3. 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.
Vascular MalformationsVascular Malformations are congenital lesions ofare congenital lesions of
vascular dysmorphogenesis that arise because ofvascular dysmorphogenesis that arise because of
errors of embryonic development. These lesionserrors of embryonic development. These lesions
exhibit normal endothelial cell turnover.exhibit normal endothelial cell turnover.
4. 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.
WhereasWhereas Vascular malformationsVascular malformations areare
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.
7. Infantile HemangiomaInfantile Hemangioma
(IH)(IH)
The most common vascular tumor of infancy, with aThe most common vascular tumor of infancy, with a
female-to-male ratio of 3 - 5:1.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%.
80% - solitary, 20% - multifocal.80% - solitary, 20% - multifocal.
Once involuted, they never recur.Once involuted, they never recur.
8. PathophysiologyPathophysiology
The proliferating phase - clonal expansion of hemangoima –The proliferating phase - clonal expansion of hemangoima –
initiation of multipotent stem cell vasculogenic activity withinitiation of multipotent stem cell vasculogenic activity with
CD 133+ve.CD 133+ve.
Histopathology- plump, rapidly dividing endothelial cells,Histopathology- plump, rapidly dividing endothelial cells,
forming a mass of sinusoidal vascular channels. Enlargedforming a mass of sinusoidal vascular channels. Enlarged
feeding arteries and draining veins vascularize the tumor.feeding arteries and draining veins vascularize the tumor.
Specific marker -Specific marker - GLUT-1 (an erythrocytetype glucoseGLUT-1 (an erythrocytetype glucose
transporter).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.
10. 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.
Most often single cutaneous lesions andMost often single cutaneous lesions and
have an anatomic predilection for the headhave an anatomic predilection for the head
and neck region (60%).and neck region (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.
11. Clinical PresentationClinical Presentation
The Proliferative phaseThe Proliferative phase - rapid growth in first 6 to 8 months,- rapid growth in first 6 to 8 months,
plateaus by age 10 to 12 months.plateaus by age 10 to 12 months.
superficial dermis component - red, raised lesionsuperficial dermis component - red, raised lesion (previously(previously
named “capillary” or “strawberry” hemangiomas;).named “capillary” or “strawberry” hemangiomas;).
Deep dermis component – soft ,warm ,bluish (prevoiuslyDeep dermis component – soft ,warm ,bluish (prevoiusly
namednamed cavernous hemangiomacavernous hemangioma))
The Involuting phaseThe Involuting phase – dull purplish hue,– dull purplish hue, decreased turgordecreased turgor
pressure, lasts upto 2-10 years, residual telengectesia seen.pressure, lasts upto 2-10 years, residual telengectesia seen.
The Involuted phase-The Involuted phase- regression complete 50% by 5 yr,70%regression complete 50% by 5 yr,70%
by 7yr, mild alteration in texture of skin.by 7yr, mild alteration in texture of skin.
12. Deep (a), mixed (b) and superficial nasal tip (c) hemangiomasDeep (a), mixed (b) and superficial nasal tip (c) hemangiomas
14. ComplicationsComplications
90% asymptomatic.90% asymptomatic.
5% MC complication – Ulceration5% MC complication – Ulceration
Others – pain, infection , local symptomsOthers – pain, infection , local symptoms
Periocular or eyelid IH – vision problemPeriocular or eyelid IH – vision problem
Pinna IH – hearing lossPinna IH – hearing loss
Upper neck & chin - airway obstructionUpper neck & chin - airway obstruction
Vicseral – in liver and GIT – bleeding.Vicseral – in liver and GIT – bleeding.
15. 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
16. ImagingImaging
USG COLOR DOPPLER – shunting pattern, decrease arterialUSG COLOR DOPPLER – shunting pattern, decrease arterial
resistance, increase venous flow (proliferative phase) &resistance, increase venous flow (proliferative phase) &
decrease venous flow (involuting phase)decrease venous flow (involuting phase)
MRI WITH Gd CONTRAST –MRI WITH Gd CONTRAST –
T1- weighted spin echo – parenchymal tissue ofT1- weighted spin echo – parenchymal tissue of
intermediate intensity.intermediate intensity.
T2 weighted spin echo – prominent flow voids located in &T2 weighted spin echo – prominent flow voids located in &
around tumor, rapid flow in feeding artery and dilated veins.around tumor, rapid flow in feeding artery and dilated veins.
19. LASER TherapyLASER Therapy
1. PULSE-DYE LASER – for flat, superficial hemangioma,1. PULSE-DYE LASER – for flat, superficial hemangioma,
residual telengectasia, PW stainresidual telengectasia, PW stain
2. Nd-YAG LASER – periorbital hemangioma2. Nd-YAG LASER – periorbital hemangioma
3. KTP LASER – periorbital and pinna hemangioma3. KTP LASER – periorbital and pinna hemangioma
4. CO2 LASER – subglottic hemangioma.4. CO2 LASER – subglottic hemangioma.
20. Indications for surgical resection ofIndications for surgical resection of
infantile hemangiomasinfantile hemangiomas
1. Bleeding1. Bleeding
2. Not respond to medical management2. Not respond to medical management
3. Life-threatning condition.3. Life-threatning condition.
4. For school going children – Cosmetic / Asthetics Purpose.4. For school going children – Cosmetic / Asthetics Purpose.
21. Indications for surgical resectionIndications for surgical resection
of infantile hemangiomasof infantile hemangiomas
22. Congenital hemangiomasCongenital hemangiomas
Clinical PresentationClinical Presentation
Rare congenital
hemangiomas are fully
developed at birth.
These are the rapidly
involuting congenital
hemangioma (RICH)
and the noninvoluting
congenital
hemangioma (NICH).
23. 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.
25. 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
26. Tufted Angioma(TA) and KaposiformTufted Angioma(TA) and Kaposiform
Hemangioendothelioma(KHE )Hemangioendothelioma(KHE )
Both tumors typically present at birth.
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).
28. Other Vascular Tumors of InfancyOther Vascular Tumors of Infancy
Pyogenic granuloma
Is an acquired vascular lesion.
Occur on the skin and mucosa
of older children and young
adults, with a mean age 6.7 years.
Arise suddenly and usually
without a history of trauma.
Frequently located on the
cheeks, eyelids, extremities.
Complications: superficial
ulceration and repetitive
episodes of bleeding.
29. 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.
30. Capillary malformationCapillary malformation
Equal gender distribution
Birth prevalence – 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
31. Capillary Malformations(CMs)Capillary Malformations(CMs)
Histology - dilatedHistology - dilated
capillary- to venule-sizecapillary- to venule-size
vessels located in thevessels located in the
superficial dermis. Thesesuperficial dermis. These
abnormal vessels graduallyabnormal vessels gradually
dilate over time, leading todilate over time, leading to
a darker color and ,a darker color and ,
occasionally, nodularoccasionally, nodular
ectasias.ectasias.
32. 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 trigeminal distribution.in the trigeminal 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.
33. Associated ConditionsAssociated Conditions
CMs of the occiput canCMs of the occiput can
signal underlyingsignal underlying
encephalocele or ectopicencephalocele or ectopic
meninges.meninges.
Cobb’s Syndrome- CMCobb’s Syndrome- CM
in upper back indicatesin upper back indicates
AVM of Spinal cord.AVM of Spinal cord.
34. Sturge-Weber syndromeSturge-Weber syndrome
Facial capillary malformaionFacial capillary malformaion
Ipsilateral occular andIpsilateral occular and
leptomeningeal vascularleptomeningeal vascular
anomaliesanomalies
Neurologic :Neurologic : seizures ,seizures ,
hemiparesis , delayed motor andhemiparesis , delayed motor and
cognitive developmentcognitive development
Ophthalmologic:Ophthalmologic:
glaucoma&retinal detachementglaucoma&retinal detachement
MRI reveals the CNSMRI reveals the CNS
abnormalities, showing pialabnormalities, showing pial
vascular enhancement andvascular enhancement and
gyriform calcificationsgyriform calcifications
35. 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
39. 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.
40. 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
42. 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
43. ImagingImaging
Well-localized and cystic
LMs are easily characterized by
ultrasonography and CT. No
flow.
MRI - 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.
44. 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, doxycycline,bleomycin,OK-432,acetic
acid that produce scarring and collapse of the cysts.
45. 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.
46. 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.
MC Vascular Malformation.MC Vascular Malformation.
Multiple in nature.Multiple in nature.
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
soft, bluish, compressible lesions willsoft, bluish, compressible lesions will
expand with dependent position andexpand with dependent position and
Valsalva maneuver.Valsalva maneuver.
47. 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.
48. 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
49. ImagingImaging
Radiologic modalities - ultrasonography, MRI, and venography.
MRI is most informative and demonstrates hyperintense lesions
with T2-weighted sequences.
51. 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
55. CLINICAL FEATURES
The pink-bluish stain 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
56. 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
57. 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
58. ManagementManagement
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.
60. 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.
61. Klippel-Trénaunay syndromeKlippel-Trénaunay syndrome
(CLVM)(CLVM)Thrombophlebitis of the anomalous veins.
Lymphatic hyploplasia
Plain radiographs are used to document limb-length
discrepancies serially.
MRI provides 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.
62. Proteus SyndromeProteus Syndrome
It is overgrowth of skin, bones,
muscles, fatty tissues, and blood and
lymphatic vessels.
A/w Congenital nevi,lipoma,unusual
tumor
C/F : asymmetrical growths vary
greatly but typically the skull, one or
more limbs, and soles of the feet will be
affected.
Complications- deep vein
thrombosis and pulmonary embolism .
64. Parkes-Weber SyndromeParkes-Weber Syndrome
2 year old girl.Nevus,
phlebectasy,
hypertrophy (Parkes
Weber syndrome).
Overgrowth an extremity together with an
AVM & multiple AVFs.
C/F: Present at birth, appearing as overgrowth
with a large geographic macular pink stain.
MRI - symmetric muscular and bony
overgrowth
Angiography - discrete AVFs.
Treatment- superselective embolization is used
to occlude the arteriovenous shunts.
Complications- symptoms of ischemia, pain, or
high-output congestive heart failure occur.
65. Bannayan-Riley-RuvalcabaBannayan-Riley-Ruvalcaba
SyndromeSyndrome
It is a rare overgrowth syndrome.
Autosomal dominat.
PETN mutation.
Cutaneous CMs, VMs, or AVMs
are found.
C/F : macrocephaly, delayed
development,
multiple lipomas,
hamartomatous polyps
of the ileum and colon, and
Hashimoto’s thyroiditis.
