VASCULAR ANOMALIESVASCULAR ANOMALIES

DR.ASHRAF HAMED PROFESSOR OF

PEDIATRIC SURGERYAL AZHAR

UNIVERSITY

INTRODUCTIONINTRODUCTIONvascular anomalies vascular anomalies can be broadly dividedcan be broadly divided into two groups: vascular tumors and vascular into two groups: vascular tumors and vascular

malformations.malformations. Vascular tumorsVascular tumors, of which the infantile , of which the infantile

hemangioma is the most common example, are true hemangioma is the most common example, are true neoplasms that arise from endothelial hyperplasia.neoplasms that arise from endothelial hyperplasia.

Conversely, Conversely, vascular malformations vascular malformations are congenital are congenital lesions of vascular dysmorphogenesis that arise lesions of vascular dysmorphogenesis that arise because of errors of embryonic development. These because of errors of embryonic development. These lesions exhibit normal endothelial cell turnover. lesions exhibit normal endothelial cell turnover.

The term The term hemangioma hemangioma refers to the common refers to the common tumor of infancy that has a proliferative tumor of infancy that has a proliferative endothelium, and exhibits rapid postnatal endothelium, and exhibits rapid postnatal growth and slow regression during childhood; growth and slow regression during childhood; this tumor this tumor nevernever appears in an adolescent or appears in an adolescent or adult. adult.

Whereas vascular malformations are Whereas vascular malformations are comprised of abnormally formed channels that comprised of abnormally formed channels that are lined by stable endothelium, present at are lined by stable endothelium, present at birth, never regress and often expand.birth, never regress and often expand.

Vascular anomalies can be Vascular anomalies can be classified into two unique groupsclassified into two unique groups

HemangiomasHemangiomas Vascular malformations which subcategories into :Vascular malformations which subcategories into :

Slow-flowSlow-flow CapillaryCapillary LymphaticLymphatic VenousVenous

Fast-flowFast-flow Arterial arterial (aneurysm, stenosis, ectasia)Arterial arterial (aneurysm, stenosis, ectasia) Arteriovenous fistulae (AVF)Arteriovenous fistulae (AVF) Arteriovenous malformation (AVM)Arteriovenous malformation (AVM)

CLASSIFICATION AND NOMENCLATURECLASSIFICATION AND NOMENCLATURE

VascularTumors

Slow-Flow VascularMalformations

Fast-Flow VascularMalformations

Combined VascularMalformations

1 -infantile Hemangioma

2-Congenital hemangioma

A-Rapidely involuting congenital hemangioma(RICH)

B-noninvoluting congenital hemangioma(NICH)

Capillarymalformation (CM)

Arteriovenousfistula (AVF)

Klippel-Trénaunay syndrome,a capillary lymphaticovenousmalformation (CLVM)

Kaposiformhemangioendothelioma

(KHE)

Venousmalformation (VM)

Arteriovenousmalformation

(AVM)

Parkes Weber syndrome, a capillaryarteriovenous malformation (CAVM)

Tufted angioma (TA) Lymphatic malformation (LM)Web Site: Web Site:

www.makboul.comwww.makboul.com

IntroductionIntroduction Virchow was the first to describe the histologic features Virchow was the first to describe the histologic features

of vascular nevi and initiated the term of vascular nevi and initiated the term angioma. The angioma. The term angioma became a base term term angioma became a base term used to describe all used to describe all such nevi regardless of natural history or other clinical such nevi regardless of natural history or other clinical features. He labeled the infantile hemangioma as features. He labeled the infantile hemangioma as “angioma simplex.”“angioma simplex.”

This same lesion has also been historically referred to This same lesion has also been historically referred to as “capillary hemangioma” and “strawberry as “capillary hemangioma” and “strawberry hemangioma.” Virchow’s “angioma cavernosum” actually hemangioma.” Virchow’s “angioma cavernosum” actually was used to label two distinct lesions, infantile was used to label two distinct lesions, infantile hemangiomas (when located deep to the skin) and VMs, hemangiomas (when located deep to the skin) and VMs, because they have similar appearances on physical because they have similar appearances on physical examination. examination.

IntroductionIntroduction Another example is Virchow’s designation of the Another example is Virchow’s designation of the

“angioma racemosum,” which referred to what is “angioma racemosum,” which referred to what is called today an AVM and which also has been called today an AVM and which also has been historically called an “arteriovenous hemangioma.”historically called an “arteriovenous hemangioma.”

Wegener, a student of Virchow, described the Wegener, a student of Virchow, described the histology of LMs, which he called “lymphangiomas.” histology of LMs, which he called “lymphangiomas.” The term The term cystic hygroma, referring to LMs, cystic hygroma, referring to LMs, unfortunately also unfortunately also continues to have common usage.continues to have common usage.

Thus, both the terms Thus, both the terms cystic hygroma and cystic hygroma and lymphangioma lymphangioma should be abandoned in favor of LM should be abandoned in favor of LM (macrocystic and microcystic, respectively(macrocystic and microcystic, respectively

Vascular tumors Vascular tumors HaemangiomaHaemangioma

Endothelial neoplasms Increased endothelial turnover •Hemangiomais the most common among vascular tumors –originates from vasculogenesis(Angiogenesis)

Infantile HemangiomasInfantile Hemangiomas(IH)(IH)

Hemangiomas are the most common tumor of infancy, Hemangiomas are the most common tumor of infancy, occurring in the skin in up to 4% - 10% of white infants, occurring in the skin in up to 4% - 10% of white infants, with a female-to-male ratio of 3 - 5:1.with a female-to-male ratio of 3 - 5:1.

The incidence may be significantly higher in premature The incidence may be significantly higher in premature infants 23%.infants 23%.

They are much more common in whites than dark-They are much more common in whites than dark-skinned individuals. skinned individuals.

Infantile hemangiomas have a unique and characteristic Infantile hemangiomas have a unique and characteristic life cycle of rapid growth in the first year of life life cycle of rapid growth in the first year of life (proliferative phase) followed by spontaneous slow (proliferative phase) followed by spontaneous slow regression from ages 1 to 7 years (involuting phase).regression from ages 1 to 7 years (involuting phase).

Once involuted, they never recur.Once involuted, they never recur.

PathophysiologyPathophysiology The proliferating phase of hemangiomas is characterized by The proliferating phase of hemangiomas is characterized by

angiogenesis in the tumor.angiogenesis in the tumor. The tumor is composed of plump, rapidly dividing The tumor is composed of plump, rapidly dividing

endothelial cells, forming a mass of sinusoidal vascular endothelial cells, forming a mass of sinusoidal vascular channels. Enlarged feeding arteries and draining veins channels. Enlarged feeding arteries and draining veins vascularize the tumor.vascularize the tumor.

In addition, a specific marker for endothelial cells of In addition, a specific marker for endothelial cells of hemangiomas that is not found in other vascular anomalies hemangiomas that is not found in other vascular anomalies is is GLUT-1 (an erythrocytetype glucose transporter).GLUT-1 (an erythrocytetype glucose transporter).

Proangiogenic factors, such as Proangiogenic factors, such as fibroblast growth factor fibroblast growth factor (FGF)(FGF) and and vascular endothelial growth factor (VEGF), lewis vascular endothelial growth factor (VEGF), lewis Y antigen Y antigen are prominent during the proliferative phase.are prominent during the proliferative phase.

Increased levels of these peptides may be found in the urine Increased levels of these peptides may be found in the urine of patients with hemangiomas.of patients with hemangiomas.

Infantile hemangiomaInfantile hemangioma

. a Prodromal phase. b Clinical findings after 1 month.

Clinical PresentationClinical Presentation The median age of appearance is 2 weeks The median age of appearance is 2 weeks

after birth ,however about 50% of IH are after birth ,however about 50% of IH are visible at birth by the presence of a fait visible at birth by the presence of a fait macule stain ,small pale spot or macule stain ,small pale spot or ecchymotic area.ecchymotic area.

Hemangiomas are most often single Hemangiomas are most often single cutaneous lesions and have an anatomic cutaneous lesions and have an anatomic predilection for the head and neck region predilection for the head and neck region (60%).(60%).

They occur in the trunk in 25% of cases They occur in the trunk in 25% of cases and on the extremities in 15% of cases. and on the extremities in 15% of cases.

Internal and visceral lesions are Internal and visceral lesions are uncommon.uncommon.

Up to 20% of patients can have multiple Up to 20% of patients can have multiple tumors.tumors.

Deep (a), mixed (b) and superficial nasal tip (c) Deep (a), mixed (b) and superficial nasal tip (c) hemangiomashemangiomas

Clinical PresentationClinical Presentation The proliferative phase The proliferative phase of hemangiomas is marked by rapid of hemangiomas is marked by rapid

growth, for the first 6 to 8 months, which typically plateaus by growth, for the first 6 to 8 months, which typically plateaus by age 10 to 12 months. Tumors that involve the superficial dermis age 10 to 12 months. Tumors that involve the superficial dermis are first seen as a red, raised lesion are first seen as a red, raised lesion (previously named (previously named “capillary” or “strawberry” hemangiomas“capillary” or “strawberry” hemangiomas;). ;).

Tumors in the lower dermis, subcutaneous tissue, or muscle Tumors in the lower dermis, subcutaneous tissue, or muscle appear bluish with slightly raised overlying skin and have appear bluish with slightly raised overlying skin and have frequently incorrectly been frequently incorrectly been called “cavernous” hemangiomascalled “cavernous” hemangiomas..

The involuting phase of hemangiomas occurs from age 1 to 7 The involuting phase of hemangiomas occurs from age 1 to 7 years, during which time the tumor slowly regresses. This years, during which time the tumor slowly regresses. This phase is notable for the fading color of the tumor to a dull phase is notable for the fading color of the tumor to a dull purple and the softening of the tumor mass. The skin usually purple and the softening of the tumor mass. The skin usually becomes pale in the center of the tumor first, spreading becomes pale in the center of the tumor first, spreading outward. Both the deep color and the bulk of the tumor show outward. Both the deep color and the bulk of the tumor show continued gradual improvement until the regression is entirely continued gradual improvement until the regression is entirely complete by age 10 to 12 years. complete by age 10 to 12 years.

Glut-1 positive stain in Glut-1 positive stain in hemangiomashemangiomas

Additionally, hemangiomas have a Additionally, hemangiomas have a unique vascular phenotype unique vascular phenotype demonstrated by glucose demonstrated by glucose transporter- 1 (GLUT-1) staining transporter- 1 (GLUT-1) staining (Fig)(Fig)

. Since its first description by . Since its first description by North in 2000, staining for GLUT- North in 2000, staining for GLUT- 1 has become widespread by 1 has become widespread by clinicians and researchers in the clinicians and researchers in the field of vascular anomalies.field of vascular anomalies.

Endothelial cells in RICH and Endothelial cells in RICH and other vascular tumors or other vascular tumors or malformations do not express malformations do not express GLUT-1 protein.GLUT-1 protein.

Posterior cervical hemangioma Posterior cervical hemangioma (left) has undergone (left) has undergone involution involution (right).(right).

ComplicationsComplications The primary local complications OF The primary local complications OF

cutaneous hemangiomas are ulceration, cutaneous hemangiomas are ulceration, bleeding, and pain.bleeding, and pain.

Lesions of the cervicofacial region may Lesions of the cervicofacial region may produce airway obstruction as they grow produce airway obstruction as they grow during the proliferative phase. during the proliferative phase.

Very large hemangiomas notably of the Very large hemangiomas notably of the liver, can lead to high-output congestive liver, can lead to high-output congestive heart failure secondary to fast flow and heart failure secondary to fast flow and vascular shunting within the tumor. vascular shunting within the tumor.

Facial lesions can result in tissue Facial lesions can result in tissue necrosis with cosmetic consequences necrosis with cosmetic consequences when involving the eyelid, nose, lip, or when involving the eyelid, nose, lip, or ear..ear..

ComplicationsComplicationsPeriorbital and eyelid lesions also may cause visual impairment or obstruction Distortion of the cornea can cause astigmatic amblyopia.

Subglottic hemangiomas may occur with stridor and lead to airway obstruction with continued growth. Gastrointestinal hemangiomas are very rare but can cause gastrointestinal bleeding.

Parotid Parotid HemangiomaHemangioma

Parotid emangioma is by far the most common tumor of the parotid gland in children.Current parotid hemangioma treatment regimens include propanolol, systemic or intralesional steroids and surgery.

Parotid hemangioma. a Huge hemangioma of Parotid hemangioma. a Huge hemangioma of the parotid gland. Clinically no airway the parotid gland. Clinically no airway

obstruction. b MRI shows deviation of the obstruction. b MRI shows deviation of the trachea but no compression. Complete trachea but no compression. Complete

regression without therapyregression without therapy

Other ManifestationsOther ManifestationsDisseminated neonatal Disseminated neonatal

hemangiomatosis (DNH).hemangiomatosis (DNH). Hemangiomatosis consists of Hemangiomatosis consists of

multiple disseminated multiple disseminated hemangiomas. The cutaneous hemangiomas. The cutaneous tumors are usually tiny (<5 tumors are usually tiny (<5 mm) and, when five or more mm) and, when five or more are present, visceral lesions are present, visceral lesions should be sought as well.should be sought as well.

Screening patients with Screening patients with ultrasonography or magnetic ultrasonography or magnetic resonance imagine (MRI) resonance imagine (MRI) may be indicated for these may be indicated for these patientspatients

Disseminated neonatal hemangiomatosis Disseminated neonatal hemangiomatosis (DNH).(DNH).

a, b The skin involvement does not reveal the severity of the disease. c-e Extended clinical investigation of abdomen and cranium is mandatory (e.g., CCDS shows diffuse hemangiomatosisin the liver)

“a Not all infantile hemangiomas regress spontaneously, some grow rapidly with complications, such as ulcerations. b Even in BNH liver hemangiomas can occur. c The CCDS shows the typical hyperperfusion in the hemangioma

Benign” neonatal hemangiomatosis (BNH).

Infantile hepatic Infantile hepatic hemangiomashemangiomas

Infantile hepatic hemangiomas must be differentiated from “hepatic hemangiomas” that are first seen in adulthood. Adult “hepatic hemangiomas,” which are sometimes called “cavernous hemangiomas,” are VMs. Hepatic hemangiomas of infancy are true tumors and have a pattern of involution similar to that of cutaneous hemangiomas. Contrary to popular belief, not all liver hemangiomas are life threatening. Indeed, the “classic triad” of heart failure, anemia, and hepatomegaly is unusual. Recently, it has been recognized that hepatic hemangiomas present in several different patterns: focal, multifocal, or diffuse.

hepatic hemangiomashepatic hemangiomas Focal lesions Focal lesions are single, generally large masses on are single, generally large masses on

antenatal imaging studies and/or present as a mass antenatal imaging studies and/or present as a mass in early infancy.They may cause moderate in early infancy.They may cause moderate thrombocytopenia that generally resolves thrombocytopenia that generally resolves spontaneously. spontaneously.

This is in contrast to the profound thrombocytopenia This is in contrast to the profound thrombocytopenia seen with Kasabach-Merritt phenomenon (KMP).seen with Kasabach-Merritt phenomenon (KMP).

These single large lesions often do not exhibit These single large lesions often do not exhibit growth after birth and are thought to represent RICH growth after birth and are thought to represent RICH lesions, which are histologically distinct from the lesions, which are histologically distinct from the typical hemangioma. typical hemangioma.

They are fully grown at birth and regress much faster They are fully grown at birth and regress much faster than the typical hemangioma.than the typical hemangioma.

Focal liver hemangiomaFocal liver hemangioma

hepatic hemangiomashepatic hemangiomas Multifocal hepatic hemangiomas Multifocal hepatic hemangiomas are histologically are histologically

identical to the typical cutaneous hemangioma. identical to the typical cutaneous hemangioma. Smaller enlarging lesions, detected by imaging Smaller enlarging lesions, detected by imaging stimulated by the presence of cutaneous stimulated by the presence of cutaneous hemangiomas, may often remain asymptomatic.hemangiomas, may often remain asymptomatic.

However, some will also have associated However, some will also have associated macrovascular shunts that may cause cardiac failure. macrovascular shunts that may cause cardiac failure.

These shunts may close with corticosteroid therapy, These shunts may close with corticosteroid therapy, but embolization may be necessary in those severe but embolization may be necessary in those severe cases in which there is insufficient time to wait for cases in which there is insufficient time to wait for involution.involution.

hepatic hemangiomashepatic hemangiomasMultifocal liver Multifocal liver hemangiomahemangioma

Diffuse liver hemangioma. Liver Diffuse liver hemangioma. Liver transplantationtransplantation

hepatic hemangiomashepatic hemangiomas The most dangerous hepatic hemangiomas are the The most dangerous hepatic hemangiomas are the diffuse diffuse

typetype, presenting with innumerable compacted nodular lesions , presenting with innumerable compacted nodular lesions replacing normal liver parenchyma, and causing massive replacing normal liver parenchyma, and causing massive hepatomegaly, abdominal compartment syndrome, and hepatomegaly, abdominal compartment syndrome, and respiratory compromise.respiratory compromise.

Massive hemangiomas Massive hemangiomas (e.g., those causing hepatomegaly) (e.g., those causing hepatomegaly) may induce profound may induce profound acquired hypothyroidism.acquired hypothyroidism. Hemangiomas Hemangiomas have been found to express type 3-iodothyronine deiodinase have been found to express type 3-iodothyronine deiodinase that inactivates circulating thyroid hormones.that inactivates circulating thyroid hormones.

Therefore, patients with large hemangiomas should be Therefore, patients with large hemangiomas should be screened by measuring thyroid-stimulating hormone levels. screened by measuring thyroid-stimulating hormone levels. When untreated, hypothyroidism in infancy will lead to severe When untreated, hypothyroidism in infancy will lead to severe mental retardation. Aggressive exogenous thyroid replacement mental retardation. Aggressive exogenous thyroid replacement and close endocrinologic consultation are mandated. The and close endocrinologic consultation are mandated. The condition is self-limiting after tumor involution.condition is self-limiting after tumor involution.

The differential diagnosis of The differential diagnosis of hepatic hemangiomashepatic hemangiomas

The differential diagnosis of hepatic The differential diagnosis of hepatic hemangiomas includes AVMs and malignant hemangiomas includes AVMs and malignant tumors such as hepatoblastoma and metastatic tumors such as hepatoblastoma and metastatic neuroblastoma. neuroblastoma.

The diagnosis is established by imaging with The diagnosis is established by imaging with ultrasonography, MRI, or computed ultrasonography, MRI, or computed tomography (CT). tomography (CT).

If typical imaging findings of one of the If typical imaging findings of one of the hemangioma patterns are not found, hemangioma patterns are not found, percutaneous biopsy may be indicated to percutaneous biopsy may be indicated to ensure a malignancy is not present.ensure a malignancy is not present.

ImagingImaging Ultrasonography and MRI are the principal useful Ultrasonography and MRI are the principal useful

modalities. Ultrasonography of proliferative phase modalities. Ultrasonography of proliferative phase hemangiomas demonstrates a mass with dense hemangiomas demonstrates a mass with dense parenchyma exhibiting parenchyma exhibiting fast-flow vascularity.fast-flow vascularity.

This distinguishes deep infantile hemangiomas from This distinguishes deep infantile hemangiomas from VMsVMs that exhibit that exhibit slow flow slow flow and larger blood-filled spaces. and larger blood-filled spaces.

MRI of proli ferating hemangiomas shows a lobulated solid MRI of proli ferating hemangiomas shows a lobulated solid mass of intermediate intensity with T1-weighted spin-echo mass of intermediate intensity with T1-weighted spin-echo sequences and moderate hyperintensity on T2-weighted sequences and moderate hyperintensity on T2-weighted spin-echo images. spin-echo images.

For the involuting phase, MRI demonstrates decreased For the involuting phase, MRI demonstrates decreased flow voids and vascularity with the mass, taking on a more flow voids and vascularity with the mass, taking on a more lobular and fatty appearance.lobular and fatty appearance.

TreatmentTreatment

Observation is the mainsty of Observation is the mainsty of management since 90% of IH are management since 90% of IH are small ,localized and do not invovle small ,localized and do not invovle vital structures.vital structures.

Because hemangiomas are tumors of Because hemangiomas are tumors of pure angiogenesis, pharmacologic pure angiogenesis, pharmacologic therapy involves angiogenesis therapy involves angiogenesis inhibition. inhibition.

TreatmentTreatment The first-line antiangiogenic therapy for The first-line antiangiogenic therapy for

hemangiomas exhibiting appropriate risk hemangiomas exhibiting appropriate risk factors or complications is systemic factors or complications is systemic corticosteroids.corticosteroids.

Oral prednisone is used at a dose of 2 to 3 Oral prednisone is used at a dose of 2 to 3 mg/kg/day. Doses up to 5 mg/kg/day have mg/kg/day. Doses up to 5 mg/kg/day have been administered for life-threatening been administered for life-threatening complications with large hemangiomas complications with large hemangiomas causing airway obstruction or heart failure.causing airway obstruction or heart failure.

The overall response rate is 80% to 90%.The overall response rate is 80% to 90%.

Intralesional corticosteroidsIntralesional corticosteroids Intralesional corticosteroids are used for hemangiomas that Intralesional corticosteroids are used for hemangiomas that

cause local deformity or ulceration, especially for facial lesions cause local deformity or ulceration, especially for facial lesions of the eyelid, nose, cheek, or lip.of the eyelid, nose, cheek, or lip.

A total of three to five injections (at a dose of 3 to 5 mg/kg per A total of three to five injections (at a dose of 3 to 5 mg/kg per injection) are typically given at intervals of 6 to 8 weeks. injection) are typically given at intervals of 6 to 8 weeks.

The response rate approaches that of systemic corticosteroids. The response rate approaches that of systemic corticosteroids. Subcutaneous atrophy is a potential complication of Subcutaneous atrophy is a potential complication of

corticosteroid injection, but it is usually temporary. corticosteroid injection, but it is usually temporary. Cases have been reported of blindness after Cases have been reported of blindness after intralesional intralesional

corticosteroid injection for periorbital hemangiomas. corticosteroid injection for periorbital hemangiomas. This has This has been presumed to be secondary to particle embolization of the been presumed to be secondary to particle embolization of the retinal artery through feeding vessels. Manual compression retinal artery through feeding vessels. Manual compression around the periphery of the tumor is recommended during around the periphery of the tumor is recommended during injection.injection.

TreatmentTreatment Recombinant Recombinant interferon interferon αα 2a& 2b 2a& 2b, ,

previously considered as a second-line previously considered as a second-line agent, has fallen out of favor, except in agent, has fallen out of favor, except in very limited circumstances, because of very limited circumstances, because of the severe potential complication of the severe potential complication of spastic diplegia that can occur in 5% to spastic diplegia that can occur in 5% to 12% of treated infants.12% of treated infants.

On the other hand, experience is On the other hand, experience is growing with low-dose, high-frequency growing with low-dose, high-frequency antiangiogenic regimens of vincristine antiangiogenic regimens of vincristine as second-line after corticosteroids. as second-line after corticosteroids.

A recent report of propranolol used to A recent report of propranolol used to treat infantile hemangioma may indeed treat infantile hemangioma may indeed revolutionize medical therapy.revolutionize medical therapy.

PROPRANOLOL(INDERAL )PROPRANOLOL(INDERAL ) IT IS NON SELECTIVE IT IS NON SELECTIVE ββ BLOCKER. BLOCKER. It may be efficacious as corticosteriod for It may be efficacious as corticosteriod for

treatment of problematic IH.treatment of problematic IH. MODE OF ACTION;unkeownMODE OF ACTION;unkeown Theories include:Theories include: Vasoconstriction.Vasoconstriction. Dowen regulation of angiogenic proteins Dowen regulation of angiogenic proteins

such as such as VEFG & bFGFVEFG & bFGF.. DOSE:2-3mg/kg/dail for 12-18 month.DOSE:2-3mg/kg/dail for 12-18 month.

laser therapylaser therapy Although it has popular appeal, laser therapy is not often Although it has popular appeal, laser therapy is not often

beneficial for infantile hemangiomas. The flashlamp beneficial for infantile hemangiomas. The flashlamp pulse-dye laser penetrates the dermis to a depth of only pulse-dye laser penetrates the dermis to a depth of only 0.75 to 1.2 mm. Most cutaneous hemangiomas are 0.75 to 1.2 mm. Most cutaneous hemangiomas are deeper and therefore not affected by laser treatment.deeper and therefore not affected by laser treatment.

Also, the use of an endoscopic continuous-wave CO2 Also, the use of an endoscopic continuous-wave CO2 laser has been shown to be a good technique for laser has been shown to be a good technique for controlling proliferative-phase hemangiomas in the controlling proliferative-phase hemangiomas in the unilateral subglottic location.unilateral subglottic location.

Finally, intralesional photocoagulation with (Nd:YAG) Finally, intralesional photocoagulation with (Nd:YAG) laser can be useful for hemangiomas in certain locations, laser can be useful for hemangiomas in certain locations, such as the upper eyelid, when visual obstruction is a such as the upper eyelid, when visual obstruction is a concern.concern.

Indications for surgical resection of infantile Indications for surgical resection of infantile hemangiomashemangiomas

well-localized or pedunculated tumors can be expeditiously well-localized or pedunculated tumors can be expeditiously resected with linear closure, especially for tumors complicated resected with linear closure, especially for tumors complicated by bleeding and ulceration. Sites that are most amenable to by bleeding and ulceration. Sites that are most amenable to resection in this stage are the scalp, trunk, and extremities.resection in this stage are the scalp, trunk, and extremities.

Tumors of the upper eyelid that obstruct vision and that do not Tumors of the upper eyelid that obstruct vision and that do not respond to pharmacologic therapy also may require surgical respond to pharmacologic therapy also may require surgical excision or debulking. Focal lesions of the gastrointestinal tract excision or debulking. Focal lesions of the gastrointestinal tract that cause bleeding and for which medical management fails that cause bleeding and for which medical management fails also may require resection by means of enterotomy or also may require resection by means of enterotomy or endoscopic band ligation.endoscopic band ligation.

Finally, for th difficult-to-treat and life-threatening large Finally, for th difficult-to-treat and life-threatening large hemangiomas, especially in the liver, angiographic embolization hemangiomas, especially in the liver, angiographic embolization may be required to manage high-output cardiac failure.may be required to manage high-output cardiac failure.

Indications for surgical Indications for surgical resection of infantile resection of infantile

hemangiomashemangiomas

Congenital hemangiomasCongenital hemangiomasClinical PresentationClinical Presentation

Rare congenital hemangiomas are fully developed at birth and do not usually exhibit postnatal tumor growth. These are the rapidly involuting congenital hemangioma (RICH) and the noninvoluting congenital hemangioma (NICH).

Rapid involuting congenital Rapid involuting congenital hemangioendothelioma (RICH)hemangioendothelioma (RICH)

. a Clinical picture at birth. b Clinical picture at 6 months. the CCDS shows typical signs. The result of spontaneous healing is, as in all other congenital hemangioendotheliomas, a chalasia of the skin and an atrophy of subcutis and fascia

This tumor is totally matured prenatally (“prenatalmature hemangioma”) and should be clearly differentiatedfrom an infantile hemangioma because it isnegative for GLUT-1.

Rapidly involuting Rapidly involuting congenital hemangioma congenital hemangioma

(RICH)(RICH)

The Non-involuting Congenital HemangioendotheliomaThe Non-involuting Congenital Hemangioendothelioma(NICH)(NICH)

The skin above is not directly infiltrated, but often shows a light bluish change in coloring along with a more pronounced telangiectasia.In some cases, there is spontaneous regression, recognizable in the sonography by an increasing fibrosis and a decrease in vascularization, and similar to RICH, a remainder of a chalasia of the cutis and an atrophy of the subcutaneous fat.

Giant non involuting congenital hemangioma in the thigh with functional gait impairment. a Excision by lenticular incision and linear closure technique. b Postoperative results

On the other hand, as long as NICH is still active, transition to a Kaposi-like hemangioendothelioma is possible at any time with the formation of a Kasabach-Merritt syndrome. Therefore, in all NICH patients, regular control of thrombocytes and, if necessary, clotting parameters is mandatory

The Non-involuting Congenital Hemangioendothelioma

(NICH)

Tufted Angioma(TA) and KaposiformTufted Angioma(TA) and KaposiformHemangioendothelioma(KHE )Hemangioendothelioma(KHE )

Both tumors typically present at birth, although they occur postnatally as well. The tumors are unifocal and are most often located on the trunk, shoulder, thigh, or retroperitoneum.TA appears as erythematous macules or plaques, and Histology reveals small tufts of capillaries. KHE is a more extensive tumor with deep red-purple skin discoloration and overlying and surrounding ecchymosis. Generalized petechiae may be appear with profound thrombocytopenia secondary to the Kasabach- Merritt Phenomenon (KMP).

Tufted Angioma(TA) and Tufted Angioma(TA) and KaposiformKaposiform

Hemangioendothelioma(KHE )Hemangioendothelioma(KHE )Imaging of KHE depicts an enhancing lesion with poorly defined margins that extend across tissue planes. This is contrasted to hemangiomas, which are well circumscribed and respective of tissue planes. Biopsy :reveals infiltrating sheets of slender endothelial cells.

A&B Early onset of a kaposiform hemangioendothelioma. c CCDS shows interstitial hypersonoric areas with palisade like vessels which are pathognomonic for congenital hemangioendothelioma

The natural history of these tumors is one of continued proliferation into early childhood followed by subsequent but incomplete regression. Unlike IH endothelial cells do not express GLUT-1 nor Lewis Y antigen.

kaposiform kaposiform hemangioendotheliomahemangioendothelioma

Early transition of NICH into kaposiform hemangioendothelioma. In spite of massive swelling with local pain (wasp sting syndrome), there are no general symptoms of disease, but continuously decreasing thrombocytes and normal clotting parameters

Kasabach-Merritt PhenomenonKasabach-Merritt Phenomenon KMPKMP was first reported in 1940 as a case of profound was first reported in 1940 as a case of profound

thrombocytopenia, petechiae, and bleeding in conjunction thrombocytopenia, petechiae, and bleeding in conjunction with a with a “giant hemangioma.“giant hemangioma.

The only known The only known true associations are with TA and KHE. true associations are with TA and KHE. The platelet count with this disorder is typically less than The platelet count with this disorder is typically less than 10,000/mm3 and may be accompanied by decreased 10,000/mm3 and may be accompanied by decreased fibrinogen levels and a mildly elevated (PT) and (PTT). fibrinogen levels and a mildly elevated (PT) and (PTT).

Treatment of KHE with KMP is primarily medical, because Treatment of KHE with KMP is primarily medical, because the tumor is usually too large and extensive to be resected. the tumor is usually too large and extensive to be resected. Corticosteroids and interferon alfa have been effective in Corticosteroids and interferon alfa have been effective in about 50% of cases. Vincristine also is beneficial, but no about 50% of cases. Vincristine also is beneficial, but no single agent has been shown to be consistently successful. single agent has been shown to be consistently successful. Platelet transfusions are ineffective and should be avoided Platelet transfusions are ineffective and should be avoided unless active bleeding occurs. Mortality rates with KMP unless active bleeding occurs. Mortality rates with KMP and KHE or TA remain high at 20% to 30%. and KHE or TA remain high at 20% to 30%.

Other Vascular Tumors of InfancyOther Vascular Tumors of InfancyPyogenic granuloma Is an acquired vascular lesion that closely resembles hemangioma upon clinical and microscopic examination. They tend to occur on the skin and mucosa of older children and young adults, with a mean age of 6.7 years.Pyogenic granulomas arise suddenly and usually without a history of trauma. Frequently located on the cheeks, eyelids, extremities. The natural history is one of superficial ulceration and repetitive episodes of bleeding.

Vascular Malformations Vascular Malformations Vascular malformations are congenital lesions of Vascular malformations are congenital lesions of

vascular dysmorphogenesis that can be local or diffuse. vascular dysmorphogenesis that can be local or diffuse. The prevalence of The prevalence of Vascular Malformations :1.2-1.5%.Vascular Malformations :1.2-1.5%. They are classified, based on the appearance of the They are classified, based on the appearance of the

abnormal channels, as resembling capillaries, abnormal channels, as resembling capillaries, lymphatics, veins, arteries, or a combination thereof.lymphatics, veins, arteries, or a combination thereof.

Malformations grow with the child and do not undergo Malformations grow with the child and do not undergo the rapid proliferative growth phase exhibited by the rapid proliferative growth phase exhibited by hemangiomas. hemangiomas.

The greatest distinction between hemangiomas and The greatest distinction between hemangiomas and malformations is that the former spontaneously malformations is that the former spontaneously involute and the latter do not.involute and the latter do not.

Capillary malformationCapillary malformation

Equal gender distributionBirth prevalence is 0.3%

Capillary malformations (CMs), which have been previously referred to as “port-wine stains,” are present at birth as permanent, flat, pink-red cutaneous lesions

Capillary Capillary Malformations(CMs) Malformations(CMs)

The histology of The histology of cutaneous CMs consists of cutaneous CMs consists of dilated capillary- to dilated capillary- to venule-size vessels located venule-size vessels located in the superficial dermis. in the superficial dermis. These abnormal vessels These abnormal vessels gradually dilate over time, gradually dilate over time, leading to a darker color leading to a darker color and,occasionally, nodular and,occasionally, nodular ectasias.ectasias.

Clinical presentationClinical presentation At birth:Well-circumscribed At birth:Well-circumscribed

red macular lesionsred macular lesions Commonly occur on the face Commonly occur on the face

in the trigerminal in the trigerminal distribution.distribution.

45% restricted to 1/3 45% restricted to 1/3 trigeminal dermatomes trigeminal dermatomes

••55% overlap dermatomes, 55% overlap dermatomes, cross the midline, bilaterally.cross the midline, bilaterally.

Clinical presentationClinical presentation

Clinical presentationClinical presentation CMs can be CMs can be

associated with associated with underlying soft tissue underlying soft tissue and skeletal and skeletal overgrowth as well overgrowth as well as other internal as other internal abnormalities.abnormalities.

CMs of the occiput CMs of the occiput can signal underlying can signal underlying encephalocele or encephalocele or ectopic meninges.ectopic meninges.

Sturge-Weber syndromeSturge-Weber syndrome Facial capillary malformaionFacial capillary malformaion Ipsilateral occular and Ipsilateral occular and

leptomeningeal vascular leptomeningeal vascular anomaliesanomalies

Neurologic : seizures , Neurologic : seizures , hemiparesis , delayed motor hemiparesis , delayed motor and cognitive developmentand cognitive development

Ophthalmologic: Ophthalmologic: glaucoma&retinal detachement glaucoma&retinal detachement

MRI reveals the central nervous MRI reveals the central nervous system abnormalities, showing system abnormalities, showing pial vascular enhancement and pial vascular enhancement and gyriform calcificationsgyriform calcifications

Sturge-Weber syndromeSturge-Weber syndrome Port wine stain (PWS). Port wine stain (PWS).

Sturge-Weber Sturge-Weber syndrome.syndrome.

Capillary Capillary malformation. malformation. Therapy is dye-laser Therapy is dye-laser or Vasculight (IPL)or Vasculight (IPL)

Sturge-Weber syndromeSturge-Weber syndrome

MANAGEMENTMANAGEMENTFlash lamp pulsed-dye laserFlash lamp pulsed-dye laser

Before laserBefore laser

After laserAfter laser

the results are better if initiated in infancy and childhood

Lymphatic malformationLymphatic malformationLymphatic MalformationsEmbryogenic disturbance of lymphatic system At birth -2 yrsNo involutionMicrocystic malformation (lymphagiomas)Macrocystic (cystic hygroma)Combined

Rich lymphatic Rich lymphatic area area : :

H&NH&NAxillaAxillaMediastinumMediastinumgroin groin retroperitoneumretroperitoneum

lymphangioma cutislymphangioma cutisInvolvement of the dermis (lymphangioma cutis) may produce puckering of the skin or vesicles that weep clear yellowish fluid .

Gorham’s syndrome, Gorham’s syndrome, “disappearing bone “disappearing bone

disease.”disease.” LMs of the extremities are seen in LMs of the extremities are seen in

conjunction with overgrowth and limb-length conjunction with overgrowth and limb-length discrepancy. discrepancy.

A rare but very difficult problem arises with A rare but very difficult problem arises with Gorham’ syndrome, in which soft tissue and Gorham’ syndrome, in which soft tissue and skeletal LMs lead to progressive osteolysis skeletal LMs lead to progressive osteolysis and “disappearing bone disease.” Pathologic and “disappearing bone disease.” Pathologic fractures and vertebral instability can become fractures and vertebral instability can become manifest with this often fatal syndrome.manifest with this often fatal syndrome.

Facial soft tissue distortionFacial soft tissue distortionBony hypertrophyBony hypertrophy

Periorbital LMs can lead to proptosis. Facial LMs can cause the associated deformities of macrocheilia, macroglossia, and macromala

EXITEXIT

Large Large cervicofacial cervicofacial LMs with airway LMs with airway obstruction------obstruction------ex utero ex utero intrapartum intrapartum treatment.treatment.

LymphedemaLymphedema

Lymphedema is a type of LMsLymphedema is a type of LMs Milroys disease:congenital form of Milroys disease:congenital form of

lymphedemalymphedema Meige disease:pubertal onset of lymphedemaMeige disease:pubertal onset of lymphedema

ImagingImagingWell-localized and cystic LMs are easily characterized by ultrasonography and CT. MRI, however, provides the most reliable diagnosis and is superior in documenting the full extent of more complexLMs as well as their macrocystic and microcystic components.

Tracheostomy may be needed in cases of oropharyngeal and airway obstruction.

ManagementManagementSurgical resection provides the only method for potential “cure,” but this is possible only for lesions that are well localized. Focal and macrocystic lesions are amenable to ablation by both sclerotherapy and resection.• In contrast, more diffuse and predominantly microcystic LMs are difficult to eradicate by any method. •Intralesional sclerotherapy is most beneficial for LMs with macrocystic components. The commonly used agents are ethanol, sodium tetradecyl sulfate, and doxycycline that produce scarring and collapse of the cysts.

VENOUS MALFORMATIONSVENOUS MALFORMATIONSPathophysiologyPathophysiology

usually manifest by childhood or early adulthood.•grow with the developing child. sometimes are not obvious at birth•do not regress.•"slow-flow" lesions •can expand in response to–trauma, –incomplete surgical resection–altered hormonal states (pregnancy, puberty, steroid use). –thrombosis or in sepsis.

Presentation Presentation VMs, often incorrectly called VMs, often incorrectly called

““cavernous hemangiomascavernous hemangiomas,” are ,” are consisting of venous channels that consisting of venous channels that can develop anywhere in the body.can develop anywhere in the body.

VMs are probably the most common VMs are probably the most common of the vascular malformations, and of the vascular malformations, and they are more likely to be multiple.they are more likely to be multiple.

VMs of the gastrointestinal tract are VMs of the gastrointestinal tract are often multiple and can affect every often multiple and can affect every part of the GIT.part of the GIT.

On examinationOn examination these soft, bluish, compressible these soft, bluish, compressible

lesions will expand with dependent lesions will expand with dependent position and Valsalva maneuver.position and Valsalva maneuver.

Blue rubber bleb nevus syndromeBlue rubber bleb nevus syndrome BRBNS BRBNS

Presentation :BRBNS is a venous malformation, formerly, incorrectly, thought to be related to the hemangioma. It carries significant potential for serious bleeding. Lesions are most commonly found on the skin and in the small intestine and distal large bowel. It usually presents soon after birth.

Blue rubber bleb nevus syndromeBlue rubber bleb nevus syndrome(or Bean’s syndrome) represents a specific (or Bean’s syndrome) represents a specific rare disorder consisting of multifocal VMs rare disorder consisting of multifocal VMs

that affect the skin and gastrointestinal tract that affect the skin and gastrointestinal tract primarilyprimarily

Multiple scattered blue to black rubbery Multiple scattered blue to black rubbery papules and nodules involving the mid-papules and nodules involving the mid-chest regionchest region

Purple to blue/black Purple to blue/black papules involving the papules involving the upper and lower lipsupper and lower lips

ImagingImagingRadiologic modalities useful for the diagnosis of VMs include ultrasonography, MRI, and venography.MRI is most informative and demonstrates hyperintense lesions with T2-weighted sequences.

ManagementManagement

Indication : appearance, functional,painIndication : appearance, functional,pain Conservative therapy: elastic Conservative therapy: elastic

compression +baby aspirin (prophylaxis compression +baby aspirin (prophylaxis painful thromboses)painful thromboses)

Sclerotherapy : main treatmentSclerotherapy : main treatment Laser (Nd:YAG)Laser (Nd:YAG) ResectionResection

Sclerotherapy is the primary treatment Sclerotherapy is the primary treatment for VMs, although subsequent surgical for VMs, although subsequent surgical

resection is often needed.resection is often needed.

VM Upper lip, Post Sclerotherapy and excision

Maffucci SyndromeMaffucci SyndromeMaffucci syndrome consists of VMs of the soft tissue and bones, bony exostoses, and endochondromas. The bony lesions and endochondromas manifest first in childhood, and the venous anomalies appear later. The endochondromas can undergo malignant transformation in 20% to 30% of cases, leading to chondrosarcomas.

ARTERIOVENOUS MALFORMATIONSARTERIOVENOUS MALFORMATIONS

AVMs are fast-flow vascular malformations characterized by abnormal connections or shunts between feeding arteries and draining veins without an intervening capillary bed. AVMs are familiar to neurosurgeon s as one of the more common vascular anomalies that occur in the central nervous system. Indeed, intracranial AVMs are more frequent than AVMs of the skin and soft tissues within the head and neck region.

EPIDEMIOLOGYEPIDEMIOLOGY•Incidence 1.3 : 100,000•Intracranial : Extracranial–20 : 1

Head and neck ,extremity, truncal, and visceral sites•The majority of patients (40-60%)present at birth

Equal gender distribution

Arteriovenous MalformationArteriovenous MalformationErrors of embryogenic vascular developmentFailure of arteriovenous channels in the primitive retiform plexus to regress

CLINICALThe pink-bluish stain of the AVM is usually noted at birthtrauma seem to trigger expansionLocal warmth , Palpable thrill , Audible bruitHeadache , seizure in Intracranial AVM

The epicenter of anAVM is called the nidus and consists of arterial feeders

Schobinger Clinical Staging System Schobinger Clinical Staging System For Arteriovenou For Arteriovenou

MalformationsMalformations

Stage Clinical FindingsI (Quiescent) Pink to bluish stain, cutaneous warmth, and arteriovenous shunting by Doppler ultrasound imagingII (Expanding) Same as stage I, plus enlargement, pulsation, thrill, bruit, and tortuous and tense veinsIII (Destructive) Same as stage II, plus skin ulceration, bleeding, persistent pain, or tissue necrosisIV (Decompensating) Same as stage III, plus cardiac failure

ImagingImagingUltrasonography and Doppler imaging are excellenttools to elucidate the fast flow of these lesions and todistinguish them from VMs. MRI and MR angiography are the most useful toolsto demonstrate the full extent of these lesions.

AVM Angiography

ManagementManagementThe mainstays of treatment for these lesions are angiographic embolization alone or in combination with surgical excision.

AVMs are usually treated when there are endangering signs and symptoms( such as ischemic pain, ulceration, bleeding) and increased cardiac output.Direct puncture sclerotherapy of the AVM nidus can be a useful adjunct to embolization, especially when the feeding arteries are too tortuous.

COMBINED VASCULAR COMBINED VASCULAR MALFORMATIONSMALFORMATIONS

Klippel-Trénaunay syndrome (CLVM) Klippel-Trénaunay syndrome (CLVM) Klippel-Trénaunay syndrome is a slow-flow combined vascular malformation involving abnormal capillaries, lymphatics, and veins. This capillary lymphaticovenousmalformation (CLVM) usually involves one or more extremities, most often a lower limb, and is associated with prominent soft tissue and bony hypertrophy.

Klippel-Trénaunay Klippel-Trénaunay syndromesyndrome (CLVM) (CLVM) Thrombophlebitis of the anomalous veins occurs

with a frequency of 20% to 45%, and pulmonary emboli are reported in 4% to 25% of cases.Plain radiographs are used to document limb-length discrepancies serially. MRI provides the foundation for describing the type and extent of each of the vascular malformation components. MR venography can elucidate the anatomy of the deep system veins. Identification of a subcutaneous vein coursing along the lateral calf and thigh (the marginal vein of Servelle) is pathognomonic For Klippel-Trénaunay syndrome. Treatment for Klippel-Trénaunay syndrome is conservative, because it is not a curable disease.

(Parkes-Weber (Parkes-Weber Syndrome)Syndrome)

2 year old girl.Nevus, phlebectasy,hypertrophy (Parkes Weber syndrome).

Parkes Weber syndrome (CAVM )is characterized by the presence of a confluent or patchy CM with an underlying complex AVM that permeates an extremityCAVM is obvious at birth, appearing as overgrowth with a large geographic macular pink stain. MRI demonstrates symmetric muscular and bony overgrowth, with generalized enlargement of the normal named arteries and veins within the affected limb.Angiography depicts the discrete AVFs. In rare cases, superselective embolization is used to occlude the arteriovenous shunts if symptoms of ischemia, pain, or high-output congestive heart failure occur.

Proteus SyndromeProteus SyndromeProteus syndrome causes an overgrowth of skin, bones, muscles, fatty tissues, and blood and lymphatic vessels.Tumors of skin and bone growths appear as they age. The severity and locations of these various asymmetrical growths vary greatly but typically the skull, one or more limbs, and soles of the feet will be affected. There is a risk of premature death in affected individuals due to deep vein thrombosis and pulmonary embolism caused by the vessel malformations that are associated with this disorder.

Bannayan-Riley-Ruvalcaba Bannayan-Riley-Ruvalcaba SyndromeSyndrome

Bannayan–Riley–Ruvalcaba syndrome (BRRS) is a rare overgrowth syndrome 

Typically, cutaneous CMs, VMs, or AVMs are found. The more prominent clinical features are macrocephaly,multiple lipomas, hamartomatous polypsof the ileum and colon, and Hashimoto’s thyroiditis.

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