VASCULAR ANOMALIES

Mehtab Ahmed
Plastic surgery LNH
LEFT PIC 3 MONTHS OLD CHILD WITH LESION THAT DEVELOPED FEW WEEKS AFTER BIRTH
RT SAME CHILD AT 18 MONTHS
1) DESCRIBLE THE LESION??
2)WHAT IS YOUR DIAGNOSIS ? DESCRIBE ITS CHARACTERISTIC FEATURES??
3) WHAT ARE TREATMENT OPTIONS AVAILABLE?
5 YEARS OLD CHILD PRESENTS WITH THIS LESION .ITS PRESENT SINCE BIRTH AND PROGRESSIVELY GROWS WITH THE GROWTH OF CHILD
IT EMPTIES WITH PRESSURE BUT FILLS RAPIDLY.
1) WHAT IS YOUR DIAGNOSIS
2) NAME FAST FILLING LESIONS
3) HOW WILL YOU MANAGE THIS CHILD?
 CLASSIFICATION
 Mullekin and Glowacki classified vascular anamolies
into hemangiomas and vascular malformation based
on
 Clinical course
 Biologic behavior
 Histological features

confusing terminology and maltreatment based on misdiagnosis has been

great impediment in the filed of vascular anomalies in past
• Vascular tumors have growth potential like tumors by
endothelial proliferation.

 hemangioma is most common type of vascular
tumors

 vascular malformations develop by abnormal

development of vascular channels during embryonic
life
 DEFINATIONS
• HEMANGIOMA
– Hemangioma is a tumor of infancy that has phase of
rapid growth followed by phase of slow but
progressive regression during childhood

• VASCULAR MALFORMATIONS
– Congenital abnormal channels that are present at
birth grow proportionately with the growth of child
and never regress.
 Vascular Malformations
SIMPLE COMBINED
• Capillary • Capillary lymphatic
• Venous • Capillary venous
• Arterial • Capillary lymphatic
• Lymphatic venous
• Arteriovenous
• Capillary Arteriovenous
• Lymphatic arteriovenous
• AV fistulae
 Vascular Malformations

FAST FLOW SLOW FLOW

• Arterial Malformation • ALL other lesion
• AV fistula
• AV malformation
• Capillary arteriovenous
malformation
• Lymphatic arteriovenous
malformation.

Fast flow lesions fill in less that 2 seconds

 INFANTILE HEMANGIOMA

• Incidence is 5 to 10 percent in Caucasian

infants at 1 year
• Female to male ratio is 3to5 :1
• Preterm is known risk factor (23%)
• 80% lesions are solitary and 20% multiple
 PATHOGENESIS

• Clonal expansion of hemangioma initiating multipotent cells

• These cells form blood vessels which express GLUT-1 and

merosin

• Low expression of VEGFR-1 and missense mutation in VEGFR_2

TEM-8.

• Hemagioma is basically abnormal endothelial cells that are

derived from mutated stem cells

• Characteristic of infantile hemagioma is GLUT_1 which stain

with immunostains
CLINICAL COURSE

• Characteristic 3 phase growth pattern .

• Proliferative phase
• Involuting phase
• involuted phase
CLINICAL COURSE

• PROLIFERATIVE PHASE :
• Proliferation occurs in rapid growth phase in
first 8-10 years with cessation by one year of
life
• Superficial component: bright red well
demarcated non compressible plaque
• deep components: ill defined subcutaneous
mass that has bluish hue.
• INVOLUTING PHASE
• Color changes to purplish with increased pallor and decreased turgor

• This phase can last somewhere between 2 to 10 years

• INVOLUTED PHASE
• Roughly 50% regression at 5 years

• 70% regression at 7 years with continued improvement till 10 to 12

years

• Complete regression often leaves an area of altered skin

• Bulky large raised lesions regress more completely than flat lesion
 COMPLICATIONS
• ULCERATION
 Leads to infection ,pain ,bleeding

 Mostly during period of rapid growth

 Common in large lesion of lip ,perineum and

intertrigenous regions.

 Ulceration in anogenital region demands special attention

because of risk of bacterial contamination and infection.
• Eyelid hemangioma can cause visual disturbance strabismus and
even amblypia

• Hemangioma in neck and mandible can be associated with airway

hemangiomas

Diffuse hemangiomatosis
 more than 5 cutaneous lesions
 Present with triad of congestive heart failure, Hepatomegaly,
Anemia
 Hepatic hemangioma is associated with hypothyroidism
(iodothyronine deiodenase enzyme)
 CONGENITAL HEMANGIOMA
• Fully grown at birth
• Does not follow growth pattern of infantile
hemangioma.
• Does not stain with GLUT-1

• They have similar appearance, gender ratio,

histological and radiological features as IH.
RICH NICH

• Solitary raised , grey or • Well circumscribed lesion

voilecious lesion with with pink,blue or purple
ectasia,radial veins, hue,central talengectesia
Telengectesia and pale and pale rim
surrounding hallo

• Accelerated regression growth

• Grows proportionately to a
pattern complete by (6-14 child.
months)

• High output cardiac failure

 DIAGNOSIS

• Detailed clinical history

• Examination

• U/s (operator depends)

• MRI (gold standard)

• Whenever diagnosis of congenital hemangioma is in doubt or differenciation

from vascular malformation is not possible on detailed history and clinical
examination further investigation in indicated (hemangioma CME PRS 2009)
ANOMALIES WITH HEMANGIOMA
MANAGEMNET

• Urgency of treatment (life threatening /less

threatening )
• Location and associated symptoms
• Size
• Growth phase
• Age of the patient at evaluation
 MANAGEMNET
• OBSERVATION .
• Many hemangioma will spontaneously regress.
• Reassurance
• Regular follow up
• Monitoring growth pattern
• Dealing with complications

• Local wound care with either barrier cream, zinc

oxide, hydrophillic petroleum or occlusive
dressings
GOALS OF HEMANGIOMA TREATMENT
• Minimizing physical complications leading to
morbidity and mortality
• Alleviating psychosocial issues
• Avoiding overly aggressive treatment with
potential side effects.

• Hemangioma CME 2009.PRS

MANAGEMNET
• MEDICAL MANAGEMENT
1. Corticosteroids
2. Propranol
3. Alpha interferon
4. Vincristine
Corticosteroids
• Overall response rate of 85%

• Inhibition of vasculogenic potential of hemangioma

derived stem cells

• Down regulation of VEGF-A,urokinase

plasminogenactivator, monocyte chemoattractant protein
1 ,interlukin-6,MMP-1

• Route of administration can be intralesional ,topical or

oral for large lesions
 Corticosteroids

• Recommended dose is 2-3mg/kg of oral

prednisolone
• Intralesional dose is repeated every 6-8 weeks
• Oral dose is given as single morning dose for 6-8
weeks and then tapered gradually .
• Common side effects are cushingoid faces.
• Irritabbility,hypertension.immunosuppresion,hir
sutism,myopathy,cardiomyopathy and
premature thelarche .
CME on hemagioma
 PROPRANOLOL
• Remarkable literature on propranolol treatment was published in
2008

• First line of treatment in many centers .

• Mechanism of action is not exactly known but a possible mechanism

is inhibition of hypoxia inducible factor 1-alpha-VEGF signaling
pathway

• Dose is 2mg/kg divided in three doses

• Treatment continues up to end of proliferative phase and then

tapering in next 2 months
 PROPRANOLOL
• Adverse effects are hypotenstion, hypoglycemia,
bradycardia
• gradual dose increment and tapering and patient
education regarding side effects is important part
of propranolol treatment
 INTERFERON ALPHA
• Interferron alpha 2a and 2b is second line agent for
life threatening and vital function compromising
hemangiomas.
• Indications
• Failure to respond to corticosteroids
• Side effects of corticosteroids
• parental refusal to corticosteroids use
• Dose is 2-3mU/m2 and increased with childs growth
• Interferron is effective in kassabach-merritt
phenomenon
 KASSABACH-MERRITT PHENONMENON
• Consumption coagulopathy as platelets are trapped in vascular channels and
destroyed with thromboctopenia,loss of clotting factors ,DIC and even death

• Classically its associated with IH but also with kaposifrom

hemangioendothelioma and tufted angioma

• No platelets transfusion until bleeding or surgical procedure indicated

• Do not use heparin it aggravates situation .

• Fever is common side effect (acetaminophen)

• Spastic diplegia is most severe complication improves with cessation of

treatment
 LASERS IN HEMANGIOMA Rx
• PDL is used in relatively superficial flat lesions

• Most useful for residual talengectasia after

regression

• Nd-YAG and KPT lasers are also used in

hemangiomas but have higher rate of scarring .
 INDICATIONS OF SURGERY IN HEMANGIOMA

EARLY LATE
• Obstructive lesions • For residual scars
• Disfiguring lesions • For cosmesis by dealing
• In area of cosmetic with remnant fibrofatty
importance tissue
• For psychosocial reasons
• Lesions with complications
(ulceration and recurrent
bledding)
 VASULAR MALFORMATIONS
• 0.3 to 0.5%population with no gender predilection
• Each of the 4 basic types have characteristic
histopathological appearance
• Multidisciplinary team approach is best whenever
warranted .
INVESTIGATIONS
• MRI is gold standard with superb details of soft
tissue.
• MRA and MRV helps further in slow and fast flow
lesions
• Plain radiograph can help in skeletal growth
abnormalities and venous phlebitis
• US and Doppler ultrasound is help but operator
dependent
• Role of CT is limited except for intraosseous
anomalies
CAPILLARY MALFORMATIONS
• Most common anomalies with 3 in 1000 live births and
equal gender distribution

• Present at birth like red or pink intradermal

discoloration

• True CMs thicken ,darken and becomes nodular with age

• A variant called macular stain and named salmon patch,

nevus simplex or vascular stain disappear in first few
years of life
SYNDROMES ASSOCIATED WITH CMs

• Klippel-trenauny syndrome (slow flow CLVM with limb hemi

hypertrophy and axial elongation)

• Parkes-webber syndrome
• (AVM, cutaneous CM and skeletal and soft tissue hypertrophy of
limb)

• Cobbs syndrome (AVM of spinal cord with CM of back )

• Sturge-webber syndrome
CM in trigeminal nerve distribution , ipsilateral leptomenigeal and
occular vascular anomalies and seizures
 TREATMENT

• Flashlamp pumped PDL is treatment of choice

for CMs
• Those not responding to PDL , Nd-YAG,
alexendrite and IPL are other options
• Surgical option can be considered in selected
patients
 VENOUS MALFORMATIONS
• Soft ,compressible blue subcutaneous masses

• Enlarge with physical activity in dependent portions

• Lesions are typically painful in morning due to stasis and microthrombi

• Head and neck is most common site

• Blue-rubber bleb nevus syndrome

• Sporadic ,multiple lesions on palm ,sole trunk and sessile and polpoid
lesions in GIT
 Management
• MRI is extremely useful for diagnosis and
combined with venography helps in surgical
planning
• Coagulation profile should be checked due to
coagulopathy and risk of DIC is there following
trauma and intervention
• Percutaneous sclerotherapy is first line treatment
• Compression stockings and aspirin for phlebitis
are adjuncts
MANAGEMENT

• SURGERY is useful for

• cosmetic reasons specially in head and neck .

• symptomatic patients with bleeding.
• Painful lesions .
• Well localized lesions.
• Debulking is considered in hand and feet lesion
and intramuscular lesions in thigh and leg.
 AV MALFORMATIONS
• Fast flow connection without capillary bed.

• 40-60% patient present at birth ,equal gender predilection

• Epicenter of AVM is called nidus and contain feeding vessels .

• Consists of feeding vessels ,micro and macro fistulas and

ectatic veins

• Most common sites in descending order are intracranial,

limbs ,trunk and viscera
 SCHOBINGER STAGING SYSTEM
• Stage 1 (quiescent phase) from birth to adolescence,
asymptomatic
• Stage 2 (progressive phase)
AVM enlarged ,darkens with thrill and palpable pulse and
murmur on auscultation
Trauma, puberty and pregnancy leads to this stage
• Stage 3 (destructive phase)
• Ulceration, pain, bleeding and bone erosions

• Stage 4 (decompensation phase )

• Cardiac decomposition with heart failure
 Treatment
• Localized lesions can be excised and
reconstructed

• Large and diffuse lesions can be embolised or

superselected embolized and resected 24-48
hours later

• Counseling for recurrence and monitoring for

years for recurrence
 LYMPHATIC MALFORMATION
• Anomalous channels, vesicles and pouches filled
with lymph fluid.
• LMs never regress but expand and contract
depending on ebb n flow of lymphatic fluid
• Classified
• Microcystic
• Macrocystic
• Combined micro-macrocystic
• Macrocytic lesions are mostly located at head, neck and
axilla reffered to as cystic hygroma

• Head and neck LMs are also characterized by skeletal

hypertrophy

• Microcytic LMs are usually located on proximal

extremities.chest and axilla termed as lymphangioma
circumscriptum

• Combined malformations are seen on cheek lip and orbit

 Treatment

SURGICAL RESECTION
 Only way to potentially cure LMs
 Complete excision may not be possible in may
areas

PERCUTANEOUS SCLEROTHERAPY
 Recently gained popularity.
 Mainly effective in macrocystic variety.