo at the growing edge of angiofibromas and so considered it as a paraganglioma
Pathogenesis conthellip
The most accepted theory is that JNAs originate from sex steroid ndash stimulated hamartomatous tissue located in the turbinate cartilage The proposed hormonal influence explains why some JNAs involute after puberty
Recent immunocytochemical techniques shown that androgen receptors are present in at least 75 percent of tumours these
receptors are present in both the vascular and stromal elements progesterone receptors were found in some In contrast oestrogen receptors have not been demonstrated
Pathogenesis conthellip
Other factors that play a role in the growth of this tumour are The angiogenic growth factor (vascular endothelial growth factor (VEGF))
has been found localized on both endothelial and stromal cells indicating both cell types play a role in tumour development
Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67
But both of them do not have any relation to its degree of aggressiveness Overexpression of insulin-like growth factor II (IGFII)
found in a large number of juvenile angio fibromas IGFII gene is situated on the chromosome 11q associated with a tendency to recurrence and poorer prognosis
Pathogenesis conthellip
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Through this foramen tumours from fossa of rosenmuller invades intracranial structures III IV V VI cranial nerves are located near to this foramen which are commonly involved when tumours invade intracranial structures via the foramen lacerum
PATHOGENESIS
Pathology
Macroscopic ndash Well-defined spongy lobulated tumours with nodules covered by
nasopharyngeal mucosa (squamous epithelium) Nodularity increases with age Color varies from pink (part seen in nasopharynx) to white or grey
(extrapharyngeal areas) On section tumour is reticulated whorled or spongy in appearance
lacking a true capsule Edges are sharply demarcated and easily distinguishable from the
surrounding tissues
Pathology conthellipMicroscopic ndash Consists of proliferating irregular vascular chan nels within a fibrous stroma Vascular component is more in young tumours and as age increases
collagen content increases Fibrous tissue increases towards periphery and vascular element tends to
be more central
Pathology conthellip
Tumour blood vessels typically lack smooth muscle and elastic fibres this is the reason for sustained bleeding
Cellular infiltration with plasma cells lymphocytes polymorphs eosinophils can be present
Stromal compartment consists spindle or stellate plump cells that give rise to varying amounts of collagen which makes some tumours very hard or firm
Mucous glands can be seen in superficial parts of the tumour underneath the epithelial covering
Pathogenesis
This tumour is almost exclusively found in adolescent boys so there is much speculation and indirect evidence that sex-hormone receptors play some part in its development
Theories associated with its aetiopathogenesis - Ringertz theory (1938) ndash JNA always arose from the periosteum of the skull
base Som amp Neffson (1940) ndash inequalities in the growth of bones forming skull base
resulted in hypertrophy of the underlying periosteum in response to hormonal influence
Bensch amp Ewing (1941) ndash tumour probably arose from embryonic fibro cartilage between basi-occiput and basi-sphenoid
Brunner (1942) ndash suggested origin from conjoined pharyngobasilar and buccopharyngeal fascia
Pathogenesis conthellip
Marten et al (1948) ndash proposed a hormonal theory suggesting that these tumours resulted from deficiency of androgens or overactivity of estrogens and that the hormonal stimulation is responsible for angiomatous components seen in JNA
Sternberg (1954) ndash proposed that JNA could be a type of hemangioma like a cutaneous hemangioma seen in children which regresses with age
Osborn (1959) ndash it could be due to either a hamartoma or residual fetal erectile tissue which were subjected to hormonal influence
Girgis amp fahmy (1973) ndash observed cell nests of undifferentiated epitheloid cells or ldquozellballenrdquo at the growing edge of angiofibromas and so considered it as a paraganglioma
Pathogenesis conthellip
The most accepted theory is that JNAs originate from sex steroid ndash stimulated hamartomatous tissue located in the turbinate cartilage The proposed hormonal influence explains why some JNAs involute after puberty
Recent immunocytochemical techniques shown that androgen receptors are present in at least 75 percent of tumours these
receptors are present in both the vascular and stromal elements progesterone receptors were found in some In contrast oestrogen receptors have not been demonstrated
Pathogenesis conthellip
Other factors that play a role in the growth of this tumour are The angiogenic growth factor (vascular endothelial growth factor (VEGF))
has been found localized on both endothelial and stromal cells indicating both cell types play a role in tumour development
Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67
But both of them do not have any relation to its degree of aggressiveness Overexpression of insulin-like growth factor II (IGFII)
found in a large number of juvenile angio fibromas IGFII gene is situated on the chromosome 11q associated with a tendency to recurrence and poorer prognosis
Pathogenesis conthellip
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Through this foramen tumours from fossa of rosenmuller invades intracranial structures III IV V VI cranial nerves are located near to this foramen which are commonly involved when tumours invade intracranial structures via the foramen lacerum
PATHOGENESIS
Pathology
Macroscopic ndash Well-defined spongy lobulated tumours with nodules covered by
nasopharyngeal mucosa (squamous epithelium) Nodularity increases with age Color varies from pink (part seen in nasopharynx) to white or grey
(extrapharyngeal areas) On section tumour is reticulated whorled or spongy in appearance
lacking a true capsule Edges are sharply demarcated and easily distinguishable from the
surrounding tissues
Pathology conthellipMicroscopic ndash Consists of proliferating irregular vascular chan nels within a fibrous stroma Vascular component is more in young tumours and as age increases
collagen content increases Fibrous tissue increases towards periphery and vascular element tends to
be more central
Pathology conthellip
Tumour blood vessels typically lack smooth muscle and elastic fibres this is the reason for sustained bleeding
Cellular infiltration with plasma cells lymphocytes polymorphs eosinophils can be present
Stromal compartment consists spindle or stellate plump cells that give rise to varying amounts of collagen which makes some tumours very hard or firm
Mucous glands can be seen in superficial parts of the tumour underneath the epithelial covering
Pathogenesis
This tumour is almost exclusively found in adolescent boys so there is much speculation and indirect evidence that sex-hormone receptors play some part in its development
Theories associated with its aetiopathogenesis - Ringertz theory (1938) ndash JNA always arose from the periosteum of the skull
base Som amp Neffson (1940) ndash inequalities in the growth of bones forming skull base
resulted in hypertrophy of the underlying periosteum in response to hormonal influence
Bensch amp Ewing (1941) ndash tumour probably arose from embryonic fibro cartilage between basi-occiput and basi-sphenoid
Brunner (1942) ndash suggested origin from conjoined pharyngobasilar and buccopharyngeal fascia
Pathogenesis conthellip
Marten et al (1948) ndash proposed a hormonal theory suggesting that these tumours resulted from deficiency of androgens or overactivity of estrogens and that the hormonal stimulation is responsible for angiomatous components seen in JNA
Sternberg (1954) ndash proposed that JNA could be a type of hemangioma like a cutaneous hemangioma seen in children which regresses with age
Osborn (1959) ndash it could be due to either a hamartoma or residual fetal erectile tissue which were subjected to hormonal influence
Girgis amp fahmy (1973) ndash observed cell nests of undifferentiated epitheloid cells or ldquozellballenrdquo at the growing edge of angiofibromas and so considered it as a paraganglioma
Pathogenesis conthellip
The most accepted theory is that JNAs originate from sex steroid ndash stimulated hamartomatous tissue located in the turbinate cartilage The proposed hormonal influence explains why some JNAs involute after puberty
Recent immunocytochemical techniques shown that androgen receptors are present in at least 75 percent of tumours these
receptors are present in both the vascular and stromal elements progesterone receptors were found in some In contrast oestrogen receptors have not been demonstrated
Pathogenesis conthellip
Other factors that play a role in the growth of this tumour are The angiogenic growth factor (vascular endothelial growth factor (VEGF))
has been found localized on both endothelial and stromal cells indicating both cell types play a role in tumour development
Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67
But both of them do not have any relation to its degree of aggressiveness Overexpression of insulin-like growth factor II (IGFII)
found in a large number of juvenile angio fibromas IGFII gene is situated on the chromosome 11q associated with a tendency to recurrence and poorer prognosis
Pathogenesis conthellip
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Through this foramen tumours from fossa of rosenmuller invades intracranial structures III IV V VI cranial nerves are located near to this foramen which are commonly involved when tumours invade intracranial structures via the foramen lacerum
PATHOGENESIS
Pathology
Macroscopic ndash Well-defined spongy lobulated tumours with nodules covered by
nasopharyngeal mucosa (squamous epithelium) Nodularity increases with age Color varies from pink (part seen in nasopharynx) to white or grey
(extrapharyngeal areas) On section tumour is reticulated whorled or spongy in appearance
lacking a true capsule Edges are sharply demarcated and easily distinguishable from the
surrounding tissues
Pathology conthellipMicroscopic ndash Consists of proliferating irregular vascular chan nels within a fibrous stroma Vascular component is more in young tumours and as age increases
collagen content increases Fibrous tissue increases towards periphery and vascular element tends to
be more central
Pathology conthellip
Tumour blood vessels typically lack smooth muscle and elastic fibres this is the reason for sustained bleeding
Cellular infiltration with plasma cells lymphocytes polymorphs eosinophils can be present
Stromal compartment consists spindle or stellate plump cells that give rise to varying amounts of collagen which makes some tumours very hard or firm
Mucous glands can be seen in superficial parts of the tumour underneath the epithelial covering
Pathogenesis
This tumour is almost exclusively found in adolescent boys so there is much speculation and indirect evidence that sex-hormone receptors play some part in its development
Theories associated with its aetiopathogenesis - Ringertz theory (1938) ndash JNA always arose from the periosteum of the skull
base Som amp Neffson (1940) ndash inequalities in the growth of bones forming skull base
resulted in hypertrophy of the underlying periosteum in response to hormonal influence
Bensch amp Ewing (1941) ndash tumour probably arose from embryonic fibro cartilage between basi-occiput and basi-sphenoid
Brunner (1942) ndash suggested origin from conjoined pharyngobasilar and buccopharyngeal fascia
Pathogenesis conthellip
Marten et al (1948) ndash proposed a hormonal theory suggesting that these tumours resulted from deficiency of androgens or overactivity of estrogens and that the hormonal stimulation is responsible for angiomatous components seen in JNA
Sternberg (1954) ndash proposed that JNA could be a type of hemangioma like a cutaneous hemangioma seen in children which regresses with age
Osborn (1959) ndash it could be due to either a hamartoma or residual fetal erectile tissue which were subjected to hormonal influence
Girgis amp fahmy (1973) ndash observed cell nests of undifferentiated epitheloid cells or ldquozellballenrdquo at the growing edge of angiofibromas and so considered it as a paraganglioma
Pathogenesis conthellip
The most accepted theory is that JNAs originate from sex steroid ndash stimulated hamartomatous tissue located in the turbinate cartilage The proposed hormonal influence explains why some JNAs involute after puberty
Recent immunocytochemical techniques shown that androgen receptors are present in at least 75 percent of tumours these
receptors are present in both the vascular and stromal elements progesterone receptors were found in some In contrast oestrogen receptors have not been demonstrated
Pathogenesis conthellip
Other factors that play a role in the growth of this tumour are The angiogenic growth factor (vascular endothelial growth factor (VEGF))
has been found localized on both endothelial and stromal cells indicating both cell types play a role in tumour development
Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67
But both of them do not have any relation to its degree of aggressiveness Overexpression of insulin-like growth factor II (IGFII)
found in a large number of juvenile angio fibromas IGFII gene is situated on the chromosome 11q associated with a tendency to recurrence and poorer prognosis
Pathogenesis conthellip
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Through this foramen tumours from fossa of rosenmuller invades intracranial structures III IV V VI cranial nerves are located near to this foramen which are commonly involved when tumours invade intracranial structures via the foramen lacerum
PATHOGENESIS
Pathology
Macroscopic ndash Well-defined spongy lobulated tumours with nodules covered by
nasopharyngeal mucosa (squamous epithelium) Nodularity increases with age Color varies from pink (part seen in nasopharynx) to white or grey
(extrapharyngeal areas) On section tumour is reticulated whorled or spongy in appearance
lacking a true capsule Edges are sharply demarcated and easily distinguishable from the
surrounding tissues
Pathology conthellipMicroscopic ndash Consists of proliferating irregular vascular chan nels within a fibrous stroma Vascular component is more in young tumours and as age increases
collagen content increases Fibrous tissue increases towards periphery and vascular element tends to
be more central
Pathology conthellip
Tumour blood vessels typically lack smooth muscle and elastic fibres this is the reason for sustained bleeding
Cellular infiltration with plasma cells lymphocytes polymorphs eosinophils can be present
Stromal compartment consists spindle or stellate plump cells that give rise to varying amounts of collagen which makes some tumours very hard or firm
Mucous glands can be seen in superficial parts of the tumour underneath the epithelial covering
Pathogenesis
This tumour is almost exclusively found in adolescent boys so there is much speculation and indirect evidence that sex-hormone receptors play some part in its development
Theories associated with its aetiopathogenesis - Ringertz theory (1938) ndash JNA always arose from the periosteum of the skull
base Som amp Neffson (1940) ndash inequalities in the growth of bones forming skull base
resulted in hypertrophy of the underlying periosteum in response to hormonal influence
Bensch amp Ewing (1941) ndash tumour probably arose from embryonic fibro cartilage between basi-occiput and basi-sphenoid
Brunner (1942) ndash suggested origin from conjoined pharyngobasilar and buccopharyngeal fascia
Pathogenesis conthellip
Marten et al (1948) ndash proposed a hormonal theory suggesting that these tumours resulted from deficiency of androgens or overactivity of estrogens and that the hormonal stimulation is responsible for angiomatous components seen in JNA
Sternberg (1954) ndash proposed that JNA could be a type of hemangioma like a cutaneous hemangioma seen in children which regresses with age
Osborn (1959) ndash it could be due to either a hamartoma or residual fetal erectile tissue which were subjected to hormonal influence
Girgis amp fahmy (1973) ndash observed cell nests of undifferentiated epitheloid cells or ldquozellballenrdquo at the growing edge of angiofibromas and so considered it as a paraganglioma
Pathogenesis conthellip
The most accepted theory is that JNAs originate from sex steroid ndash stimulated hamartomatous tissue located in the turbinate cartilage The proposed hormonal influence explains why some JNAs involute after puberty
Recent immunocytochemical techniques shown that androgen receptors are present in at least 75 percent of tumours these
receptors are present in both the vascular and stromal elements progesterone receptors were found in some In contrast oestrogen receptors have not been demonstrated
Pathogenesis conthellip
Other factors that play a role in the growth of this tumour are The angiogenic growth factor (vascular endothelial growth factor (VEGF))
has been found localized on both endothelial and stromal cells indicating both cell types play a role in tumour development
Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67
But both of them do not have any relation to its degree of aggressiveness Overexpression of insulin-like growth factor II (IGFII)
found in a large number of juvenile angio fibromas IGFII gene is situated on the chromosome 11q associated with a tendency to recurrence and poorer prognosis
Pathogenesis conthellip
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Through this foramen tumours from fossa of rosenmuller invades intracranial structures III IV V VI cranial nerves are located near to this foramen which are commonly involved when tumours invade intracranial structures via the foramen lacerum
PATHOGENESIS
Pathology
Macroscopic ndash Well-defined spongy lobulated tumours with nodules covered by
nasopharyngeal mucosa (squamous epithelium) Nodularity increases with age Color varies from pink (part seen in nasopharynx) to white or grey
(extrapharyngeal areas) On section tumour is reticulated whorled or spongy in appearance
lacking a true capsule Edges are sharply demarcated and easily distinguishable from the
surrounding tissues
Pathology conthellipMicroscopic ndash Consists of proliferating irregular vascular chan nels within a fibrous stroma Vascular component is more in young tumours and as age increases
collagen content increases Fibrous tissue increases towards periphery and vascular element tends to
be more central
Pathology conthellip
Tumour blood vessels typically lack smooth muscle and elastic fibres this is the reason for sustained bleeding
Cellular infiltration with plasma cells lymphocytes polymorphs eosinophils can be present
Stromal compartment consists spindle or stellate plump cells that give rise to varying amounts of collagen which makes some tumours very hard or firm
Mucous glands can be seen in superficial parts of the tumour underneath the epithelial covering
Pathogenesis
This tumour is almost exclusively found in adolescent boys so there is much speculation and indirect evidence that sex-hormone receptors play some part in its development
Theories associated with its aetiopathogenesis - Ringertz theory (1938) ndash JNA always arose from the periosteum of the skull
base Som amp Neffson (1940) ndash inequalities in the growth of bones forming skull base
resulted in hypertrophy of the underlying periosteum in response to hormonal influence
Bensch amp Ewing (1941) ndash tumour probably arose from embryonic fibro cartilage between basi-occiput and basi-sphenoid
Brunner (1942) ndash suggested origin from conjoined pharyngobasilar and buccopharyngeal fascia
Pathogenesis conthellip
Marten et al (1948) ndash proposed a hormonal theory suggesting that these tumours resulted from deficiency of androgens or overactivity of estrogens and that the hormonal stimulation is responsible for angiomatous components seen in JNA
Sternberg (1954) ndash proposed that JNA could be a type of hemangioma like a cutaneous hemangioma seen in children which regresses with age
Osborn (1959) ndash it could be due to either a hamartoma or residual fetal erectile tissue which were subjected to hormonal influence
Girgis amp fahmy (1973) ndash observed cell nests of undifferentiated epitheloid cells or ldquozellballenrdquo at the growing edge of angiofibromas and so considered it as a paraganglioma
Pathogenesis conthellip
The most accepted theory is that JNAs originate from sex steroid ndash stimulated hamartomatous tissue located in the turbinate cartilage The proposed hormonal influence explains why some JNAs involute after puberty
Recent immunocytochemical techniques shown that androgen receptors are present in at least 75 percent of tumours these
receptors are present in both the vascular and stromal elements progesterone receptors were found in some In contrast oestrogen receptors have not been demonstrated
Pathogenesis conthellip
Other factors that play a role in the growth of this tumour are The angiogenic growth factor (vascular endothelial growth factor (VEGF))
has been found localized on both endothelial and stromal cells indicating both cell types play a role in tumour development
Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67
But both of them do not have any relation to its degree of aggressiveness Overexpression of insulin-like growth factor II (IGFII)
found in a large number of juvenile angio fibromas IGFII gene is situated on the chromosome 11q associated with a tendency to recurrence and poorer prognosis
Pathogenesis conthellip
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Macroscopic ndash Well-defined spongy lobulated tumours with nodules covered by
nasopharyngeal mucosa (squamous epithelium) Nodularity increases with age Color varies from pink (part seen in nasopharynx) to white or grey
(extrapharyngeal areas) On section tumour is reticulated whorled or spongy in appearance
lacking a true capsule Edges are sharply demarcated and easily distinguishable from the
surrounding tissues
Pathology conthellipMicroscopic ndash Consists of proliferating irregular vascular chan nels within a fibrous stroma Vascular component is more in young tumours and as age increases
collagen content increases Fibrous tissue increases towards periphery and vascular element tends to
be more central
Pathology conthellip
Tumour blood vessels typically lack smooth muscle and elastic fibres this is the reason for sustained bleeding
Cellular infiltration with plasma cells lymphocytes polymorphs eosinophils can be present
Stromal compartment consists spindle or stellate plump cells that give rise to varying amounts of collagen which makes some tumours very hard or firm
Mucous glands can be seen in superficial parts of the tumour underneath the epithelial covering
Pathogenesis
This tumour is almost exclusively found in adolescent boys so there is much speculation and indirect evidence that sex-hormone receptors play some part in its development
Theories associated with its aetiopathogenesis - Ringertz theory (1938) ndash JNA always arose from the periosteum of the skull
base Som amp Neffson (1940) ndash inequalities in the growth of bones forming skull base
resulted in hypertrophy of the underlying periosteum in response to hormonal influence
Bensch amp Ewing (1941) ndash tumour probably arose from embryonic fibro cartilage between basi-occiput and basi-sphenoid
Brunner (1942) ndash suggested origin from conjoined pharyngobasilar and buccopharyngeal fascia
Pathogenesis conthellip
Marten et al (1948) ndash proposed a hormonal theory suggesting that these tumours resulted from deficiency of androgens or overactivity of estrogens and that the hormonal stimulation is responsible for angiomatous components seen in JNA
Sternberg (1954) ndash proposed that JNA could be a type of hemangioma like a cutaneous hemangioma seen in children which regresses with age
Osborn (1959) ndash it could be due to either a hamartoma or residual fetal erectile tissue which were subjected to hormonal influence
Girgis amp fahmy (1973) ndash observed cell nests of undifferentiated epitheloid cells or ldquozellballenrdquo at the growing edge of angiofibromas and so considered it as a paraganglioma
Pathogenesis conthellip
The most accepted theory is that JNAs originate from sex steroid ndash stimulated hamartomatous tissue located in the turbinate cartilage The proposed hormonal influence explains why some JNAs involute after puberty
Recent immunocytochemical techniques shown that androgen receptors are present in at least 75 percent of tumours these
receptors are present in both the vascular and stromal elements progesterone receptors were found in some In contrast oestrogen receptors have not been demonstrated
Pathogenesis conthellip
Other factors that play a role in the growth of this tumour are The angiogenic growth factor (vascular endothelial growth factor (VEGF))
has been found localized on both endothelial and stromal cells indicating both cell types play a role in tumour development
Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67
But both of them do not have any relation to its degree of aggressiveness Overexpression of insulin-like growth factor II (IGFII)
found in a large number of juvenile angio fibromas IGFII gene is situated on the chromosome 11q associated with a tendency to recurrence and poorer prognosis
Pathogenesis conthellip
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Macroscopic ndash Well-defined spongy lobulated tumours with nodules covered by
nasopharyngeal mucosa (squamous epithelium) Nodularity increases with age Color varies from pink (part seen in nasopharynx) to white or grey
(extrapharyngeal areas) On section tumour is reticulated whorled or spongy in appearance
lacking a true capsule Edges are sharply demarcated and easily distinguishable from the
surrounding tissues
Pathology conthellipMicroscopic ndash Consists of proliferating irregular vascular chan nels within a fibrous stroma Vascular component is more in young tumours and as age increases
collagen content increases Fibrous tissue increases towards periphery and vascular element tends to
be more central
Pathology conthellip
Tumour blood vessels typically lack smooth muscle and elastic fibres this is the reason for sustained bleeding
Cellular infiltration with plasma cells lymphocytes polymorphs eosinophils can be present
Stromal compartment consists spindle or stellate plump cells that give rise to varying amounts of collagen which makes some tumours very hard or firm
Mucous glands can be seen in superficial parts of the tumour underneath the epithelial covering
Pathogenesis
This tumour is almost exclusively found in adolescent boys so there is much speculation and indirect evidence that sex-hormone receptors play some part in its development
Theories associated with its aetiopathogenesis - Ringertz theory (1938) ndash JNA always arose from the periosteum of the skull
base Som amp Neffson (1940) ndash inequalities in the growth of bones forming skull base
resulted in hypertrophy of the underlying periosteum in response to hormonal influence
Bensch amp Ewing (1941) ndash tumour probably arose from embryonic fibro cartilage between basi-occiput and basi-sphenoid
Brunner (1942) ndash suggested origin from conjoined pharyngobasilar and buccopharyngeal fascia
Pathogenesis conthellip
Marten et al (1948) ndash proposed a hormonal theory suggesting that these tumours resulted from deficiency of androgens or overactivity of estrogens and that the hormonal stimulation is responsible for angiomatous components seen in JNA
Sternberg (1954) ndash proposed that JNA could be a type of hemangioma like a cutaneous hemangioma seen in children which regresses with age
Osborn (1959) ndash it could be due to either a hamartoma or residual fetal erectile tissue which were subjected to hormonal influence
Girgis amp fahmy (1973) ndash observed cell nests of undifferentiated epitheloid cells or ldquozellballenrdquo at the growing edge of angiofibromas and so considered it as a paraganglioma
Pathogenesis conthellip
The most accepted theory is that JNAs originate from sex steroid ndash stimulated hamartomatous tissue located in the turbinate cartilage The proposed hormonal influence explains why some JNAs involute after puberty
Recent immunocytochemical techniques shown that androgen receptors are present in at least 75 percent of tumours these
receptors are present in both the vascular and stromal elements progesterone receptors were found in some In contrast oestrogen receptors have not been demonstrated
Pathogenesis conthellip
Other factors that play a role in the growth of this tumour are The angiogenic growth factor (vascular endothelial growth factor (VEGF))
has been found localized on both endothelial and stromal cells indicating both cell types play a role in tumour development
Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67
But both of them do not have any relation to its degree of aggressiveness Overexpression of insulin-like growth factor II (IGFII)
found in a large number of juvenile angio fibromas IGFII gene is situated on the chromosome 11q associated with a tendency to recurrence and poorer prognosis
Pathogenesis conthellip
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Pathology conthellipMicroscopic ndash Consists of proliferating irregular vascular chan nels within a fibrous stroma Vascular component is more in young tumours and as age increases
collagen content increases Fibrous tissue increases towards periphery and vascular element tends to
be more central
Pathology conthellip
Tumour blood vessels typically lack smooth muscle and elastic fibres this is the reason for sustained bleeding
Cellular infiltration with plasma cells lymphocytes polymorphs eosinophils can be present
Stromal compartment consists spindle or stellate plump cells that give rise to varying amounts of collagen which makes some tumours very hard or firm
Mucous glands can be seen in superficial parts of the tumour underneath the epithelial covering
Pathogenesis
This tumour is almost exclusively found in adolescent boys so there is much speculation and indirect evidence that sex-hormone receptors play some part in its development
Theories associated with its aetiopathogenesis - Ringertz theory (1938) ndash JNA always arose from the periosteum of the skull
base Som amp Neffson (1940) ndash inequalities in the growth of bones forming skull base
resulted in hypertrophy of the underlying periosteum in response to hormonal influence
Bensch amp Ewing (1941) ndash tumour probably arose from embryonic fibro cartilage between basi-occiput and basi-sphenoid
Brunner (1942) ndash suggested origin from conjoined pharyngobasilar and buccopharyngeal fascia
Pathogenesis conthellip
Marten et al (1948) ndash proposed a hormonal theory suggesting that these tumours resulted from deficiency of androgens or overactivity of estrogens and that the hormonal stimulation is responsible for angiomatous components seen in JNA
Sternberg (1954) ndash proposed that JNA could be a type of hemangioma like a cutaneous hemangioma seen in children which regresses with age
Osborn (1959) ndash it could be due to either a hamartoma or residual fetal erectile tissue which were subjected to hormonal influence
Girgis amp fahmy (1973) ndash observed cell nests of undifferentiated epitheloid cells or ldquozellballenrdquo at the growing edge of angiofibromas and so considered it as a paraganglioma
Pathogenesis conthellip
The most accepted theory is that JNAs originate from sex steroid ndash stimulated hamartomatous tissue located in the turbinate cartilage The proposed hormonal influence explains why some JNAs involute after puberty
Recent immunocytochemical techniques shown that androgen receptors are present in at least 75 percent of tumours these
receptors are present in both the vascular and stromal elements progesterone receptors were found in some In contrast oestrogen receptors have not been demonstrated
Pathogenesis conthellip
Other factors that play a role in the growth of this tumour are The angiogenic growth factor (vascular endothelial growth factor (VEGF))
has been found localized on both endothelial and stromal cells indicating both cell types play a role in tumour development
Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67
But both of them do not have any relation to its degree of aggressiveness Overexpression of insulin-like growth factor II (IGFII)
found in a large number of juvenile angio fibromas IGFII gene is situated on the chromosome 11q associated with a tendency to recurrence and poorer prognosis
Pathogenesis conthellip
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Tumour blood vessels typically lack smooth muscle and elastic fibres this is the reason for sustained bleeding
Cellular infiltration with plasma cells lymphocytes polymorphs eosinophils can be present
Stromal compartment consists spindle or stellate plump cells that give rise to varying amounts of collagen which makes some tumours very hard or firm
Mucous glands can be seen in superficial parts of the tumour underneath the epithelial covering
Pathogenesis
This tumour is almost exclusively found in adolescent boys so there is much speculation and indirect evidence that sex-hormone receptors play some part in its development
Theories associated with its aetiopathogenesis - Ringertz theory (1938) ndash JNA always arose from the periosteum of the skull
base Som amp Neffson (1940) ndash inequalities in the growth of bones forming skull base
resulted in hypertrophy of the underlying periosteum in response to hormonal influence
Bensch amp Ewing (1941) ndash tumour probably arose from embryonic fibro cartilage between basi-occiput and basi-sphenoid
Brunner (1942) ndash suggested origin from conjoined pharyngobasilar and buccopharyngeal fascia
Pathogenesis conthellip
Marten et al (1948) ndash proposed a hormonal theory suggesting that these tumours resulted from deficiency of androgens or overactivity of estrogens and that the hormonal stimulation is responsible for angiomatous components seen in JNA
Sternberg (1954) ndash proposed that JNA could be a type of hemangioma like a cutaneous hemangioma seen in children which regresses with age
Osborn (1959) ndash it could be due to either a hamartoma or residual fetal erectile tissue which were subjected to hormonal influence
Girgis amp fahmy (1973) ndash observed cell nests of undifferentiated epitheloid cells or ldquozellballenrdquo at the growing edge of angiofibromas and so considered it as a paraganglioma
Pathogenesis conthellip
The most accepted theory is that JNAs originate from sex steroid ndash stimulated hamartomatous tissue located in the turbinate cartilage The proposed hormonal influence explains why some JNAs involute after puberty
Recent immunocytochemical techniques shown that androgen receptors are present in at least 75 percent of tumours these
receptors are present in both the vascular and stromal elements progesterone receptors were found in some In contrast oestrogen receptors have not been demonstrated
Pathogenesis conthellip
Other factors that play a role in the growth of this tumour are The angiogenic growth factor (vascular endothelial growth factor (VEGF))
has been found localized on both endothelial and stromal cells indicating both cell types play a role in tumour development
Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67
But both of them do not have any relation to its degree of aggressiveness Overexpression of insulin-like growth factor II (IGFII)
found in a large number of juvenile angio fibromas IGFII gene is situated on the chromosome 11q associated with a tendency to recurrence and poorer prognosis
Pathogenesis conthellip
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
This tumour is almost exclusively found in adolescent boys so there is much speculation and indirect evidence that sex-hormone receptors play some part in its development
Theories associated with its aetiopathogenesis - Ringertz theory (1938) ndash JNA always arose from the periosteum of the skull
base Som amp Neffson (1940) ndash inequalities in the growth of bones forming skull base
resulted in hypertrophy of the underlying periosteum in response to hormonal influence
Bensch amp Ewing (1941) ndash tumour probably arose from embryonic fibro cartilage between basi-occiput and basi-sphenoid
Brunner (1942) ndash suggested origin from conjoined pharyngobasilar and buccopharyngeal fascia
Pathogenesis conthellip
Marten et al (1948) ndash proposed a hormonal theory suggesting that these tumours resulted from deficiency of androgens or overactivity of estrogens and that the hormonal stimulation is responsible for angiomatous components seen in JNA
Sternberg (1954) ndash proposed that JNA could be a type of hemangioma like a cutaneous hemangioma seen in children which regresses with age
Osborn (1959) ndash it could be due to either a hamartoma or residual fetal erectile tissue which were subjected to hormonal influence
Girgis amp fahmy (1973) ndash observed cell nests of undifferentiated epitheloid cells or ldquozellballenrdquo at the growing edge of angiofibromas and so considered it as a paraganglioma
Pathogenesis conthellip
The most accepted theory is that JNAs originate from sex steroid ndash stimulated hamartomatous tissue located in the turbinate cartilage The proposed hormonal influence explains why some JNAs involute after puberty
Recent immunocytochemical techniques shown that androgen receptors are present in at least 75 percent of tumours these
receptors are present in both the vascular and stromal elements progesterone receptors were found in some In contrast oestrogen receptors have not been demonstrated
Pathogenesis conthellip
Other factors that play a role in the growth of this tumour are The angiogenic growth factor (vascular endothelial growth factor (VEGF))
has been found localized on both endothelial and stromal cells indicating both cell types play a role in tumour development
Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67
But both of them do not have any relation to its degree of aggressiveness Overexpression of insulin-like growth factor II (IGFII)
found in a large number of juvenile angio fibromas IGFII gene is situated on the chromosome 11q associated with a tendency to recurrence and poorer prognosis
Pathogenesis conthellip
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Marten et al (1948) ndash proposed a hormonal theory suggesting that these tumours resulted from deficiency of androgens or overactivity of estrogens and that the hormonal stimulation is responsible for angiomatous components seen in JNA
Sternberg (1954) ndash proposed that JNA could be a type of hemangioma like a cutaneous hemangioma seen in children which regresses with age
Osborn (1959) ndash it could be due to either a hamartoma or residual fetal erectile tissue which were subjected to hormonal influence
Girgis amp fahmy (1973) ndash observed cell nests of undifferentiated epitheloid cells or ldquozellballenrdquo at the growing edge of angiofibromas and so considered it as a paraganglioma
Pathogenesis conthellip
The most accepted theory is that JNAs originate from sex steroid ndash stimulated hamartomatous tissue located in the turbinate cartilage The proposed hormonal influence explains why some JNAs involute after puberty
Recent immunocytochemical techniques shown that androgen receptors are present in at least 75 percent of tumours these
receptors are present in both the vascular and stromal elements progesterone receptors were found in some In contrast oestrogen receptors have not been demonstrated
Pathogenesis conthellip
Other factors that play a role in the growth of this tumour are The angiogenic growth factor (vascular endothelial growth factor (VEGF))
has been found localized on both endothelial and stromal cells indicating both cell types play a role in tumour development
Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67
But both of them do not have any relation to its degree of aggressiveness Overexpression of insulin-like growth factor II (IGFII)
found in a large number of juvenile angio fibromas IGFII gene is situated on the chromosome 11q associated with a tendency to recurrence and poorer prognosis
Pathogenesis conthellip
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
The most accepted theory is that JNAs originate from sex steroid ndash stimulated hamartomatous tissue located in the turbinate cartilage The proposed hormonal influence explains why some JNAs involute after puberty
Recent immunocytochemical techniques shown that androgen receptors are present in at least 75 percent of tumours these
receptors are present in both the vascular and stromal elements progesterone receptors were found in some In contrast oestrogen receptors have not been demonstrated
Pathogenesis conthellip
Other factors that play a role in the growth of this tumour are The angiogenic growth factor (vascular endothelial growth factor (VEGF))
has been found localized on both endothelial and stromal cells indicating both cell types play a role in tumour development
Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67
But both of them do not have any relation to its degree of aggressiveness Overexpression of insulin-like growth factor II (IGFII)
found in a large number of juvenile angio fibromas IGFII gene is situated on the chromosome 11q associated with a tendency to recurrence and poorer prognosis
Pathogenesis conthellip
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Other factors that play a role in the growth of this tumour are The angiogenic growth factor (vascular endothelial growth factor (VEGF))
has been found localized on both endothelial and stromal cells indicating both cell types play a role in tumour development
Vessel density and both the expression and localization of VEGF correlate with the proliferative marker Ki67
But both of them do not have any relation to its degree of aggressiveness Overexpression of insulin-like growth factor II (IGFII)
found in a large number of juvenile angio fibromas IGFII gene is situated on the chromosome 11q associated with a tendency to recurrence and poorer prognosis
Pathogenesis conthellip
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Sporadic juvenile angiofibromas develop 25 times more frequently in patients with familial adenomatous polyposis (FAP) a condition that is associated with the germline mutations of adenomatous polyposis coli (APC) gene present on chromosome 5q
This gene regulates the beta-catenin pathway which influences cell to cell adhesion
Mutations of beta-catenin have been found in recurrent juvenile angiofibromas also
Beta-catenin localised only in the nuclei of stromal cells suggest that these cells have a critical role in the development of these neoplasms
Pathophysiology
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
The proposed origin of JNA is located along postero-lateral wall in the roof of nasopharynx usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate
Fetal histology confirms large areas of endothelial tissues in this region Rather than invading surrounding tissue this tumour displaces and distorts
relying on pressure necrosis to destroy and push through its bony confines Intracranial extension is noted in 10-20 of cases JNA are seldom seen in children below the age of 8 Rate of growth of tumour and period of maximum development coincides
with rate of erectile tissue of penis both increasing in size during the period of sexual development
PRESENTATION
Clinical features
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Nasal obstruction Intermittent unprovoked epistaxes Chronic anaemia may be present due to repeated epistaxis Complete nasal obstruction may cause stasis of secretions and may also
lead to sepsis Patients may have hyposmia or anosmia
2 cardinal symptoms
Clinical features conthellip
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Voice acquires a nasal intonation and may become plummy if swelling enlarges to force the soft palate down
Blockage of ET orifice may cause deafness and otalgia Headache may be present due to chronic sinusitis history or intracranial
extension Pressure on optic chaisma due to erosion of mass into the cranial cavity
may cause diplopia Tenting of the optic nerve by tumour mass may cause failing vision ( as
observed by shaheen et al)
Clinical features conthellip
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
On Anterior rhinoscopy ndash Abundant purulent nasal secretions Bowing of nasal septum to uninvolved side
On Posterior rhinoscopy ndash Pink or red mass filling the nasopharynx can be seen
Clinical features conthellip
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
When disease becomes extensive involving nose and infratemporal fossa gross physical signs will be seen ndash Nasal bones become spayed out Swelling in the temple and cheek occur
Intraoral palpation in the interval between ascending ramus of the mandible and the side of the maxilla may reveal the thickening of disease which has crept around the back of the antrum
Impaction of bulky mass in the infratemporal fossa results in trismus and bulging of the parotid
Proptosis is seen if orbital fissures are penetrated Frog face appearance may be seen if tumour is extensively spread involving
the ethmoidal region
Extension of the tumour
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
It follows the lines of least resistance ndash It mostly arises from SPF area It may have 2 components ndash one filling the
nasopharynx and the other extending out into the pterygopalatine and infratemporal fossa
Central stalk joining the 2 components occupy the SPF at the upper end of the vertical plate of palatine bone
1gt hangs down in the nasopharynx and may depress the soft palate when large enough
2gt may grow into IL nasal passage towards anterior nares It can cause pressure on lateral wall and also on septum bending it to opposite side Corresponding turbinates and ethmoidal air cells and the related antral wall may suffer pressure atrophy Lateral spread into the maxillary sinus may be responsible for the cheek swelling
3gt it can encroach into the orbit through infra orbital fissure 4gt it can erode skull base and cause intracranial problems
ASSESSMENT
Investigations
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Plain lateral view skull x-ray ndash Opacity in nose and PNS may be seenMass in the nasopharynx can be seenAnterior bowing of the posterior wall of the maxillary sinus can be seen called Holman-miller sign
Now-a-days the diagnosis is based on the CT and MR appearances that are sometimes confirmed by angiography
Biopsy is contra-indicated because of brisk haemorrhage
Investigations conthellip
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
The exact extent or stage of the tumour can only be determined by a combination of CT and MR imaging and this is vital for planning the surgical resection
CT is excellent for bone detail Both plain and contrast (lesion enhances) CT should be done CT reveals the extent of the lesion and helps in staging of the disease
Investigations conthellip
Investigations conthellip
Coronal CT bone window showing
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Widening of left sphenopalatine foramen Lesion filling left choanae Lesion extending into sphenoid sinus
Investigations conthellip
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Coronal CT soft tissue window with contrast showing Homogenous enhancement Widening of left sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Axial CT soft tissue window with contrast showing Homogenous enhancement Widening of right sphenopalatine
foramen Extension into nasopharynx and
pterygopalatine fossa
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Investigations conthellipMRI reveals the precise extent of the mass It differentiates tumour from other soft tissue structures
Investigations conthellip
Axial MRI T1 showingbull Heterogenous
intermediate signalbull Flow voids representing
enlarged vesselsbull Extension into
nasopharynx and masticator space
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Coronal MRI T1 with contrast showingbull Diffuse intense
enhancementbull Multiple flow voids within
hypervascular massbull Extension into
nasopharynx and pterygopalatine fossa
Axial MRI T2 showingbull Heterogenous
intermediate to high signal enhancement
bull Multiple flow voids within hypervascular mass
bull Extension into nasopharynx and pterygopalatine fossa
Investigations conthellip
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Diagnostic angiography is performed to identify the feeder vessel and to embolise it pre-operatively
Staging systems
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Staging is done for prognosis and for therapeutic approaches
Several staging systems have been proposed ndash Fisch Billers Andrews Radkowski
Fisch staging system ndash most robust and practical defines clearly which surgical approach is required
Staging conthellip
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Type 1 - Tumour limited to the nasopharyngeal cavity bone destruction negligible or limited to the sphenopalatine foramen
Type 2 - Tumour invading the pterygopalatine fossa or the maxillary ethmoid or sphenoid sinus with bone destruction
Type 3a - Tumour invading the infratemporal fossa or orbital region without intracranial involvement
Type 3b - Tumour invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement
Type 4a - Intracranial intradural tumour without infiltration of the cavernous sinus pituitary fossa or optic chiasma
Type 4b - Intracranial intradural tumour with infiltration of the cavernous sinus pituitary fossa or optic chiasma
Staging conthellip
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Radkowski staging system appeals to those involved with the management of smaller tumours as
there are more subdivisions but in reality this adds little to its utility Type 1a - Limited to the nose and nasopharyngeal area Type 1b - Extension into one or more sinuses Type 2a - Minimal extension into pterygopalatine fossa Type 2b - Occupation of the pterygopalatine fossa without orbital erosion Type 2c - Infratemporal fossa extension without cheek or pterygoid plate
involvement Type 3a - Erosion of the skull base (middle cranial fossa or pterygoids) Type 3b - Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
Staging conthellip
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Billers staging system - T1 - Tumour in nose and PNS except sphenoid with or without erosion of
bone T2 ndash Tumour extension into orbit or protruding into anterior cranial fossa T3 ndash Tumour involvement in the brain that is resectable with margins T4 ndash Unresectable tumour
Staging conthellip
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Andrews staging system ndash It is the currently accepted staging system Type I ndash tumour limited to the nasopharyngeal cavity bone destruction
negligible or limited to sphenopalatine foramen Type II ndash Tumour invading the pterygopalatine fossa or the maxillary
ethmoid or sphenoid sinus with bone destruction Type III ndash Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement
Type IV ndash Intracranial intradural tumour (a) without infiltration of cavernous sinus pituitary fossa optic chaisma (b) with infiltration of cavernous sinus pituitary fossa optic chaisma
Upto IVa surgery is advisable and for IVb radiotherapy is recommended
TREATMENT
History
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Hippocrates (470-410 BC) removed a hard nasal polyp through midline nose splitting incision and later it was found to be juvenile angiofibroma
Liston performed the first successful resection of an angiofibroma on a 21-year-old man with a history of symptoms present for at least 3-4 years and the mass filled the pharynx to the extent that it caused significant airway obstruction
It extended into his cheek and had eroded the alveolar process The patient had experienced a number of severe epistaxes losing two to three
pints of blood on each occasion He removed the tumour by performing a total maxillectomy through a Weber
Fergusson incision without anaesthesia Histopathological examination of the operative specimen showed the tumour
had a fibro-vascular nature
Preoperative embolization
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Its role is controversial For smaller tumours or if the feeder vessels are terminal branches of
internal maxillary then not needed For extensive lesions that get blood supply from branches of both ICA amp
ECA then it is necessary For medium-sized tumours the benefits of preoperative embolization are
doubtful Intraoperative blood loss after embolization is definitely less
Preoperative embolization conthellip
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
The maxillary or external carotid artery can be controlled or ligated relatively easily at an early stage in any open procedure regardless of whether embolization has been undertaken or not
Recurrence rate is thought to be reduced by this but it was found to be increased
May be tumour shrinkage makes the borders ill-defined in the bottom of a deep and bloody operative field and leads to inadequate resection
Preoperative embolization conthellip
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Done 24ndash72 hours pre-operatively Gelfoam or polyvinyl alcohol foam is used Gelfoam is resorbed in approximately 2 weeks Efficacy ndash stage 1 reduced from 840ml to 275ml blood loss
Preoperative chemotherapy
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Oestrogens causes shrinkage but it delays surgery and the unwanted secondary feminizing effects in an adolescent boy makes its usage limited
Gates et al observed 44 tumour shrinkage with flutamide (nonsteroidal androgen receptor blocker) in a small series of patients
Flutamide is regularly used in the management of prostatic cancer with side effects like nausea breast tenderness and gynaecomastia which were only temporary and disappeared completely at the end of therapy
So it seemed that this drug might have a role in the preoperative preparation of patients with very advanced tumours (with intracranial extension)
Unfortunately Labra et al observed shrinkage in only 75 cases in a pilot study and hence it was considered insignificant
SURGICAL RESECTION
Approaches
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Various approaches are available to access the area ndash Trans-nasal endoscopic Trans-palatal (wilson) Lateral rhinotomy Transhyoid Transmandibular (kermen) Sublabial Midfacial degloving (conley) Transzygomatic (sami amp girgis)
Approaches conthellip
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Combined approaches ndash A combination of various approaches can be attempted depending on the size and extent of the mass Trans-palatal sublabial (saldana) Transpalatal transantral (denker) Transcervical transmandibular (biller) Transpalatal + lateral rhinotomy Triple approach (hiranandani) = transpalatal + lateral rhinotomy + caldwell luc Butterfly sub-brow incision or extended lateral rhinotomy Frontotemporal craniotomy + transpalatal + lateral rhinotomy + dural tegmen
approach for stage IV
ENDOSCOPIC ENDONASAL TECHNIQUES
Endoscopic endonasal
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
These techniques became more widespread recently as there are advantages like reduced intra-op blood loss fewer post-op complications reduced length of hospital stay
Fisch - type 1 type 2 and some type 3 (with limited medial invasion of the infratem poral fossa) are done through this technique
Larger tumours and those extending across or through the skull base are difficult to remove through this technique
Endoscopic endonasal conthellip
Endoscopic endonasal conthellip
Procedure ndash
Preoperative embolization is undertaken
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
After the induction of anaesthesia the nose is prepared with a vasoconstrictor solution (4 cocaine or epinephrine 110000)
The anterior end of the middle turbinate is resected
Endoscopic endonasal conthellip
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
An anterior ethmoidectomy together with removal of the medial wall of the maxillary sinus is done
Access to the posterior wall of the antrum is gained
This wall is then removed to achieve complete lateral exposure of the tumour
Endoscopic endonasal conthellip
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Dissection is then continued into the sphenoid until its rostrum is reached
Tumour is peeled off inferiorly
A similar technique can be used to deliver the lateral extension of the tumour into the operative field
Endoscopic endonasal conthellip
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Bipolar diathermy is used throughout the procedure Ligaclips are used to control the feeding blood vessels A second surgeon can be helpful in aiding the resection of larger tumours
by applying the traction to the tumour and improve visibility by additional suction by accessing the nasal cavity through the contralateral nostril
OPEN APPROACHES
MIDFACIAL DEGLOVING APPROACH
Midfacial degloving
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Mid-facial degloving technique is adopted by most surgeons than other transpalatal or lateral rhinotomy because of the exposure it gives
It is a bilateral extended transnasal maxillary approach Anterior medial and posterior walls of the maxillary antrum can be
removed and a very large cavity that is confluent with the nasal cavity and post-nasal space is produced which gives adequate access for tumour removal together with control of its blood supply
Extensions into the inferior part of the orbit and infratemporal fossa can also be removed
There is no visible scarring and so cosmetically most feasible
Midfacial degloving conthellip
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
A sublabial degloving approach is suitable for larger tumors involving anteroinferior aspect of the nasal cavity and the infrastructure of the maxillary sinus and particularly when access to the posterosuperior part of the nasal cavity is not satisfactory through other approaches
bull Gingivo buccal incision is given
bull Nasal intercartilaginous incisions with transfixation incision is given
Midfacial degloving conthellip
bull Soft tissue elevation is done
bull Le fort I osteotomy is done
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
bull Mass in the nasopharynx is now accessible for removal
Midfacial degloving conthellip
Midfacial degloving approach
Midfacial degloving conthellip
>
TRANSPALATAL APPROACH
Transpalatal
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Axial view of CT scan of a patient with a small angiofibroma in the nasopharynx presenting superolat-eral to the soft palate on the left-hand side
Coronal view of MRI scan showing presence of the tumor at the posterior choana and the lateral nasopharyngeal wall on the left-hand side
Sagittal view of MRI scan showing cephalo-caudad location of the tumor confined to nasopharynx between the superior margin of the posterior choana and upto the upper surface of the soft palate
Transpalatal conthellip
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
General anaesthesia is induced through an orotracheal tube A Dingman self-retaining retractor is used to expose the hard palate and the
roof of the oral cavity An inverted U-shaped incision is outlined extending from one maxillary
tubercle to the other
Transpalatal excision conthellip
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
The mucosal incision is deepened through the mucoperiosteum upto the underlying bone of the hard palate
Using a periosteal elevator the posteriorly based bipedicled mucoperiosteal flap of the palate is elevated
Transpalatal excision conthellip
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
The blood supply to the bipedicled flap is derived from the palatine arteries on each side which are carefully preserved as the elevation of the flap approaches the soft palate
With use of an osteotome or a highspeed drill with a burr the posterior margin of the hard palate is excised to gain access to the region of the posterior choana on the left-hand side
Transpalatal excision conthellip
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
The mucoperiosteal flap is now retracted caudad with a tongue depressor to gain adequate exposure of the posterior choana and nasopharynx
Transpalatal excision conthellip
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
A close-up view of the exposure shows the lower border of the tumour presenting from the nasopharynx on the left-hand side
Transpalatal excision conthellip
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
A heavy silk suture is placed through the tumor and used as a retractor to permit mobilization of the tumor
Transpalatal excision conthellip
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
With use of careful and diligent alternate blunt and sharp dissection with the electrocautery or a curved scissors the tumour is mobilized from its various soft tissue attachments and dislodged from its bed in the nasopharynx
Transpalatal excision conthellip
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Brisk hemorrhage can occur and can be controlled easily with electrocautery or with sutures as appropriate
The blood supply usually is derived from the sphenopalatine artery which is electrocoagulated for haemostasis
The surgical defect shown demonstrates the empty space created by excision of the tumour at the posterior aspect of the left nasal cavity communicating with the nasopharynx
Transpalatal excision conthellip
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
After the wound is irrigated the surgical defect in the nasopharynx is left open to epithelialize by secondary intention
The mucoperiosteal bipedicled palatal flap is now returned to its position and sutured to the mucoperiosteal edge of the anterior aspect of the hard palate mucosa with interrupted Vicryl sutures
Transpalatal excision conthellip
monobloc excision ofthe bilobed tumor
yellowish-whitecompact fibromatous tumor
Transpalatal excision conthellip
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Postoperative Care Intense humidification to avoid crusting and drying of clots in the nasal
cavity Oral irrigations are started on the first postoperative day and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing speech or breathing
Transpalatal excision conthellip
>
MEDIAL MAXILLECTOMY APPROACH
Medial maxillectomy
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Larger angiofibromas extending from the nasal cavity and nasopharynx into the maxillary ethmoid or sphenoid sinuses require a wider exposure through a modified Weber-Ferguson incision or via a sublabial degloving approach
The patient presented with a history of nasal obstruction and epistaxis of 6 monthsrsquo duration
Appearance of the patient at the time of presentation
Medial maxillectomy conthellip
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Examination of the nasopharynx through the oral cavity with a 90-degree telescope showed the presence of a tumor projecting from the left posterior choana into the nasopharynx
Howeverthe posterior choana on the right-hand side is within normal limits
Medial maxillectomy conthellip
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
The CT scan in a coronal plane shows the tumor filling up the entire nasal cavity with extension into the nasopharynx and breaking through the medial wall of the maxilla into the left maxillary antrum
Medial maxillectomy conthellip
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Carotid angiography shows that this lesion is a vascular tumor deriving its blood supply mostly from the branches of the external carotid artery through the internal maxillary and sphenopalatine vessels
During the venous phase of the angiogram a highly vascular lesion is demonstrated
Selective embolization of the feeding vessels is recommended when the angiogram is performed
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
The surgical approach for a nasopharyngeal angiofibroma of this extent is via a medial maxillectomy
The eye is protected with a corneal shield A modified Weber-Ferguson incision with a Lynch
extension is preferred The incision is extended through the soft tissues of
the cheek to expose the anterior wall of the maxilla
Weber ndash ferguson incision
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
The incision divides the upper lip in the midline through the philtrum of the upper lip up to the columella
It then turns laterally and cephalad to enter the floor of the nasal vestibule along the root of the columella and takes a 45-degree turn and exits the floor of the nasal cavity remaining in the groove of the lateral aspect of the ala and follows the alar subunit all the way up to the lateral aspect of the nose
At this point the incision proceeds cephalad along the lateral aspect of the dorsal subunit of the nose up to the level of the medial canthus
Weber ndash ferguson incision
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
The incision can be modified with Lynch or subciliary extensions for larger tumors with significant superior or lateral extension
For the Lynch extension the incision continues cephalad on the lateral aspect of the bridge of the nose up to the medial aspect of the eyebrow
A subciliary extension takes 90-degree turn laterally onto the infraorbital skin and follows the most prominent skin crease of the lower eyelid toward the zygomatic process
Medial maxillectomy conthellip
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
An anterior wall antrotomy is made using a high-speed drill with a burr
Medial maxillectomy conthellip
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
The opening in the anterior wall of the maxilla is made wide enough to provide good digital access to the antrum
Care is taken to prevent injury to the infraorbital nerve which is carefully preserved
The opening in the anterior wall is extended up to the frontonasal process of the maxilla
In a close-up view of the exposure obtained the nodular tumor is seen presenting into the maxillary antrum from the nasal cavity
Medial maxillectomy conthellip
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Entry is now made into the nasal cavity by retracting the ala of the nostril to the right hand side
Because this tumor is benign and has a rubbery consistency it can be mobilized easily by digital maneuvers through the antrum and the nasal cavity and by palpation and mobilization of the tumor through the nasopharynx with a finger behind the soft palate into the nasopharynx
The nasal process of the maxilla is excised to create a large opening between the nasal cavity and the maxilla to facilitate delivery of the tumor
With use of appropriate digital maneuvers the angiofibroma is removed Meticulous attention should be given to gradual smooth complete removal
of all the lobulations of the tumor because it is easy to fracture the tumor and leave parts of it behind
Medial maxillectomy conthellip
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Brisk haemorrhage is to be anticipated during mobilization and removal of the specimen
However as soon as the specimen is delivered all bleeding can be controlled with packing and electrocoagulation
The surgical defect shows a large hollow space in the nasal cavity nasopharynx and maxillary antrum
Medial maxillectomy conthellip
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
All sharp bony spicules are smoothed out from the edges of the surgical defect
The wound is irrigated with Bacitracin solution and Xeroform packing is used in the surgical defect which is brought out through the left nostril
The skin incision is closed in two layers using 3-0 chromic catgut interrupted sutures for subcutaneous tissues and 5-0 nylon for skin
Medial maxillectomy conthellip
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
The surgical specimen shows a multilobulated nasopharyngeal angiofibroma removed from the sphenoid sinus nasopharynx nasal cavity and left maxillary antrum
Medial maxillectomy conthellip
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Postoperative care requires frequent irrigations of the nasal cavity and intranasal wound after the packing is removed in 5 to 7 days
Irrigations are continued until all crusting has cleared up and the nasal mucosa has epithelialized
Extra humidity is provided in the postoperative period to prevent crusting and bleeding from the nasal cavity The postoperative photograph of the patient approximately 3 months after surgery shows a well-healed aesthetically acceptable scar
Medial maxillectomy conthellip
A post-op endoscopic view of the nasopharynx shows total removal of tumor with clear choana and a well-epithelialized mucosal surface
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Wide anterior antrostomy is done Ascending process of maxilla is removed Inferior half of lateral nasal wall is also removed
RADIOTHERAPY
Radiotherapy
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
External beam radiation was delivered in several fractions to achieve a total tumour dose of 30-55 Gy
Reduction in the size of the tumour takes place but residual tumour remains
Local control rates of 80-85 percent have been achieved as assessed by clinical examination but no objective assessment was done on follow-up and recurrence rates were found to be more
Treatment failure was apparent usually within the first two to three years and surgical salvage was generally successful in all these patients
There are no reports on the efficacy of gamma-knife therapy as yet though some patients must have been treated by this means
COMPLICATIONS
Complications
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Recurrence ndash most common complication encountered reported in up to 25 percent of patients regardless of the method of
treatment more likely in patients with advanced disease and in those treated by
inexperienced surgeons Preoperative embolization is not associated The more younger the patient the more likely that future recurrence will
develop Most recurrences develop as a consequence of invasion of the
basisphenoid from surgical standpoint
Complications conthellip
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
drilling out the basisphenoid ensures that no residual tumour remains in the pterygoid canal or cancellous bone of the sphenoid
Prolonged clinical and radiological monitoring is neces sary for all these patients in view of the very high incidence of recurrent disease
Disease-free status five years after primary surgery probably represents cure Infra orbital nerve sensory deficits induced surgically and also nasal vestib ular
stenosis are recognized as a potential complication of mid-facial deglovingProlonged nasal crusting may develop into ozaena regular nasal douching with saline and the use of glucose in glycerine drops
can alleviate this unpleasant complica tion
Complications conthellip
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
Ocular problems - Displacement of the globe caused by loss of bony support Ophthalmoplegia Visual loss may also be present
Late complications that may develop after radiother apy include Growth retardation panhypopituitarism temporal lobe necrosis cataracts radiation keratopathy together with skin thyroid and nasopharyngeal malignancies
Some second neoplasms have developed in the radiation field at an even later date
CONCLUSION
Conclusion
Rare benign vascular tumor found almost exclusively in young males
Surgery is the gold standard with a trend towards endoscopic approaches Frequent follow-up after treatment is necessary
