Vascular Anomalies including management of hemangioma

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.