Vascular anomalies are classified as hemangiomas or vascular malformations based on their clinical course, biological behavior, and histological features. Hemangiomas are vascular tumors that grow rapidly during infancy through endothelial proliferation and then slowly regress. Vascular malformations are present at birth and grow proportionately with the patient. They never regress and consist of abnormal vascular channels. Diagnosis involves clinical history, examination, imaging like ultrasound or MRI, and biopsy if needed. Management depends on type, location, growth phase and involves observation, medical therapies like corticosteroids or propranolol, procedures like sclerotherapy or laser, or surgery.

1. VASCULAR ANOMALIES

2. 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

3. ISSVA CLASSIFICATION 1996

4. • 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

5. 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.

6. Vascular Malformations
SIMPLE
• Capillary
• Venous
• Arterial
• Lymphatic
COMBINED
• Capillary lymphatic
• Capillary venous
• Capillary lymphatic
venous
• Arteriovenous
• Capillary Arteriovenous
• Lymphatic
arteriovenous
• AV fistulae

7. Vascular Malformations
FAST FLOW
• Arterial Malformation
• AV fistula
• AV malformation
• Capillary arteriovenous
malformation
• Lymphatic arteriovenous
malformation.
SLOW FLOW
• ALL other lesion
Fast flow lesions fill in less that 2 seconds

8. 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

9. 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

10. CLINICAL COURSE
• Characteristic 3 phase growth pattern .
• Proliferative phase
• Involuting phase
• involuted phase

11. 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.

12. • 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

13. COMPLICATIONS
• ULCERATION
 Leads to infection ,pain ,bleeding
 Mostly during period of rapid growth
 Common in large lesion of lip ,perineum and
intertrigenous regions.

14. • Eyelid hemangioma can
cause visual disturbance
strabismus and even
amblypia
• Hemangioma in neck and
mandible can be assocaited

15. 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.

16. RICH
• Solitary raised or grey or
voilecious lesion with
ectasia,radialveins,
• Telengectesia and pale
surrounding hallo
• Accelerated regression
growth pattern complete
by (6-14 months)
• High output cardiac failure
NICH
• Well circumscribed lesion
with pink,blue or purple
hue,central talengectesia
and pale rim
• Grows proportionately to a
child.

17. DIAGNOSIS
• Detailed clinical history
• Examination
• U/s (operator depends)
• MRI (gold standard)

18. ANOMALIES WITH HEMANGIOMA

22. MANAGEMNET
• Urgency of treatment (life threatening /less
threatening )
• Location and associated symptoms
• Size
• Growth phase
• Age of the patient at evaluation

23. 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

24. MANAGEMNET
• MEDICAL MANAGEMENT
1. Corticosteroids
2. Propranol
3. Alpha interferon
4. Vincristine

25. 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

26. 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 .

27. 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

28. PROPRANOLOL
• Adverse effects are hypotenstion, hypoglycemia,
bradycardia
• gradual dose increment and tapering and patient
education regarding side effects is important part
of propranolol treatment

29. 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 corticosteriods use
• Dose is 2-3mU/m2 and increased with child
growth
• Interferron is effective in kassabach-merritt

30. 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 assocaited with IH but also with kaposifrom
hemangioendothelioma and tufted angioma
• No platelete transfusion untill bleeding or surgical procedure
indicated
• Do not use heparin it aggravates situation .
• Fever is common side effect (acetaminophen)

31. 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
.

32. SURGICAL MANAGMENT
• Indications for surgery are
• For residual scarring
• Localized lesions for cosmetic reasons
• Persistent ulceration and bleeding
• School going children for normal appearance

33. 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 .

34. 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

35. CAPILLARY MALFORMATIONS
• Most common anomalies with 3 in 1000 live
births and equal gender distribution
• Present at birth like red or pink ntradermal
discoloration
• True CMs thicken ,darken and becmoes
nodular with age
• A variant called macular stain and named

36. 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 )

37. 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

38. 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

39. Management
• MRI is extremely useful for diagnosis and
combined with venography helps in surgical
planning
• Coagulation profile should be checked due to
coagulopathy and rsik of DIC is there folowing
trauma and intervention
• Percutaneous sclerotherapy is first line treatment
• Compression stockings and aspirin for phlebitis
are adjuncts

40. 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.

41. AV MALFORMATIONS
• Fast flow connection without capillary bed.
• 40-60% patient present at birth ,equal gender
predilection
• Epicenter of AVM is called nidus
• Consists of feeding vessels ,micro and macro
fistulas and ectatic veins

42. 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

43. Treatment
• Localized lesions can be excised and
reconstructed
• Large and diffuse lesions can be embolised or
super selected embolized and resected 24-48
hours later
• Counseling for recurrence and monitoring for
years for recurrence

44. 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

45. • 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

46. 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.