Vascular anomalies ashraf

VASCULAR ANOMALIESVASCULAR ANOMALIES
DR.ASHRAF HAMED
PROFESSOR OF
PEDIATRIC SURGERY
AL AZHAR UNIVERSITY

INTRODUCTIONINTRODUCTION
vascular anomaliesvascular anomalies can be broadly dividedcan be broadly divided
into two groups: vascular tumors and vascularinto 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, arehemangioma is the most common example, are
true neoplasms that arise from endothelialtrue neoplasms that arise from endothelial
hyperplasia.hyperplasia.
 Conversely,Conversely, vascular malformationsvascular malformations areare
congenital lesions of vascular dysmorphogenesiscongenital lesions of vascular dysmorphogenesis
that arise because of errors of embryonicthat arise because of errors of embryonic
development. These lesions exhibit normaldevelopment. These lesions exhibit normal
endothelial cell turnover.endothelial cell turnover.

 The termThe term hemangiomahemangioma refers to the commonrefers to the common
tumor of infancy that has a proliferativetumor of infancy that has a proliferative
endothelium, and exhibits rapid postnatalendothelium, and exhibits rapid postnatal
growth and slow regression during childhood;growth and slow regression during childhood;
this tumorthis tumor nevernever appears in an adolescent orappears in an adolescent or
adult.adult.
 Whereas vascular malformations areWhereas vascular malformations are
comprised of abnormally formed channels thatcomprised of abnormally formed channels that
are lined by stable endothelium, present atare lined by stable endothelium, present at
birth, never regress and often expand.birth, never regress and often expand.

Vascular anomalies can beVascular anomalies can be
classified into two unique groupsclassified into two unique groups
 HemangiomasHemangiomas
 Vascular malformations which subcategoriesVascular malformations which subcategories
into :into :
 Slow-flowSlow-flow
 CapillaryCapillary
 LymphaticLymphatic
 VenousVenous
 Fast-flowFast-flow
 Arterial arterial (aneurysm, stenosis,Arterial arterial (aneurysm, stenosis,
ectasia)ectasia)
 Arteriovenous fistulae (AVF)Arteriovenous fistulae (AVF)


CLASSIFICATION AND NOMENCLATURECLASSIFICATION AND NOMENCLATURE
Vascular
Tumors
Slow-Flow Vascular
Malformations
Fast-Flow
Vascular
Malformations
Combined Vascular
Malformations
1-infantile
Hemangioma
2-Congenital hemangioma
A-Rapidely involuting
congenital
hemangioma(RICH(
B-noninvoluting
congenital
hemangioma(NICH(
Capillary
malformation (CM(
Arteriovenous
fistula (AVF(
Klippel-Trénaunay
syndrome,
a capillary
lymphaticovenous
malformation (CLVM(
Kaposiform
hemangioendothelioma
)KHE(
Venous
malformation (VM(
Arteriovenous
malformation
)AVM(
Parkes Weber syndrome,
a capillary
arteriovenous
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 ofVirchow was the first to describe the histologic features of
vascular nevi and initiated the termvascular nevi and initiated the term angioma. The termangioma. The term
angioma became a base termangioma became a base term used to describe all such neviused to describe all such nevi
regardless of natural history or other clinical features. Heregardless of natural history or other clinical features. He
labeled the infantile hemangioma as “angioma simplex.”labeled the infantile hemangioma as “angioma simplex.”
 This same lesion has also been historically referred to asThis same lesion has also been historically referred to as
“capillary hemangioma” and “strawberry hemangioma.”“capillary hemangioma” and “strawberry hemangioma.”
Virchow’s “angioma cavernosum” actually was used to labelVirchow’s “angioma cavernosum” actually was used to label
two distinct lesions, infantile hemangiomas (when locatedtwo distinct lesions, infantile hemangiomas (when located
deep to the skin) and VMs, because they have similardeep to the skin) and VMs, because they have similar
appearances on physical examination.appearances on physical examination.

IntroductionIntroduction
 Another example is Virchow’s designation of the “angiomaAnother example is Virchow’s designation of the “angioma
racemosum,” which referred to what is called today anracemosum,” which referred to what is called today an
AVM and which also has been historically called anAVM and which also has been historically called an
“arteriovenous hemangioma.”“arteriovenous hemangioma.”
 Wegener, a student of Virchow, described the histology ofWegener, a student of Virchow, described the histology of
LMs, which he called “lymphangiomas.” The termLMs, which he called “lymphangiomas.” The term cysticcystic
hygroma, referring to LMs, unfortunately alsohygroma, referring to LMs, unfortunately also continuescontinues
to have common usage.to have common usage.
 Thus, both the termsThus, both the terms cystic hygroma and lymphangiomacystic hygroma and lymphangioma
should be abandoned in favor of LM (macrocystic andshould be abandoned in favor of LM (macrocystic and
microcystic, respectivelymicrocystic, respectively

Vascular tumorsVascular tumors
HaemangiomaHaemangioma
Endothelial neoplasms
Increased endothelial
turnover
•Hemangiomais the
most common among
vascular tumors –
originates from
vasculogenesis(Angiogen
esis)

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 prematureThe incidence may be significantly higher in premature
infants 23%.infants 23%.
 They are much more common in whites than dark-skinnedThey are much more common in whites than dark-skinned
individuals.individuals.
 Infantile hemangiomas have a unique and characteristic lifeInfantile hemangiomas have a unique and characteristic life
cycle of rapid growth in the first year of life (proliferativecycle of rapid growth in the first year of life (proliferative
phase) followed by spontaneous slow regression from ages 1phase) followed by spontaneous slow regression from ages 1
to 7 years (involuting phase).to 7 years (involuting phase).
 Once involuted, they never recur.Once involuted, they never recur.

PathophysiologyPathophysiology
 The proliferating phase of hemangiomas is characterized byThe proliferating phase of hemangiomas is characterized by
angiogenesis in the tumor.angiogenesis in the tumor.
 The tumor is composed of plump, rapidly dividingThe tumor is composed of plump, rapidly dividing
endothelial cells, forming a mass of sinusoidal vascularendothelial cells, forming a mass of sinusoidal vascular
channels. Enlarged feeding arteries and draining veinschannels. Enlarged feeding arteries and draining veins
vascularize the tumor.vascularize the tumor.
 In addition, a specific marker for endothelial cells ofIn addition, a specific marker for endothelial cells of
hemangiomas that is not found in other vascular anomalies ishemangiomas that is not found in other vascular anomalies is
GLUT-1 (an erythrocytetype glucose transporter).GLUT-1 (an erythrocytetype glucose transporter).
 Proangiogenic factors, such asProangiogenic factors, such as fibroblast growth factor (FGF)fibroblast growth factor (FGF)
andand vascular endothelial growth factor (VEGF), lewis Yvascular endothelial growth factor (VEGF), lewis Y
antigenantigen are prominent during the proliferative phase.are prominent during the proliferative phase.
 Increased levels of these peptides may be found in the urineIncreased 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 weeksThe median age of appearance is 2 weeks
after birth ,however about 50% of IH areafter birth ,however about 50% of IH are
visible at birth by the presence of a faitvisible at birth by the presence of a fait
macule stain ,small pale spot or ecchymoticmacule stain ,small pale spot or ecchymotic
area.area.
 Hemangiomas are most often singleHemangiomas are most often single
cutaneous lesions and have an anatomiccutaneous lesions and have an anatomic
predilection for the head and neck regionpredilection for the head and neck region
(60%).(60%).
 They occur in the trunk in 25% of casesThey 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 areInternal and visceral lesions are
uncommon.uncommon.
 Up to 20% of patients can have multipleUp to 20% of patients can have multiple
tumors.tumors.

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

 The proliferative phaseThe proliferative phase of hemangiomas is marked by rapidof hemangiomas is marked by rapid
growth, for the first 6 to 8 months, which typically plateaus bygrowth, for the first 6 to 8 months, which typically plateaus by
age 10 to 12 months. Tumors that involve the superficialage 10 to 12 months. Tumors that involve the superficial
dermis are first seen as a red, raised lesiondermis 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 muscleTumors in the lower dermis, subcutaneous tissue, or muscle
appear bluish with slightly raised overlying skin and haveappear bluish with slightly raised overlying skin and have
frequently incorrectly beenfrequently incorrectly been called “cavernous” hemangiomascalled “cavernous” hemangiomas..
 The involuting phase of hemangiomas occurs from age 1 to 7The involuting phase of hemangiomas occurs from age 1 to 7
years, during which time the tumor slowly regresses. Thisyears, during which time the tumor slowly regresses. This
phase is notable for the fading color of the tumor to a dullphase is notable for the fading color of the tumor to a dull
purple and the softening of the tumor mass. The skin usuallypurple and the softening of the tumor mass. The skin usually
becomes pale in the center of the tumor first, spreadingbecomes pale in the center of the tumor first, spreading
outward. Both the deep color and the bulk of the tumor showoutward. Both the deep color and the bulk of the tumor show
continued gradual improvement until the regression iscontinued gradual improvement until the regression is
entirely complete by age 10 to 12 years.

Glut-1 positive stain in hemangiomasGlut-1 positive stain in hemangiomas
 Additionally, hemangiomas haveAdditionally, hemangiomas have
a unique vascular phenotypea unique vascular phenotype
demonstrated by glucosedemonstrated by glucose
transporter- 1 (GLUT-1) stainingtransporter- 1 (GLUT-1) staining
(Fig)(Fig)
 . Since its first description by. Since its first description by
North in 2000, staining forNorth in 2000, staining for
GLUT- 1 has become widespreadGLUT- 1 has become widespread
by clinicians and researchers inby clinicians and researchers in
the field of vascular anomalies.the field of vascular anomalies.
 Endothelial cells in RICH andEndothelial cells in RICH and
other vascular tumors orother vascular tumors or
malformations do not expressmalformations do not express
GLUT-1 protein.GLUT-1 protein.

Posterior cervical hemangiomaPosterior cervical hemangioma (left)(left)
has undergonehas undergone involutioninvolution (right).(right).

ComplicationsComplications
 The primary local complications OFThe primary local complications OF
cutaneous hemangiomas are ulceration,cutaneous hemangiomas are ulceration,
bleeding, and pain.bleeding, and pain.
 Lesions of the cervicofacial region mayLesions of the cervicofacial region may
produce airway obstruction as they growproduce airway obstruction as they grow
during the proliferative phase.during the proliferative phase.
 Very large hemangiomas notably of theVery large hemangiomas notably of the
liver, can lead to high-output congestiveliver, can lead to high-output congestive
heart failure secondary to fast flow andheart failure secondary to fast flow and
vascular shunting within the tumor.vascular shunting within the tumor.
 Facial lesions can result in tissue necrosisFacial lesions can result in tissue necrosis
with cosmetic consequences whenwith cosmetic consequences when
involving the eyelid, nose, lip, or ear..involving the eyelid, nose, lip, or ear..

ComplicationsComplications
Periorbital 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 HemangiomaParotid Hemangioma
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 theParotid hemangioma. a Huge hemangioma of the
parotid gland. Clinically no airway obstruction. bparotid gland. Clinically no airway obstruction. b
MRI shows deviation of the trachea but noMRI shows deviation of the trachea but no
compression. Complete regression without therapycompression. Complete regression without therapy

Other ManifestationsOther Manifestations
Disseminated neonatal hemangiomatosisDisseminated neonatal hemangiomatosis
(DNH).(DNH).
 Hemangiomatosis consists ofHemangiomatosis consists of
multiple disseminatedmultiple disseminated
hemangiomas. The cutaneoushemangiomas. The cutaneous
tumors are usually tiny (<5tumors are usually tiny (<5
mm) and, when five or moremm) and, when five or more
are present, visceral lesionsare present, visceral lesions
should be sought as well.should be sought as well.
 Screening patients withScreening patients with
ultrasonography or magneticultrasonography or magnetic
resonance imagine (MRI)resonance imagine (MRI)
may be indicated for thesemay be indicated for these
patientspatients

Disseminated neonatal hemangiomatosisDisseminated 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 hemangiomatosis
in 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 hemangiomasInfantile hepatic hemangiomas
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 lesionsFocal lesions are single, generally large masses onare single, generally large masses on
antenatal imaging studies and/or present as a massantenatal imaging studies and/or present as a mass
in early infancy.They may cause moderatein early infancy.They may cause moderate
thrombocytopenia that generally resolvesthrombocytopenia that generally resolves
spontaneously.spontaneously.
 This is in contrast to the profound thrombocytopeniaThis 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 exhibitThese single large lesions often do not exhibit
growth after birth and are thought to represent RICHgrowth after birth and are thought to represent RICH
lesions, which are histologically distinct from thelesions, which are histologically distinct from the
typical hemangioma.typical hemangioma.
 They are fully grown at birth and regress much fasterThey are fully grown at birth and regress much faster
than the typical hemangioma.than the typical hemangioma.

Focal liver hemangiomaFocal liver hemangioma

 Multifocal hepatic hemangiomasMultifocal hepatic hemangiomas are histologicallyare histologically
identical to the typical cutaneous hemangioma.identical to the typical cutaneous hemangioma.
Smaller enlarging lesions, detected by imagingSmaller enlarging lesions, detected by imaging
stimulated by the presence of cutaneousstimulated by the presence of cutaneous
hemangiomas, may often remain asymptomatic.hemangiomas, may often remain asymptomatic.
 However, some will also have associatedHowever, 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 severebut embolization may be necessary in those severe
cases in which there is insufficient time to wait forcases in which there is insufficient time to wait for
involution.involution.

Multifocal liverMultifocal liver
hemangiomahemangioma
Diffuse liver hemangioma.Diffuse liver hemangioma.
Liver transplantationLiver transplantation

 The most dangerous hepatic hemangiomas are theThe most dangerous hepatic hemangiomas are the diffusediffuse
typetype, presenting with innumerable compacted nodular lesions, presenting with innumerable compacted nodular lesions
replacing normal liver parenchyma, and causing massivereplacing normal liver parenchyma, and causing massive
hepatomegaly, abdominal compartment syndrome, andhepatomegaly, abdominal compartment syndrome, and
respiratory compromise.respiratory compromise.
 Massive hemangiomasMassive hemangiomas (e.g., those causing hepatomegaly)(e.g., those causing hepatomegaly)
may induce profoundmay induce profound acquired hypothyroidism.acquired hypothyroidism. HemangiomasHemangiomas
have been found to express type 3-iodothyronine deiodinasehave 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 screenedTherefore, patients with large hemangiomas should be screened
by measuring thyroid-stimulating hormone levels. Whenby measuring thyroid-stimulating hormone levels. When
untreated, hypothyroidism in infancy will lead to severe mentaluntreated, hypothyroidism in infancy will lead to severe mental
retardation. Aggressive exogenous thyroid replacement andretardation. Aggressive exogenous thyroid replacement and
close endocrinologic consultation are mandated. The conditionclose endocrinologic consultation are mandated. The condition
is self-limiting after tumor involution.is self-limiting after tumor involution.

The differential diagnosis of hepaticThe differential diagnosis of hepatic
hemangiomashemangiomas
 The differential diagnosis of hepaticThe differential diagnosis of hepatic
hemangiomas includes AVMs and malignanthemangiomas includes AVMs and malignant
tumors such as hepatoblastoma and metastatictumors such as hepatoblastoma and metastatic
neuroblastoma.neuroblastoma.
 The diagnosis is established by imaging withThe diagnosis is established by imaging with
ultrasonography, MRI, or computed tomographyultrasonography, MRI, or computed tomography
(CT).(CT).
 If typical imaging findings of one of theIf typical imaging findings of one of the
hemangioma patterns are not found,hemangioma patterns are not found,
percutaneous biopsy may be indicated to ensurepercutaneous biopsy may be indicated to ensure
a malignancy is not present.a malignancy is not present.

ImagingImaging
 Ultrasonography and MRI are the principal useful modalities.Ultrasonography and MRI are the principal useful modalities.
Ultrasonography of proliferative phase hemangiomasUltrasonography of proliferative phase hemangiomas
demonstrates a mass with dense parenchyma exhibitingdemonstrates a mass with dense parenchyma exhibiting fast-fast-
flow vascularity.flow vascularity.
 This distinguishes deep infantile hemangiomas fromThis distinguishes deep infantile hemangiomas from VMsVMs thatthat
exhibitexhibit slow flowslow flow and larger blood-filled spaces.and larger blood-filled spaces.
 MRI of proli ferating hemangiomas shows a lobulated solidMRI of proli ferating hemangiomas shows a lobulated solid
mass of intermediate intensity with T1-weighted spin-echomass of intermediate intensity with T1-weighted spin-echo
sequences and moderate hyperintensity on T2-weighted spin-sequences and moderate hyperintensity on T2-weighted spin-
echo images.echo images.
 For the involuting phase, MRI demonstrates decreased flowFor the involuting phase, MRI demonstrates decreased flow
voids and vascularity with the mass, taking on a more lobularvoids and vascularity with the mass, taking on a more lobular
and fatty appearance.and fatty appearance.

TreatmentTreatment
 Observation is the mainsty of managementObservation is the mainsty of management
since 90% of IH are small ,localized andsince 90% of IH are small ,localized and
do not invovle vital structures.do not invovle vital structures.
 Because hemangiomas are tumors of pureBecause hemangiomas are tumors of pure
angiogenesis, pharmacologic therapyangiogenesis, pharmacologic therapy
involves angiogenesis inhibition.involves angiogenesis inhibition.

TreatmentTreatment
 The first-line antiangiogenic therapy forThe first-line antiangiogenic therapy for
hemangiomas exhibiting appropriate risk factors orhemangiomas exhibiting appropriate risk factors or
complications is systemic corticosteroids.complications is systemic corticosteroids.
 Oral prednisone is used at a dose of 2 to 3Oral prednisone is used at a dose of 2 to 3
mg/kg/day. Doses up to 5 mg/kg/day have beenmg/kg/day. Doses up to 5 mg/kg/day have been
administered for life-threatening complications withadministered for life-threatening complications with
large hemangiomas causing airway obstruction orlarge hemangiomas causing airway obstruction or
heart failure.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 thatIntralesional corticosteroids are used for hemangiomas that
cause local deformity or ulceration, especially for facial lesionscause 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 perA 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 ofSubcutaneous 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 afterCases have been reported of blindness after intralesionalintralesional
corticosteroid injection for periorbital hemangiomas.corticosteroid injection for periorbital hemangiomas. This hasThis has
been presumed to be secondary to particle embolization of thebeen presumed to be secondary to particle embolization of the
retinal artery through feeding vessels. Manual compressionretinal artery through feeding vessels. Manual compression
around the periphery of the tumor is recommended duringaround the periphery of the tumor is recommended during
injection.injection.

TreatmentTreatment
 RecombinantRecombinant interferoninterferon αα 2a& 2b2a& 2b,,
previously considered as a second-linepreviously considered as a second-line
agent, has fallen out of favor, except inagent, has fallen out of favor, except in
very limited circumstances, because of thevery limited circumstances, because of the
severe potential complication of spasticsevere potential complication of spastic
diplegia that can occur in 5% to 12% ofdiplegia that can occur in 5% to 12% of
treated infants.treated infants.
 On the other hand, experience is growingOn the other hand, experience is growing
with low-dose, high-frequencywith low-dose, high-frequency
antiangiogenic regimens of vincristine asantiangiogenic regimens of vincristine as
second-line after corticosteroids.second-line after corticosteroids.
A recent report of propranolol used to treatA recent report of propranolol used to treat
infantile hemangioma may indeedinfantile hemangioma may indeed
revolutionize medical therapy.revolutionize medical therapy.

PROPRANOLOL(INDERAL )PROPRANOLOL(INDERAL )
 IT IS NON SELECTIVEIT IS NON SELECTIVE ββ BLOCKER.BLOCKER.
 It may be efficacious as corticosteriod forIt 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 proteinsDowen regulation of angiogenic proteins
such assuch 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 oftenAlthough it has popular appeal, laser therapy is not often
beneficial for infantile hemangiomas. The flashlampbeneficial for infantile hemangiomas. The flashlamp
pulse-dye laser penetrates the dermis to a depth of onlypulse-dye laser penetrates the dermis to a depth of only
0.75 to 1.2 mm. Most cutaneous hemangiomas are deeper0.75 to 1.2 mm. Most cutaneous hemangiomas are deeper
and therefore not affected by laser treatment.and therefore not affected by laser treatment.
 Also, the use of an endoscopic continuous-wave CO2 laserAlso, the use of an endoscopic continuous-wave CO2 laser
has been shown to be a good technique for controllinghas been shown to be a good technique for controlling
proliferative-phase hemangiomas in the unilateralproliferative-phase hemangiomas in the unilateral
subglottic location.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 asuch as the upper eyelid, when visual obstruction is a
concern.concern.

Indications for surgical resection of infantileIndications for surgical resection of infantile
hemangiomashemangiomas
 well-localized or pedunculated tumors can be expeditiouslywell-localized or pedunculated tumors can be expeditiously
resected with linear closure, especially for tumors complicatedresected with linear closure, especially for tumors complicated
by bleeding and ulceration. Sites that are most amenable toby 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 doTumors of the upper eyelid that obstruct vision and that do
not respond to pharmacologic therapy also may requirenot respond to pharmacologic therapy also may require
surgical excision or debulking. Focal lesions of thesurgical excision or debulking. Focal lesions of the
gastrointestinal tract that cause bleeding and for whichgastrointestinal tract that cause bleeding and for which
medical management fails also may require resection bymedical management fails also may require resection by
means of enterotomy or endoscopic band ligation.means of enterotomy or endoscopic band ligation.
 Finally, for th difficult-to-treat and life-threatening largeFinally, for th difficult-to-treat and life-threatening large
hemangiomas, especially in the liver, angiographichemangiomas, especially in the liver, angiographic
embolization may be required to manage high-output cardiacembolization may be required to manage high-output cardiac
failure.failure.

Indications for surgical resectionIndications for surgical resection
of infantile hemangiomasof infantile hemangiomas

Congenital hemangiomasCongenital hemangiomas
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 hemangioendotheliomaRapid involuting congenital hemangioendothelioma
(RICH)(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
(“prenatal
mature
hemangioma”) and
should be clearly
differentiated
from an infantile
hemangioma
because it is
negative for GLUT-1.

Rapidly involuting congenitalRapidly involuting congenital
hemangioma (RICH)hemangioma (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 Kaposiform
Hemangioendothelioma(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 KaposiformTufted Angioma(TA) and Kaposiform
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 hemangioendotheliomakaposiform hemangioendothelioma
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 profoundwas first reported in 1940 as a case of profound
thrombocytopenia, petechiae, and bleeding in conjunction withthrombocytopenia, petechiae, and bleeding in conjunction with
aa “giant hemangioma.“giant hemangioma.
 The only knownThe only known true associations are with TA and KHE. Thetrue associations are with TA and KHE. The
platelet count with this disorder is typically less thanplatelet count with this disorder is typically less than
10,000/mm3 and may be accompanied by decreased fibrinogen10,000/mm3 and may be accompanied by decreased fibrinogen
levels and a mildly elevated (PT) and (PTT).levels and a mildly elevated (PT) and (PTT).
 Treatment of KHE with KMP is primarily medical, because theTreatment of KHE with KMP is primarily medical, because the
tumor is usually too large and extensive to be resected.tumor is usually too large and extensive to be resected.
Corticosteroids and interferon alfa have been effective in aboutCorticosteroids and interferon alfa have been effective in about
50% of cases. Vincristine also is beneficial, but no single agent50% of cases. Vincristine also is beneficial, but no single agent
has been shown to be consistently successful. Platelethas been shown to be consistently successful. Platelet
transfusions are ineffective and should be avoided unless activetransfusions are ineffective and should be avoided unless active
bleeding occurs. Mortality rates with KMP and KHE or TAbleeding occurs. Mortality rates with KMP and KHE or TA
remain high at 20% to 30%.remain high at 20% to 30%.

Other Vascular Tumors of InfancyOther Vascular Tumors of Infancy
Pyogenic 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 MalformationsVascular Malformations
 Vascular malformations are congenital lesions ofVascular malformations are congenital lesions of
vascular dysmorphogenesis that can be local orvascular dysmorphogenesis that can be local or
diffuse.diffuse.
 The prevalence ofThe prevalence of Vascular Malformations :1.2-1.5%.Vascular Malformations :1.2-1.5%.
 They are classified, based on the appearance of theThey 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 notMalformations grow with the child and do not
undergo the rapid proliferative growth phaseundergo the rapid proliferative growth phase
exhibited by hemangiomas.exhibited by hemangiomas.
 The greatest distinction between hemangiomas andThe greatest distinction between hemangiomas and
malformations is that the former spontaneouslymalformations is that the former spontaneously
involute and the latter do not.involute and the latter do not.

Capillary malformationCapillary malformation
Equal gender distribution
Birth 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 Malformations(CMs)Capillary Malformations(CMs)

The histology ofThe histology of
cutaneous CMs consists ofcutaneous CMs consists of
dilated capillary- todilated capillary- to
venule-size vessels locatedvenule-size vessels located
in the superficial dermis.in the superficial dermis.
These abnormal vesselsThese abnormal vessels
gradually dilate over time,gradually dilate over time,
leading to a darker colorleading to a darker color
and,occasionally, nodularand,occasionally, nodular
ectasias.ectasias.

Clinical presentationClinical presentation
 At birth:Well-circumscribedAt birth:Well-circumscribed
red macular lesionsred macular lesions
 Commonly occur on the faceCommonly occur on the face
in the trigerminalin the trigerminal
distribution.distribution.
 45% restricted to 1/345% restricted to 1/3
trigeminal dermatomestrigeminal dermatomes
 ••55% overlap dermatomes,55% overlap dermatomes,
cross the midline, bilaterally.cross the midline, bilaterally.

 CMs can be associatedCMs can be associated
with underlying softwith underlying soft
tissue and skeletaltissue and skeletal
overgrowth as well asovergrowth as well as
other internalother internal
abnormalities.abnormalities.
 CMs of the occiputCMs of the occiput
can signal underlyingcan signal underlying
encephalocele orencephalocele or
ectopic meninges.ectopic meninges.

Sturge-Weber syndromeSturge-Weber syndrome
 Facial capillary malformaionFacial capillary malformaion
 Ipsilateral occular andIpsilateral occular and
leptomeningeal vascularleptomeningeal vascular
anomaliesanomalies
 Neurologic : seizures ,Neurologic : seizures ,
hemiparesis , delayed motor andhemiparesis , delayed motor and
cognitive developmentcognitive development
 Ophthalmologic:Ophthalmologic:
glaucoma&retinal detachementglaucoma&retinal detachement
 MRI reveals the central nervousMRI reveals the central nervous
system abnormalities, showingsystem abnormalities, showing
pial vascular enhancement andpial vascular enhancement and
gyriform calcificationsgyriform calcifications

 Port wine stain (PWS).Port wine stain (PWS).
Sturge-WeberSturge-Weber
syndrome.syndrome.
 Capillary malformation.Capillary malformation.
Therapy is dye-laser orTherapy is dye-laser or
Vasculight (IPL)Vasculight (IPL)

MANAGEMENTMANAGEMENT
Flash 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 malformation
Lymphatic Malformations
Embryogenic disturbance of lymphatic
system
At birth -2 yrs
No involution
Microcystic malformation (lymphagiomas)
Macrocystic (cystic hygroma)
Combined

Rich lymphaticRich lymphatic
areaarea ::
H&NH&N
AxillaAxilla
MediastinumMediastinum
groingroin
retroperitoneumretroperitoneum

lymphangioma cutislymphangioma cutis
Involvement 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 disease.”“disappearing bone disease.”
 LMs of the extremities are seen in conjunction withLMs of the extremities are seen in conjunction with
overgrowth and limb-length discrepancy.overgrowth and limb-length discrepancy.
 A rare but very difficult problem arises with Gorham’A rare but very difficult problem arises with Gorham’
syndrome, in which soft tissue and skeletal LMs leadsyndrome, in which soft tissue and skeletal LMs lead
to progressive osteolysis and “disappearing boneto progressive osteolysis and “disappearing bone
disease.” Pathologic fractures and vertebraldisease.” Pathologic fractures and vertebral
instability can become manifest with this often fatalinstability can become manifest with this often fatal
syndrome.syndrome.

Facial soft tissue distortionFacial soft tissue distortion
Bony hypertrophyBony hypertrophy
Periorbital LMs can lead to proptosis.
Facial LMs can cause the associated
deformities of macrocheilia,
macroglossia, and macromala

EXITEXIT
Large cervicofacialLarge cervicofacial
LMs with airwayLMs with airway
obstruction------obstruction------exex
utero intrapartumutero intrapartum
treatment.treatment.

LymphedemaLymphedema
 Lymphedema is a type of LMsLymphedema is a type of LMs
 Milroys disease:congenital form of lymphedemaMilroys disease:congenital form of lymphedema
 Meige disease:pubertal onset of lymphedemaMeige disease:pubertal onset of lymphedema

ImagingImaging
Well-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 complex
LMs as well as their
macrocystic and microcystic
components.
Tracheostomy may be needed
in cases of oropharyngeal and
airway obstruction.

ManagementManagement
Surgical 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 MALFORMATIONS
PathophysiologyPathophysiology
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.

PresentationPresentation
 VMs, often incorrectly calledVMs, often incorrectly called
““cavernous hemangiomascavernous hemangiomas,” are,” are
consisting of venous channels thatconsisting of venous channels that
can develop anywhere in the body.can develop anywhere in the body.
 VMs are probably the most commonVMs are probably the most common
of the vascular malformations, andof the vascular malformations, and
they are more likely to be multiple.they are more likely to be multiple.
 VMs of the gastrointestinal tract areVMs of the gastrointestinal tract are
often multiple and can affect everyoften multiple and can affect every
part of the GIT.part of the GIT.
On examinationOn examination
 these soft, bluish, compressiblethese soft, bluish, compressible
lesions will expand with dependentlesions will expand with dependent
position and Valsalva maneuver.position and Valsalva maneuver.

Blue rubber bleb nevus syndromeBlue rubber bleb nevus syndrome
BRBNSBRBNS
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 rare(or Bean’s syndrome) represents a specific rare
disorder consisting of multifocal VMs that affect thedisorder consisting of multifocal VMs that affect the
skin and gastrointestinal tract primarilyskin and gastrointestinal tract primarily
Multiple scattered blue to blackMultiple scattered blue to black
rubbery papules and nodulesrubbery papules and nodules
involving the mid-chest regioninvolving the mid-chest region
Purple to blue/black papulesPurple to blue/black papules
involving the upper andinvolving the upper and
lower lipslower lips

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

 Indication : appearance, functional,painIndication : appearance, functional,pain
 Conservative therapy: elastic compressionConservative therapy: elastic compression
+baby aspirin (prophylaxis painful+baby aspirin (prophylaxis painful
thromboses)thromboses)
 Sclerotherapy : main treatmentSclerotherapy : main treatment
 Laser (Nd:YAG)Laser (Nd:YAG)
 ResectionResection

Sclerotherapy is the primary treatment forSclerotherapy is the primary treatment for
VMs, although subsequent surgical resection isVMs, although subsequent surgical resection is
often needed.often needed.
VM Upper lip, Post Sclerotherapy and excision

Maffucci SyndromeMaffucci Syndrome
Maffucci 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 Malformation
Errors of embryogenic vascular development
Failure of arteriovenous channels in the
primitive retiform plexus to regress

CLINICAL
The pink-bluish stain of the AVM
is usually noted at birth
trauma seem to trigger expansion
Local warmth , Palpable thrill ,
Audible bruit
Headache , seizure in
Intracranial AVM
The epicenter of anAVM is
called the nidus and consists of
arterial feeders

Schobinger Clinical Staging SystemSchobinger Clinical Staging System
For Arteriovenou MalformationsFor Arteriovenou Malformations
Stage Clinical Findings
I (Quiescent) Pink to bluish stain, cutaneous
warmth, and arteriovenous
shunting by Doppler ultrasound
imaging
II (Expanding) Same as stage I, plus enlargement,
pulsation, thrill, bruit, and
tortuous and tense veins
III (Destructive) Same as stage II, plus skin
ulceration, bleeding, persistent
pain, or tissue necrosis
IV (Decompensating) Same as stage III, plus cardiac failure

ImagingImaging
Ultrasonography and Doppler
imaging are excellent
tools to elucidate the fast flow of
these lesions and to
distinguish them from VMs.
MRI and MR angiography are
the most useful tools
to demonstrate the full extent of
these lesions.
AVM Angiography

The 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 MALFORMATIONSCOMBINED VASCULAR MALFORMATIONS
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
lymphaticovenous
malformation (CLVM)
usually involves one or more
extremities, most often a
lower limb, and is associated
with prominent soft tissue
and bony hypertrophy.

Klippel-Trénaunay syndromeKlippel-Trénaunay syndrome
(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 Syndrome)(Parkes-Weber 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 extremity
CAVM 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 Syndrome
Proteus 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

Bannayan-Riley-RuvalcabaBannayan-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 polyps
of the ileum and colon, and
Hashimoto’s thyroiditis.

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