Orbital defects1

Dr. Ali RazaDr. Ali RazaAssociate ProfessorAssociate ProfessorRawalpindi Medical CollegeRawalpindi Medical CollegeHoly Family Hospital Holy Family Hospital RawalpindiRawalpindi

Bony OrbitBony Orbit F: Frontal bone with F: Frontal bone with

associated suture and associated suture and notch.notch.

<- Trochlea<- Trochlea <- Optical Canal<- Optical Canal S: Superior orbital S: Superior orbital

fissurefissure I: Inferior orbital I: Inferior orbital

fissurefissure L: Lacrimal boneL: Lacrimal bone E: Ethmoid bone.E: Ethmoid bone.

Other Locations.Other Locations.

The lacrimal gland and The lacrimal gland and lacrimal sac as well as the lacrimal sac as well as the potential for multiple potential for multiple compartment involvement.compartment involvement.

Spaces of the Retrobulbar OrbitSpaces of the Retrobulbar Orbit

Intraconal space: Intraconal space: Contains CN II, ophthalmic artery, superior Contains CN II, ophthalmic artery, superior

division of CN III, nasociliary nerve (V1), division of CN III, nasociliary nerve (V1), inferior division of CN III, and CN VI.inferior division of CN III, and CN VI.

Extraconal space:Extraconal space: Contains ophthalmic vein, lacrimal nerve Contains ophthalmic vein, lacrimal nerve

(V1), CN IV and frontal nerve (V1).(V1), CN IV and frontal nerve (V1).

Spaces of the Retrobulbar OrbitSpaces of the Retrobulbar Orbit

Cone:Cone: Composed of the four rectus muscles and the Composed of the four rectus muscles and the

thin intramuscular membrane which joins thin intramuscular membrane which joins them and extends posteriorly to the insertion them and extends posteriorly to the insertion of the muscle tendons at the orbital apex.of the muscle tendons at the orbital apex.

THYROID OPHTHALMOPATHYTHYROID OPHTHALMOPATHY

PATHOLOGYPATHOLOGY HYPERTROPHY OF EXTRAOCULAR HYPERTROPHY OF EXTRAOCULAR

MUSCLESMUSCLES CELLULAR INFILTRATIONCELLULAR INFILTRATION PROLIFRATION OFORBITAL FATPROLIFRATION OFORBITAL FAT

SOFT TISSUE INVOLVEMENTSOFT TISSUE INVOLVEMENT

PERIORBITAL AND LID SWELLINGPERIORBITAL AND LID SWELLING CONJUNCTIVAL HYPERAEMIA CONJUNCTIVAL HYPERAEMIA CHEMOSISCHEMOSIS SUPERIOR LIMBIC SUPERIOR LIMBIC

KERATOCONJUNCTIVITISKERATOCONJUNCTIVITIS KERTOCONJUNCTIVITIS SICCAKERTOCONJUNCTIVITIS SICCA

Soft Tissue InvolvementSoft Tissue Involvement

Conjunctival InjectionConjunctival Injection

Severe ChemosisSevere Chemosis

LID RETRACTIONLID RETRACTION

CONTRACTION OF LEVATORCONTRACTION OF LEVATOR OVER ACTION OF OVER ACTION OF

LEVATOR/SUPERIOR RECTUSLEVATOR/SUPERIOR RECTUS OVER ACTION OF MULLER MUSCLEOVER ACTION OF MULLER MUSCLE

LID RETRACTIONLID RETRACTION

DALRYMPLE SIGN IN PRIMARY GAZEDALRYMPLE SIGN IN PRIMARY GAZE VON GRAEFE SIGN(LID LAG)VON GRAEFE SIGN(LID LAG) KOCHER SIGNKOCHER SIGN

Lid RetractionLid Retraction

Lid RetractionLid Retraction

Dalrymple SignDalrymple Sign

PROPTOSISPROPTOSIS

AXIAL BILATERAL/UNILATERALAXIAL BILATERAL/UNILATERAL COMPLICATIONSCOMPLICATIONS EXPOSUREEXPOSURE

ULCER ULCER

ProptosisProptosis

ProptosisProptosis

MeasurementMeasurement

ExophthalmometerExophthalmometer

ExophthalmometerExophthalmometer

OPTIC NEUROPATHYOPTIC NEUROPATHY

VISUAL ACUITY DECREASEDVISUAL ACUITY DECREASED VISUAL FIELD DEFECTS VISUAL FIELD DEFECTS

CENTRAL/PARACENTRALSCOTOMACENTRAL/PARACENTRALSCOTOMA OPTIC ATROPHYOPTIC ATROPHY

RESTRICTIVE MYOPATHYRESTRICTIVE MYOPATHY

ELEVATION- INF RECTUS FIBROSISELEVATION- INF RECTUS FIBROSIS ABDUCTION- MEDIAL RECTUS ABDUCTION- MEDIAL RECTUS

FIBROSISFIBROSIS DEPRESSION- SUPERIOR RECTUS DEPRESSION- SUPERIOR RECTUS

FIBROSISFIBROSIS ADDUCTION- LATERAL RECTUS ADDUCTION- LATERAL RECTUS

FIBROSISFIBROSIS

Restricted Eye MovementsRestricted Eye Movements

Restricted Elevation LE Restricted Abduction LE

Thyroid OphthalmopathyThyroid Ophthalmopathy

Thyroid (Muscle Thickening)Thyroid (Muscle Thickening)

Thyroid (Muscle Thickening)Thyroid (Muscle Thickening)

THYROID EYE THYROID EYE DISEASEDISEASE

Autoimmune disorder Autoimmune disorder characterised by infiltrative characterised by infiltrative

orbitopathy orbitopathy

THYROID EYE DISEASETHYROID EYE DISEASE

Associated with normal to abnormal Associated with normal to abnormal thyroid function which may coexist, thyroid function which may coexist, precede or follow the orbitopathy.precede or follow the orbitopathy.

Related to but not the same as Graves Related to but not the same as Graves Ophthalmopathy (GO) The natural history Ophthalmopathy (GO) The natural history was described by Rundle and Wilson in was described by Rundle and Wilson in 19451945

Thyroid status-Thyroid status-

Of those patients with thyroid orbitopathy, Of those patients with thyroid orbitopathy, approximately 80% are clinically hyperthyroid approximately 80% are clinically hyperthyroid and 20% are clinically euthyroid.4 Most patients and 20% are clinically euthyroid.4 Most patients with euthyroid Graves' orbitopathy, however, with euthyroid Graves' orbitopathy, however, have some detectable laboratory evidence of have some detectable laboratory evidence of subclinical hyperthyroidism.subclinical hyperthyroidism.

Both hyperthyroid and euthyroid patients can Both hyperthyroid and euthyroid patients can develop clinical signs and symptoms of thyroid develop clinical signs and symptoms of thyroid orbitopathy. In general, patients with euthyroid orbitopathy. In general, patients with euthyroid Graves' disease tend to have less severe Graves' disease tend to have less severe orbitopathyorbitopathy


The goal is to identify and treat patients who are at The goal is to identify and treat patients who are at particular risk of sight threatening complications. The particular risk of sight threatening complications. The disease has a finite period of activity until it becomes disease has a finite period of activity until it becomes burnt out.The yellow region shows the early phase burnt out.The yellow region shows the early phase where there is the best response to treatment.where there is the best response to treatment.

Type 1 younger age group, whiter eyes with proptosis. Type 1 younger age group, whiter eyes with proptosis. Inflammation is mostly in orbital fat not muscles.Inflammation is mostly in orbital fat not muscles.

Type 11 older patient with red eyes, severe sight Type 11 older patient with red eyes, severe sight threatening disease, tobacco addiction is frequent.threatening disease, tobacco addiction is frequent.

THYROID EYE DISEASETHYROID EYE DISEASE LID RETRACTION1. LID RETRACTION1.

sympathetic overactivity sympathetic overactivity infiltration of levator / SR infiltration of levator / SR complex. hypotropia complex. hypotropia (retraction disappears on (retraction disappears on downgaze)downgaze)

SIGNS:- Dalrymples (lid SIGNS:- Dalrymples (lid retraction), von Graefe retraction), von Graefe (lid lag), Kocher´s (staring (lid lag), Kocher´s (staring appearance)appearance)


INFILTRATIONINFILTRATION 1. soft tissue 1. soft tissue

involvement :- involvement :- chemosis, chemosis, conjunctival injection conjunctival injection over the recti over the recti insertions, puffy lidsinsertions, puffy lids


Superior limbic Superior limbic keratoconjunctivitis keratoconjunctivitis (SLK)(SLK)

Optic neuropathy with visual Optic neuropathy with visual lossloss

The prevalence of optic neuropathy with visual loss in The prevalence of optic neuropathy with visual loss in patients with thyroid orbitopathy is less than 5%.patients with thyroid orbitopathy is less than 5%.

Optic neuropathy is, however, the most common cause Optic neuropathy is, however, the most common cause of blindness secondary to thyroid orbitopathy. Its onset is of blindness secondary to thyroid orbitopathy. Its onset is often insidious and may be masked by other symptoms. often insidious and may be masked by other symptoms. These patients are usually older (age 50 to 70) are more These patients are usually older (age 50 to 70) are more frequently male, have a later onset of thyroid disease, frequently male, have a later onset of thyroid disease, and more often have diabetes.and more often have diabetes.

Optic neuropathy is usually bilateral, but up to one third Optic neuropathy is usually bilateral, but up to one third of cases may be unilateral.of cases may be unilateral.

Optic neuropathyOptic neuropathy Although a history of decreased vision should be carefully sought, it Although a history of decreased vision should be carefully sought, it

is important to realize that optic neuropathy can occur in a is important to realize that optic neuropathy can occur in a significant number (18%) of patients with visual acuities in the range significant number (18%) of patients with visual acuities in the range of 20/20 to 20/25 (6/6 to 6/7.5).*An afferent pupillary defect is of 20/20 to 20/25 (6/6 to 6/7.5).*An afferent pupillary defect is present in 35%. An abnormal disc (either swollen or pale) is seen in present in 35%. An abnormal disc (either swollen or pale) is seen in only 52%. Visual field defects are present in 66%.Other tests that only 52%. Visual field defects are present in 66%.Other tests that can be useful include color vision testing and visual evoked can be useful include color vision testing and visual evoked potentials (VEPs). The Farnsworth-Munsell 100-hue test is a potentials (VEPs). The Farnsworth-Munsell 100-hue test is a sensitive indicator of optic nerve dysfunction, but sensitive indicator of optic nerve dysfunction, but pseudoisochromatic screening procedures (e.g.,Ishihara plates) pseudoisochromatic screening procedures (e.g.,Ishihara plates) rarely identify an acquired color defect unless optic neuropathy is rarely identify an acquired color defect unless optic neuropathy is severe.The pattern reversal VEP is very sensitive at detecting early severe.The pattern reversal VEP is very sensitive at detecting early optic neuropathy and may be a useful means of following patients optic neuropathy and may be a useful means of following patients after treatment.after treatment.

Intraocular pressureIntraocular pressure The increased intraocular pressure measured during The increased intraocular pressure measured during

upgaze in patients with thyroid orbitopathy has been a upgaze in patients with thyroid orbitopathy has been a controversial finding. When restriction of the inferior controversial finding. When restriction of the inferior rectus muscle occurs, the intraocular pressure may rectus muscle occurs, the intraocular pressure may increase by 6 mm Hg or more in upgaze as compared increase by 6 mm Hg or more in upgaze as compared with primary gaze. The increased intraocular pressure in with primary gaze. The increased intraocular pressure in upgaze is a normal phenomenon exaggerated by thyroid upgaze is a normal phenomenon exaggerated by thyroid orbitopathyorbitopathy

In patients with severe infiltrative disease there is an In patients with severe infiltrative disease there is an increased pressure on upgaze as compared with normal increased pressure on upgaze as compared with normal controls and patients with mild disease. It is often not an controls and patients with mild disease. It is often not an indicator of early disease because it occurs infrequently indicator of early disease because it occurs infrequently in patients with minimal eye findingsin patients with minimal eye findings

INVESTIGATIONINVESTIGATION SEROLOGICALT3 (hyperthyroid)T4 TSH SEROLOGICALT3 (hyperthyroid)T4 TSH

(hypothyroid)TSI (thyroid stimulating immunoglobulin).(hypothyroid)TSI (thyroid stimulating immunoglobulin). RADIOLOGICAL TESTSOrbital CT (enlarged muscle RADIOLOGICAL TESTSOrbital CT (enlarged muscle

belly, tendon normal). Coca-Cola bottle sign = muscle belly, tendon normal). Coca-Cola bottle sign = muscle swelling deforming ethmoidal bones.MRI T2 showing swelling deforming ethmoidal bones.MRI T2 showing oedema of muscles; repeating the scan in different oedema of muscles; repeating the scan in different positions of gaze can create a pseudo-video of eye positions of gaze can create a pseudo-video of eye movements (for assessment of muscle restriction).movements (for assessment of muscle restriction).

RADIOISOTOPE TESTS Octreoscan: quantitative RADIOISOTOPE TESTS Octreoscan: quantitative uptake of radio-labelled octreotide (which is a uptake of radio-labelled octreotide (which is a somatostatin analogue).somatostatin analogue).

VISUAL FIELDVISUAL FIELD

A visual field should be performed in all patients A visual field should be performed in all patients suspected to have optic neuropathy and is suspected to have optic neuropathy and is useful when following patients after initiation of useful when following patients after initiation of treatment. Characteristically, a central scotoma treatment. Characteristically, a central scotoma or an inferior altitudinal defect is seen in cases or an inferior altitudinal defect is seen in cases of compressive optic neuropathy. Other visual of compressive optic neuropathy. Other visual field defects include an enlarged blind spot, field defects include an enlarged blind spot, paracentral scotoma, nerve fiber bundle defect, paracentral scotoma, nerve fiber bundle defect, or generalized constriction.or generalized constriction.

CT findings in thyroid orbitopathyCT findings in thyroid orbitopathy

The most characteristic CT finding in thyroid orbitopathy The most characteristic CT finding in thyroid orbitopathy is enlargement of the extraocular muscles with normal is enlargement of the extraocular muscles with normal tendinous insertions onto the globe. Other findings tendinous insertions onto the globe. Other findings include proptosis and anterior prolapse of the orbital include proptosis and anterior prolapse of the orbital septum due to excessive orbital fat and muscle swelling septum due to excessive orbital fat and muscle swelling (see Fig. 4).Patients at risk for developing optic (see Fig. 4).Patients at risk for developing optic neuropathy may also have severe apical crowding, a neuropathy may also have severe apical crowding, a dilated superior ophthalmic vein, and anterior dilated superior ophthalmic vein, and anterior displacement of the lacrimal gland. Of these, apical displacement of the lacrimal gland. Of these, apical crowding is the most sensitive indicator for the presence crowding is the most sensitive indicator for the presence of optic neuropathy The CT scan should be done in the of optic neuropathy The CT scan should be done in the coronal plane to assess the enlargement of the coronal plane to assess the enlargement of the extraocular muscles at the apex because axial sections extraocular muscles at the apex because axial sections can sometimes be misleading.can sometimes be misleading.


Orbital CT -(enlarged Orbital CT -(enlarged muscle belly, tendon muscle belly, tendon normal)normal)

SYSTEMIC THYROID DISEASESYSTEMIC THYROID DISEASE

There are no good recent studies of the natural There are no good recent studies of the natural history of untreated hyperthyroidism, but based history of untreated hyperthyroidism, but based on older reports, Wilson56 determined that on older reports, Wilson56 determined that about one third of patients spontaneously about one third of patients spontaneously improve, one third remain chronically improve, one third remain chronically hyperthyroid, and one third progress to thyroid hyperthyroid, and one third progress to thyroid storm and occasionally death. Because it is not storm and occasionally death. Because it is not possible to predict which patients will possible to predict which patients will spontaneously improve, treatment of thyroid spontaneously improve, treatment of thyroid dysfunction is recommended.dysfunction is recommended.

TreatmentTreatment

acute congestive ophthalmopathy, acute congestive ophthalmopathy, compressive optic neuropathy, compressive optic neuropathy, motility disorders,motility disorders, eyelid abnormalities.eyelid abnormalities.

TREATMENTTREATMENTAcute Congestive OrbitopathyAcute Congestive Orbitopathy

1. SYMPTOMATIC:- elevate bedhead, lubricants, lid taping, 1. SYMPTOMATIC:- elevate bedhead, lubricants, lid taping, diureticsdiuretics

2. SYSTEMIC:- 2. SYSTEMIC:- a) Normalise thyroid function with or without thyroxine.a) Normalise thyroid function with or without thyroxine.

Patients rendered euthyroid do improve their GO scorePatients rendered euthyroid do improve their GO score

Tallstedt trial N Eng J Med 1992Tallstedt trial N Eng J Med 1992

antithyroid drugs cause a 10% chance of new or worsening GOantithyroid drugs cause a 10% chance of new or worsening GO

but radio-iodine causes a 30% chance of new or worsening GO.but radio-iodine causes a 30% chance of new or worsening GO.

Corticosteroids have been used successfully in the Corticosteroids have been used successfully in the treatment of acute congestive orbitopathytreatment of acute congestive orbitopathy

Corticosteroids have been used successfully in the treatment of Corticosteroids have been used successfully in the treatment of acute congestive orbitopathy. They are believed to work by altering acute congestive orbitopathy. They are believed to work by altering cell-mediated immune response and diminishing the production of cell-mediated immune response and diminishing the production of mucopolysaccharides by the orbital fibroblasts.Corticosteroids result mucopolysaccharides by the orbital fibroblasts.Corticosteroids result in improvement of soft tissue involvement and compressive optic in improvement of soft tissue involvement and compressive optic neuropathy (but do not have as much of an effect on diplopia neuropathy (but do not have as much of an effect on diplopia Traditionally, a "short burst" of high-dose corticosteroids has been Traditionally, a "short burst" of high-dose corticosteroids has been given, usually in the range of 60 to 120 mg/day of oral prednisone. given, usually in the range of 60 to 120 mg/day of oral prednisone. Improvement in subjective symptoms such as pain and tearing Improvement in subjective symptoms such as pain and tearing usually occurs first, often as early as 24 to 48 hours, followed by usually occurs first, often as early as 24 to 48 hours, followed by improvement in soft tissue congestion and muscle function over a improvement in soft tissue congestion and muscle function over a period of days to weeks.period of days to weeks.

Steroid TherapySteroid Therapy

Prednisone or prednisolonePrednisone or prednisolone This is standard treatment but there are frequent side effects. No This is standard treatment but there are frequent side effects. No

response in 35% of patients and anyway the response is only response in 35% of patients and anyway the response is only partial. High dose steroids given early in the disease when muscle partial. High dose steroids given early in the disease when muscle swelling occurs does not necessarily limit the long term course of swelling occurs does not necessarily limit the long term course of the disease. If there is no response to high dose steroids in the first the disease. If there is no response to high dose steroids in the first three weeks they should be rapidly reduced. Prednisolone + orbital three weeks they should be rapidly reduced. Prednisolone + orbital radiotherapy has slightly more effect than either alone.Use high radiotherapy has slightly more effect than either alone.Use high dose pulsed methylprednisolone if urgent optic nerve dose pulsed methylprednisolone if urgent optic nerve decompression is required, This is more effective than oral decompression is required, This is more effective than oral treatment but it is expensive and not justified in most cases of TED.treatment but it is expensive and not justified in most cases of TED.

Radiation therapyRadiation therapy

During the past few years, radiation therapy has reemerged as a During the past few years, radiation therapy has reemerged as a useful form of treatment of severe orbitopathy. The rationale for the useful form of treatment of severe orbitopathy. The rationale for the use of radiation therapy is reduction or elimination of the pathogenic use of radiation therapy is reduction or elimination of the pathogenic orbital lymphocytes, which are markedly radiosensitive. It is also orbital lymphocytes, which are markedly radiosensitive. It is also thought that the glycosaminoglycan production by fibroblasts is thought that the glycosaminoglycan production by fibroblasts is reduced, thereby reducing orbital edema, orbital tension, and reduced, thereby reducing orbital edema, orbital tension, and conjunctival injection. Although congestive findings improve most conjunctival injection. Although congestive findings improve most consistently, significant improvement in proptosis and extraocular consistently, significant improvement in proptosis and extraocular muscle function has been reported.Like corticosteroids, radiation muscle function has been reported.Like corticosteroids, radiation therapy is most effective within the first year, when significant therapy is most effective within the first year, when significant fibrotic changes have not yet occurred. Mourits and associates,135 fibrotic changes have not yet occurred. Mourits and associates,135 however, suggest that periods of active orbital inflammation within however, suggest that periods of active orbital inflammation within the long natural history of thyroid orbitopathy would benefit from the long natural history of thyroid orbitopathy would benefit from corticosteroids or radiation therapy. corticosteroids or radiation therapy.

RadiotherapyRadiotherapy

RETROBULBAR RADIOTHERAPY:- Trial of RETROBULBAR RADIOTHERAPY:- Trial of prednisone versus radiotherapy showed no prednisone versus radiotherapy showed no difference in clinical improvement (about difference in clinical improvement (about 50%).The patients all tolerated retrobulbar 50%).The patients all tolerated retrobulbar radiotherapy better than steroids Consider if radiotherapy better than steroids Consider if steroid maintenance > 25mg/ day. Best effect in steroid maintenance > 25mg/ day. Best effect in acute disease.Do not irradiate patients with acute disease.Do not irradiate patients with diabetes mellitus as they are more susceptible diabetes mellitus as they are more susceptible to radiation retinopathy.2000rads/ 10days, effect to radiation retinopathy.2000rads/ 10days, effect starts at 4 weeks, maximal 4 months.starts at 4 weeks, maximal 4 months.

Compressive Optic NeuropathyCompressive Optic Neuropathy

Compressive optic neuropathy can cause Compressive optic neuropathy can cause permanent visual loss. The treatment permanent visual loss. The treatment possibilities include high doses of possibilities include high doses of corticosteroids, irradiation, and orbital corticosteroids, irradiation, and orbital decompression. Some patients require only one decompression. Some patients require only one of these modalities, while other patients need of these modalities, while other patients need combined therapies. combined therapies.


As in the treatment of acute congestive thyroid As in the treatment of acute congestive thyroid orbitopathy, radiation therapy is becoming increasingly orbitopathy, radiation therapy is becoming increasingly popular. A retrospective series of 84 patients with popular. A retrospective series of 84 patients with compressive optic neuropathy treated with either compressive optic neuropathy treated with either corticosteroids or radiation therapy supports mounting corticosteroids or radiation therapy supports mounting evidence that radiation therapy may be safer and more evidence that radiation therapy may be safer and more effective than corticosteroids.effective than corticosteroids.

Radiation therapy, however, must be administered in Radiation therapy, however, must be administered in fractionated doses, which delays its beneficial effect. For fractionated doses, which delays its beneficial effect. For this reason, if visual dysfunction progresses while the this reason, if visual dysfunction progresses while the patient is on corticosteroids, surgical decompression is patient is on corticosteroids, surgical decompression is usually recommended if the patient is a surgical usually recommended if the patient is a surgical candidate.candidate.

Orbital decompressionOrbital decompression

Orbital decompression is indicated for compressive optic Orbital decompression is indicated for compressive optic neuropathy when there has been failure of or neuropathy when there has been failure of or contraindication for corticosteroids or radiation therapy or contraindication for corticosteroids or radiation therapy or if corticosteroid dependence has developed with if corticosteroid dependence has developed with intolerable side effects. Other indications include intolerable side effects. Other indications include excessive proptosis with exposure keratitis and corneal excessive proptosis with exposure keratitis and corneal ulceration, pain relief, and cosmesis for disfiguring ulceration, pain relief, and cosmesis for disfiguring exophthalmos. Orbital decompression may also be exophthalmos. Orbital decompression may also be indicated as a preliminary procedure to extraocular indicated as a preliminary procedure to extraocular muscle surgery on a patient with sufficient proptosis to muscle surgery on a patient with sufficient proptosis to suggest that decompression might ultimately be suggest that decompression might ultimately be required.required.


A variety of approaches may be used, each with A variety of approaches may be used, each with its own advantages and associated its own advantages and associated complications.complications.

The transorbital (via fornix or eyelid) approach to The transorbital (via fornix or eyelid) approach to inferior and medial wall decompression is the inferior and medial wall decompression is the most common approach used by most common approach used by ophthalmologists. The addition of a lateral wall ophthalmologists. The addition of a lateral wall advancement has the advantage of both further advancement has the advantage of both further increasing the orbital volume and simultaneously increasing the orbital volume and simultaneously improving upper eyelid retraction; this is the improving upper eyelid retraction; this is the technique we prefer.technique we prefer.

ORBITAL DECOMPRESSIONORBITAL DECOMPRESSION

Subciliary approach.Inferior & medial wall (6mm Subciliary approach.Inferior & medial wall (6mm proptosis).Remove bone to posterior wall proptosis).Remove bone to posterior wall maxillary sinus (5mm more posterior on medial maxillary sinus (5mm more posterior on medial wall), Avoid IO neurovascular bundle, and the wall), Avoid IO neurovascular bundle, and the anterior and posterior ethmoidal arteries.Incise anterior and posterior ethmoidal arteries.Incise periosteum in A-P direction posteriorly and periosteum in A-P direction posteriorly and circumferentially anteriorly.circumferentially anteriorly.

Complications: Complications: visual loss, visual loss, A pattern ETA pattern ET

Motility DisordersMotility Disorders

A major source of morbidity in thyroid orbitopathy, and the most A major source of morbidity in thyroid orbitopathy, and the most frequent problem associated with orbital decompression surgery, frequent problem associated with orbital decompression surgery, has been strabismus. In patients with relatively minimal degrees of has been strabismus. In patients with relatively minimal degrees of ocular misalignment, diplopia can be avoided with a compensatory ocular misalignment, diplopia can be avoided with a compensatory head posture, Fresnel plastic press-on prisms, or temporary head posture, Fresnel plastic press-on prisms, or temporary occlusion. Unfortunately there is significant image degradation as occlusion. Unfortunately there is significant image degradation as larger prisms are used, limiting their efficacy. If there is marked larger prisms are used, limiting their efficacy. If there is marked asymmetry in ocular deviation in different fields of gaze, prisms are asymmetry in ocular deviation in different fields of gaze, prisms are also less effective. In some cases during the inflammatory period, also less effective. In some cases during the inflammatory period, use of intramuscular botulinum toxin has shown some efficacy.use of intramuscular botulinum toxin has shown some efficacy.

Extraocular muscle surgery should be postponed until the muscles Extraocular muscle surgery should be postponed until the muscles are no longer inflamed and the deviation has remained stable for at are no longer inflamed and the deviation has remained stable for at least 6 months.least 6 months.

Eyelid AbnormalitiesEyelid Abnormalities

As with other thyroid eye problems, eyelid retraction will often As with other thyroid eye problems, eyelid retraction will often improve with time, and only an estimated 50% of patients with eyelid improve with time, and only an estimated 50% of patients with eyelid retraction have a significant eyelid abnormality 5 years later.retraction have a significant eyelid abnormality 5 years later.

Eyelid retraction can result from excessive autonomic discharge, Eyelid retraction can result from excessive autonomic discharge, levator fibrosis, or contraction of the inferior rectus muscle.levator fibrosis, or contraction of the inferior rectus muscle.

Surgical correction of eyelid abnormalities should be performed only Surgical correction of eyelid abnormalities should be performed only after orbital or extraocular muscle surgery because these operations after orbital or extraocular muscle surgery because these operations may change eyelid position. For example, inferior rectus muscle may change eyelid position. For example, inferior rectus muscle restriction may cause upper eyelid retraction because of the restriction may cause upper eyelid retraction because of the superior rectus/levator palpebrae superioris overaction against the superior rectus/levator palpebrae superioris overaction against the restriction. Specific techniques for repair of eyelid retraction are restriction. Specific techniques for repair of eyelid retraction are discussed in other chapters.discussed in other chapters.

Steroid TherapySteroid Therapy

Prednisone or prednisolonePrednisone or prednisolone This is standard treatment but there are frequent side effects. No This is standard treatment but there are frequent side effects. No

response in 35% of patients and anyway the response is only response in 35% of patients and anyway the response is only partial. High dose steroids given early in the disease when muscle partial. High dose steroids given early in the disease when muscle swelling occurs does not necessarily limit the long term course of swelling occurs does not necessarily limit the long term course of the disease. If there is no response to high dose steroids in the first the disease. If there is no response to high dose steroids in the first three weeks they should be rapidly reduced. Prednisolone + orbital three weeks they should be rapidly reduced. Prednisolone + orbital radiotherapy has slightly more effect than either alone.Use high radiotherapy has slightly more effect than either alone.Use high dose pulsed methylprednisolone if urgent optic nerve dose pulsed methylprednisolone if urgent optic nerve decompression is required, This is more effective than oral decompression is required, This is more effective than oral treatment but it is expensive and not justified in most cases of TED.treatment but it is expensive and not justified in most cases of TED.

Radiation therapyRadiation therapy

During the past few years, radiation therapy has reemerged as a During the past few years, radiation therapy has reemerged as a useful form of treatment of severe orbitopathy. The rationale for the useful form of treatment of severe orbitopathy. The rationale for the use of radiation therapy is reduction or elimination of the pathogenic use of radiation therapy is reduction or elimination of the pathogenic orbital lymphocytes, which are markedly radiosensitive. It is also orbital lymphocytes, which are markedly radiosensitive. It is also thought that the glycosaminoglycan production by fibroblasts is thought that the glycosaminoglycan production by fibroblasts is reduced, thereby reducing orbital edema, orbital tension, and reduced, thereby reducing orbital edema, orbital tension, and conjunctival injection. Although congestive findings improve most conjunctival injection. Although congestive findings improve most consistently, significant improvement in proptosis and extraocular consistently, significant improvement in proptosis and extraocular muscle function has been reported.Like corticosteroids, radiation muscle function has been reported.Like corticosteroids, radiation therapy is most effective within the first year, when significant therapy is most effective within the first year, when significant fibrotic changes have not yet occurred. Mourits and associates,135 fibrotic changes have not yet occurred. Mourits and associates,135 however, suggest that periods of active orbital inflammation within however, suggest that periods of active orbital inflammation within the long natural history of thyroid orbitopathy would benefit from the long natural history of thyroid orbitopathy would benefit from corticosteroids or radiation therapy. corticosteroids or radiation therapy.

RadiotherapyRadiotherapy

RETROBULBAR RADIOTHERAPY:- Trial of RETROBULBAR RADIOTHERAPY:- Trial of prednisone versus radiotherapy showed no prednisone versus radiotherapy showed no difference in clinical improvement (about difference in clinical improvement (about 50%).The patients all tolerated retrobulbar 50%).The patients all tolerated retrobulbar radiotherapy better than steroids Consider if radiotherapy better than steroids Consider if steroid maintenance > 25mg/ day. Best effect in steroid maintenance > 25mg/ day. Best effect in acute disease.Do not irradiate patients with acute disease.Do not irradiate patients with diabetes mellitus as they are more susceptible diabetes mellitus as they are more susceptible to radiation retinopathy.2000rads/ 10days, effect to radiation retinopathy.2000rads/ 10days, effect starts at 4 weeks, maximal 4 months.starts at 4 weeks, maximal 4 months.


Compressive optic neuropathy can cause Compressive optic neuropathy can cause permanent visual loss. The treatment permanent visual loss. The treatment possibilities include high doses of possibilities include high doses of corticosteroids, irradiation, and orbital corticosteroids, irradiation, and orbital decompression. Some patients require only one decompression. Some patients require only one of these modalities, while other patients need of these modalities, while other patients need combined therapies. combined therapies.


As in the treatment of acute congestive thyroid As in the treatment of acute congestive thyroid orbitopathy, radiation therapy is becoming increasingly orbitopathy, radiation therapy is becoming increasingly popular. A retrospective series of 84 patients with popular. A retrospective series of 84 patients with compressive optic neuropathy treated with either compressive optic neuropathy treated with either corticosteroids or radiation therapy supports mounting corticosteroids or radiation therapy supports mounting evidence that radiation therapy may be safer and more evidence that radiation therapy may be safer and more effective than corticosteroids.effective than corticosteroids.

Radiation therapy, however, must be administered in Radiation therapy, however, must be administered in fractionated doses, which delays its beneficial effect. For fractionated doses, which delays its beneficial effect. For this reason, if visual dysfunction progresses while the this reason, if visual dysfunction progresses while the patient is on corticosteroids, surgical decompression is patient is on corticosteroids, surgical decompression is usually recommended if the patient is a surgical usually recommended if the patient is a surgical candidate.candidate.


Orbital decompression is indicated for compressive optic Orbital decompression is indicated for compressive optic neuropathy when there has been failure of or neuropathy when there has been failure of or contraindication for corticosteroids or radiation therapy or contraindication for corticosteroids or radiation therapy or if corticosteroid dependence has developed with if corticosteroid dependence has developed with intolerable side effects. Other indications include intolerable side effects. Other indications include excessive proptosis with exposure keratitis and corneal excessive proptosis with exposure keratitis and corneal ulceration, pain relief, and cosmesis for disfiguring ulceration, pain relief, and cosmesis for disfiguring exophthalmos. Orbital decompression may also be exophthalmos. Orbital decompression may also be indicated as a preliminary procedure to extraocular indicated as a preliminary procedure to extraocular muscle surgery on a patient with sufficient proptosis to muscle surgery on a patient with sufficient proptosis to suggest that decompression might ultimately be suggest that decompression might ultimately be required.required.


A variety of approaches may be used, each with A variety of approaches may be used, each with its own advantages and associated its own advantages and associated complications.complications.

The transorbital (via fornix or eyelid) approach to The transorbital (via fornix or eyelid) approach to inferior and medial wall decompression is the inferior and medial wall decompression is the most common approach used by most common approach used by ophthalmologists. The addition of a lateral wall ophthalmologists. The addition of a lateral wall advancement has the advantage of both further advancement has the advantage of both further increasing the orbital volume and simultaneously increasing the orbital volume and simultaneously improving upper eyelid retraction; this is the improving upper eyelid retraction; this is the technique we prefer.technique we prefer.

ORBITAL DECOMPRESSIONORBITAL DECOMPRESSION

Subciliary approach.Inferior & medial wall (6mm Subciliary approach.Inferior & medial wall (6mm proptosis).Remove bone to posterior wall proptosis).Remove bone to posterior wall maxillary sinus (5mm more posterior on medial maxillary sinus (5mm more posterior on medial wall), Avoid IO neurovascular bundle, and the wall), Avoid IO neurovascular bundle, and the anterior and posterior ethmoidal arteries.Incise anterior and posterior ethmoidal arteries.Incise periosteum in A-P direction posteriorly and periosteum in A-P direction posteriorly and circumferentially anteriorly.circumferentially anteriorly.

Complications: Complications: visual loss, visual loss, A pattern ETA pattern ET

Motility DisordersMotility Disorders

A major source of morbidity in thyroid orbitopathy, and the most A major source of morbidity in thyroid orbitopathy, and the most frequent problem associated with orbital decompression surgery, frequent problem associated with orbital decompression surgery, has been strabismus. In patients with relatively minimal degrees of has been strabismus. In patients with relatively minimal degrees of ocular misalignment, diplopia can be avoided with a compensatory ocular misalignment, diplopia can be avoided with a compensatory head posture, Fresnel plastic press-on prisms, or temporary head posture, Fresnel plastic press-on prisms, or temporary occlusion. Unfortunately there is significant image degradation as occlusion. Unfortunately there is significant image degradation as larger prisms are used, limiting their efficacy. If there is marked larger prisms are used, limiting their efficacy. If there is marked asymmetry in ocular deviation in different fields of gaze, prisms are asymmetry in ocular deviation in different fields of gaze, prisms are also less effective. In some cases during the inflammatory period, also less effective. In some cases during the inflammatory period, use of intramuscular botulinum toxin has shown some efficacy.use of intramuscular botulinum toxin has shown some efficacy.

Extraocular muscle surgery should be postponed until the muscles Extraocular muscle surgery should be postponed until the muscles are no longer inflamed and the deviation has remained stable for at are no longer inflamed and the deviation has remained stable for at least 6 months.least 6 months.

Surgery Surgery STRABISMUS SURGERY:-Aim for maximal area of fusion without abnormal STRABISMUS SURGERY:-Aim for maximal area of fusion without abnormal

head posture.IR recession on adjustable +/- contra SR recessionhead posture.IR recession on adjustable +/- contra SR recessioniii) EYELID SURGERY:-iii) EYELID SURGERY:- Upper Lid retraction - Muller´s tenotomy (<2mm), levator Z myotomy or Upper Lid retraction - Muller´s tenotomy (<2mm), levator Z myotomy or

recession on hangback sutures, levator tenotomy +/- horns. recession on hangback sutures, levator tenotomy +/- horns. Lower Lid retraction - Usually needs a spacer from donor sclera (lid Lower Lid retraction - Usually needs a spacer from donor sclera (lid

retraction X 2 = amount of sclera required)retraction X 2 = amount of sclera required)

iv) BLEPHAROPLASTY for excess skin and fat iv) BLEPHAROPLASTY for excess skin and fat Ideally treatment combines a multidisciplinary coherent approach such as Ideally treatment combines a multidisciplinary coherent approach such as Combined radiotherapy and immunosuppression trialCombined radiotherapy and immunosuppression trial

INFLAMMATORY INFLAMMATORY CONDITIONSCONDITIONS

Orbital CellulitisOrbital Cellulitis

MyositisMyositis

Orbital ScleritisOrbital Scleritis

Inflammatory Lesion at the Apex of Inflammatory Lesion at the Apex of OrbitOrbit

Lymphoid InfiltrationLymphoid Infiltration

ORBITAL TUMORSORBITAL TUMORS

MucocoeleMucocoele

PseudotumorsPseudotumors

Dermoid CystDermoid Cyst

Dermoid CystDermoid Cyst

Capillary HaemangiomaCapillary Haemangioma

LymphangiomaLymphangioma

LymphangiomaLymphangioma

Optic Nerve GliomaOptic Nerve Glioma

Optic Nerve GliomaOptic Nerve Glioma

NeurofibromaNeurofibroma

NeurofibromaNeurofibroma

Optic Nerve MeningiomaOptic Nerve Meningioma(Rail Road Track Appearance RE)(Rail Road Track Appearance RE)

MeningiomaMeningioma

RhabdomyosarcomaRhabdomyosarcoma

RhabdomyosarcomaRhabdomyosarcoma

Mucoid TumorMucoid Tumor

Metastasis (CA Breast)Metastasis (CA Breast)

VASCULAR ANNOMALIESVASCULAR ANNOMALIES

Arterio-Venous MalformationArterio-Venous Malformation

VaricesVarices

Venous Malformation (Valsalva’s Venous Malformation (Valsalva’s ManeuverManeuver

VaricesVarices

Thank YouThank You

Thank YouThank You

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Orbital defects1