Haemangiomas And Vascular Malformations

Web Site: www.makboul.com Dr. Mohamed Makboul, MD

Vascular Vascular Anomalies;Anomalies;

Haemangiomas and Haemangiomas and Vascular MalformationsVascular Malformations

Dr. Mohamed Makboul, MDDr. Mohamed Makboul, MDPlastic Surgery DepartmentPlastic Surgery Department

Assiut UniversityAssiut University


The term The term hemangiomahemangioma 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 growth endothelium, and exhibits rapid postnatal growth and slow regression during childhood; this tumor and slow regression during childhood; this tumor

nevernever appears in an adolescent or adult. appears in an adolescent or adult. Whereas vascular malformations are comprised Whereas vascular malformations are comprised

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


Vascular anomalies can be classified into two Vascular anomalies can be classified into two unique groups:-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)


HaemangiomaHaemangioma


HaemangiomaHaemangioma

Hemangioma is the most Hemangioma is the most common tumor of infancy, common tumor of infancy, appears in neonatalhood, appears in neonatalhood, usually within the first 2 usually within the first 2 weeks and has a defined weeks and has a defined natural history. natural history.


Clinical Features Clinical Features

The natural history of Hemangioma is The natural history of Hemangioma is characterized by three phases:characterized by three phases:

Proliferating PhaseProliferating Phase Involuting PhaseInvoluting Phase Involuted PhaseInvoluted Phase


Proliferating PhaseProliferating Phase Hemangioma grows rapidly during the first 6 Hemangioma grows rapidly during the first 6

to 8 months of infancy, the skin becomes to 8 months of infancy, the skin becomes raised, bosselated, and a vivid crimson color. raised, bosselated, and a vivid crimson color.

Palpation at this stage reveals a tense and Palpation at this stage reveals a tense and noncompressible mass.noncompressible mass.


Involuting PhaseInvoluting Phase Hemangioma reaches its peak before the first year; Hemangioma reaches its peak before the first year;

and for a time thereafter, growth is proportionate to and for a time thereafter, growth is proportionate to that of the child. that of the child.

The first signs of the involuting phase appear as the The first signs of the involuting phase appear as the crimson color fades to a dull purplish color, the skin crimson color fades to a dull purplish color, the skin gradually pales, and the tumor feels less tense. gradually pales, and the tumor feels less tense.

The involuting phase continues until the child is 5 to The involuting phase continues until the child is 5 to 10 years of age. 10 years of age.


Involuted PhaseInvoluted Phase Regression is complete in 50% of children by age 5 Regression is complete in 50% of children by age 5

years and in 70% by age 7 years, with continued years and in 70% by age 7 years, with continued improvement until age 10 to 12 years. improvement until age 10 to 12 years.

The hemangioma may persist in the form of residual The hemangioma may persist in the form of residual tumor, loose skin, telangiectasias, or scarring. tumor, loose skin, telangiectasias, or scarring.


Hemangiomas can be Hemangiomas can be superficial, deep, or visceral in superficial, deep, or visceral in locationlocation Superficial lesions:Superficial lesions: often exhibit the classic crimson often exhibit the classic crimson

color of the so-called strawberry hemangioma. color of the so-called strawberry hemangioma. Deep lesions:Deep lesions: Are those within the deep dermis or Are those within the deep dermis or

subcutaneous tissues often present as pale blue or subcutaneous tissues often present as pale blue or purple masses that may be confused with venous purple masses that may be confused with venous

malformations.malformations. Visceral lesions:Visceral lesions: Are not apparent on physical Are not apparent on physical

examination, and in 50% of cases, there are no examination, and in 50% of cases, there are no accompanying cutaneous hemangiomas. In this accompanying cutaneous hemangiomas. In this

setting, their presence may only be suggested by setting, their presence may only be suggested by physiologic findings such as hepatomegaly, physiologic findings such as hepatomegaly,

congestive heart failure, or stridor.congestive heart failure, or stridor.


DIAGNOSTIC IMAGINGDIAGNOSTIC IMAGING

Hemangiomas are readily distinguished from Hemangiomas are readily distinguished from other tumors by:other tumors by: Ultrasonography (US)Ultrasonography (US) Computerized tomography (CT)Computerized tomography (CT) Magnetic resonance imaging (MRI)Magnetic resonance imaging (MRI) ArteriographyArteriography


DIFFERENTIAL DIFFERENTIAL DIAGNOSISDIAGNOSIS

Hemangiomas must be differentiated from:Hemangiomas must be differentiated from: Macular stains, Macular stains, Vascular malformations, and Vascular malformations, and Other vascular tumors of infancy.Other vascular tumors of infancy.


Macular StainsMacular Stains The most common type of vascular birthmark is the macular The most common type of vascular birthmark is the macular

stain. stain. These are flat lesions, ranging in color from pink to red, seen These are flat lesions, ranging in color from pink to red, seen

in as many as 40 percent of newborns. in as many as 40 percent of newborns. Typically seen in the neck, glabella, eyelid, and forehead, Typically seen in the neck, glabella, eyelid, and forehead,

these probably are physiologic phenomena that will resolve these probably are physiologic phenomena that will resolve with time.with time.


Vascular MalformationsVascular Malformations The natural history of vascular malformations is The natural history of vascular malformations is

distinct from hemangiomas. distinct from hemangiomas. Boys and girls are affected equally. Boys and girls are affected equally. All malformations are present at birth. All malformations are present at birth. The physical appearance of vascular malformations The physical appearance of vascular malformations

is dependent on the type of vessels involved. is dependent on the type of vessels involved. Venous malformations may appear as bluish masses Venous malformations may appear as bluish masses

and be confused with subcutaneous hemangiomas, and be confused with subcutaneous hemangiomas, but they are distinguished by the ability to be but they are distinguished by the ability to be emptied of blood with compression. emptied of blood with compression.


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

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

Hemangiomas, unlike vascular malformations, Hemangiomas, unlike vascular malformations, rarely cause bony distortion or hypertrophy.rarely cause bony distortion or hypertrophy.

Vascular Malformations Vascular Malformations (cont.)(cont.)


Other Vascular Tumors of Other Vascular Tumors of InfancyInfancy

Pyogenic granulomaPyogenic granuloma Is an acquired vascular lesion that closely resembles Is an acquired vascular lesion that closely resembles

hemangioma upon clinical and microscopic examination. hemangioma upon clinical and microscopic examination. They tend to occur on the skin and mucosa of older They tend to occur on the skin and mucosa of older

children and young adults, with a mean age of 6.7 years.children and young adults, with a mean age of 6.7 years. Pyogenic granulomas arise suddenly and usually without a Pyogenic granulomas arise suddenly and usually without a

history of trauma. history of trauma. Frequently located on the cheeks, eyelids, extremities.Frequently located on the cheeks, eyelids, extremities. The natural history is one of superficial ulceration and The natural history is one of superficial ulceration and

repetitive episodes of bleeding.repetitive episodes of bleeding.


COMPLICATIONSCOMPLICATIONS

UlcerationUlceration InfectionInfection

Visual ImpairmentVisual Impairment Airway ObstructionAirway Obstruction

Auditory Canal ObstructionAuditory Canal Obstruction Congestive Heart Failure : usually occurs in Congestive Heart Failure : usually occurs in

one of two settings: diffuse neonatal one of two settings: diffuse neonatal hemangiomatosis or large visceral hemangiomatosis or large visceral

hemangiomashemangiomas


MANAGEMENTMANAGEMENT

In most instances, parental education and reassurance In most instances, parental education and reassurance will suffice.will suffice.

Because most hemangiomas undergo spontaneous Because most hemangiomas undergo spontaneous involution, treatment may be reserved for those involution, treatment may be reserved for those lesions of functional or psychological concern. lesions of functional or psychological concern.

Hemangiomas obstructing the visual axis, airway, and Hemangiomas obstructing the visual axis, airway, and auditory canals, or those associated with congestive auditory canals, or those associated with congestive heart failure, ulceration, or bleeding, should be heart failure, ulceration, or bleeding, should be considered for treatment.considered for treatment.


A number of options are available for the A number of options are available for the treatment of hemangiomas. treatment of hemangiomas.

Effective treatments may be divided into: Effective treatments may be divided into: Those that are nonspecific (e.g., excision, Those that are nonspecific (e.g., excision,

cryotherapy); cryotherapy); Antiangiogenic (e.g., corticosteroids, interferon); Antiangiogenic (e.g., corticosteroids, interferon);

and and Antiproliferative (e.g., chemotherapy, radiation).Antiproliferative (e.g., chemotherapy, radiation).


Surgical ExcisionSurgical Excision

Surgical excision may be indicated for: Surgical excision may be indicated for: Infancy for obstructing effect, Infancy for obstructing effect, Ulcerating, or Ulcerating, or Large hemangiomas unresponsive to Large hemangiomas unresponsive to

pharmacologic therapy.pharmacologic therapy.


Capillary Malformation Capillary Malformation (CM)(CM)


CLINICAL PRESENTATIONCLINICAL PRESENTATION

Port wine stains are intradermal capillary or Port wine stains are intradermal capillary or venular malformations that are present at birth. venular malformations that are present at birth.


CM is a macular, red, CM is a macular, red, vascular stain that presents vascular stain that presents at birth and persists at birth and persists throughout life. throughout life.

CM can be localized or CM can be localized or extensive, on the face, extensive, on the face, trunk, or limbs. The trunk, or limbs. The incidence rate in newborns incidence rate in newborns is 0.3%. is 0.3%.


Two-thirds of these lesions darken in color Two-thirds of these lesions darken in color over time. over time.

Most CMs are harmless cutaneous birthmarks, Most CMs are harmless cutaneous birthmarks, but some are red flags that signal underlying but some are red flags that signal underlying abnormalities. abnormalities.

CM in a limb often is associated with axial and CM in a limb often is associated with axial and transverse hypertrophy.transverse hypertrophy.


TreatmentTreatment CM is treated by flashlamp pulsed-dye laser; CM is treated by flashlamp pulsed-dye laser;

the results are better if initiated in infancy and the results are better if initiated in infancy and childhood. childhood.

Soft-tissue and skeletal hypertrophy also can Soft-tissue and skeletal hypertrophy also can require surgical therapy. require surgical therapy.

Laser therapy cannot correct soft-tissue Laser therapy cannot correct soft-tissue overgrowth.overgrowth.


Laser treatment


Hypertrophy of the upper lip, treated by partial debulking


Venous Malformation Venous Malformation (VM)(VM)



Venous malformations are localized or extensive, Venous malformations are localized or extensive, minor or distorting, single or multiple, and located minor or distorting, single or multiple, and located anywhere on the head, limbs, or trunk. anywhere on the head, limbs, or trunk.

Most VMs are sporadic, blue color, soft, compressible Most VMs are sporadic, blue color, soft, compressible on palpation, a slow refill, and increased size with on palpation, a slow refill, and increased size with dependency are pathognomonic for venous dependency are pathognomonic for venous malformation.malformation.


DIAGNOSISDIAGNOSIS

Clinical picture.Clinical picture. MRIMRI


TREATMENTTREATMENT

Sclerotherapy is the primary treatment for Sclerotherapy is the primary treatment for VMs, although subsequent surgical resection is VMs, although subsequent surgical resection is often needed.often needed.

VM Upper lip,

Post Sclerotherapy and excision


VM Rt. Cheek, Post Sclerotherapy


Extensive venous malformation of the buttock and right thigh.Staged surgical reduction of the right buttock and thigh


Lymphatic Lymphatic Malformation (LM)Malformation (LM)



LMs can be described as LMs can be described as microcystic, macrocystic, and microcystic, macrocystic, and combined lymphaticovenous (LVM) combined lymphaticovenous (LVM) forms. forms.

Lymphatic anomalies present in a Lymphatic anomalies present in a wide spectrum, from lymphedema wide spectrum, from lymphedema to large cystic malformations. to large cystic malformations.

LMs are generally detected at birth LMs are generally detected at birth or shortly, thereafter LMs grow with or shortly, thereafter LMs grow with the child. the child.


Lymphatic malformations can affect all areas of the Lymphatic malformations can affect all areas of the body, but most are located in the cervicofacial region body, but most are located in the cervicofacial region and axilla. and axilla.

LMs are the most common cause of congenital LMs are the most common cause of congenital tongue enlargement (macroglossia), lip enlargement tongue enlargement (macroglossia), lip enlargement (macrocheilia), and ear enlargement (macrotia). (macrocheilia), and ear enlargement (macrotia).

Skeletal hypertrophy and distortion in 80% of Skeletal hypertrophy and distortion in 80% of cervicofacial LMs occur by 10 years of age.cervicofacial LMs occur by 10 years of age.

LM never regresses.LM never regresses.


TREATMENTTREATMENT Sclerotherapy has assumed a major role in the Sclerotherapy has assumed a major role in the

management of LM. management of LM. Surgical resection is the only way to Surgical resection is the only way to

potentially cure LM.potentially cure LM.


Large lymphatic malformation of the right lateral forehead and temple region.

After excision.


Arteriovenous Arteriovenous Malformation (AVM)Malformation (AVM)


AVM is a high-flow vascular malformation AVM is a high-flow vascular malformation comprised of micro- and macro-aneriovenous fistulas comprised of micro- and macro-aneriovenous fistulas (AVFs).(AVFs).

The epicenter of an AVM is called the The epicenter of an AVM is called the nidusnidus and and consists of arterial feeders, micro- and consists of arterial feeders, micro- and macroarteriovenous fistulas (AVFs), and ectatic veins.macroarteriovenous fistulas (AVFs), and ectatic veins.


Clinical Staging SystemClinical Staging System Introduced by SchobingerIntroduced by Schobinger Stage I (Quiescence): Stage I (Quiescence): Pink-blush stain, warmth, and AV Pink-blush stain, warmth, and AV

shunting by Doppler shunting by Doppler Stage II (Expansion):Stage II (Expansion): Same as stage I, plus enlargement, Same as stage I, plus enlargement,

pulsations, thrill, bruit, and tortuous/tense veinspulsations, thrill, bruit, and tortuous/tense veins Stage III (Destruction):Stage III (Destruction): Same as above, plus dystrophic Same as above, plus dystrophic

changes, ulceration, bleeding persistent pain, and expansion/ changes, ulceration, bleeding persistent pain, and expansion/ destructiondestruction

Stage IV (Decompensation):Stage IV (Decompensation): Same as stage II, plus cardiac Same as stage II, plus cardiac FailureFailure


DiagnosisDiagnosis Confirmation of clinical diagnosis is helpful Confirmation of clinical diagnosis is helpful

with Color Doppler examination, Magnetic with Color Doppler examination, Magnetic Resonance Imaging, and Angiography.Resonance Imaging, and Angiography.


TreatmentTreatment

Usually AVM is treated whenever endangering Usually AVM is treated whenever endangering signs and symptoms arise, such as ischemic signs and symptoms arise, such as ischemic pain, ulceration, bleeding, or increased cardiac pain, ulceration, bleeding, or increased cardiac output (Stages III and IVoutput (Stages III and IV). ).

Arterial embolization is done to Arterial embolization is done to temporarily temporarily occludeocclude the nidus in preparation for resection the nidus in preparation for resection 24 to 72 hours later.24 to 72 hours later.


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Haemangiomas And Vascular Malformations

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