Vascular Anomalies

Question 1

Hemangioma

Answer 1

1. Infantile hemangioma (appear during the first 8wks of life)
2. Congenital hemangioma (present at birth) - 2 types:
   i) RICH - rapidly involuting congenital hemangioma
   ii) NICH - noninvoluting congenital hemangioma

Question 2

Clinical presentation of infantile hemangioma

Answer 2

Appear postnataly
After birth within 8wks of life
3 phases 
1. Rapid growth (6-12months)
Firm lesion, superficial, erythematous - strawberry hemangioma
2. Involution (1 - 10 yrs)
Shrinks and fading
3. Involuted (5 yrs+)
Redundant atrophic skin 
Yellowish discoloration
Telangiectasia

Question 3

Infantile hemangioma is asssociated with what syndrome?

Answer 3

PHACE syndrome
Posterior fossa brain anomalies
Hemangioma -cervical segmental hemangioma
Arterial anomalies
Cardiac defects / coarctation of aorta
Eye anomalies

Question 4

Mx of hemangioma

Answer 4

Onservation - monthly during the rapid growth, annually during involution stage

Intervention - lesion destructs, distorts and obstructs other structures

1. Intralesional injection for <2cm
   
   • steroid (triamcinolone 3mg/kg)
   • bleomycin
2. Systemic steroid / intralesional vincristine - large destructive lesions
3. Surgery - when vital structures are involved, or when involuted stage for revision/cosmetic

Question 5

Congenital hemangioma

Answer 5

Present at birth
2 types
RICH - rapidly involuting CH
NICH - noninvoluting CH

Question 6

Vascular malformation types

Answer 6

Can be divided into

1. Types of flow
2. Types of vessels

Low flow

• capillary
• lymphatic
• venous

High flow
- ateriovenous malformation(AVM)

Question 7

Mx of vascular malformation

Answer 7

Ix with MRI to determine the type of flow and extent
For intrabony lesions - biopsy of multilocular radiolucent lesion -> bleeding/pulsatile bleeding (avm)

Tx. 
Small lesion- excise surgically
Large lesion- combination
   Low flow - intralesional injection
   High flow - embolization
Surgery for debulking

Question 8

Intrabony vasc malformation

Answer 8

Hemangioma
Venous malformation
AVM

Presentations:
Asymptomatic 
Mobility of teeth
Gingival bleeding
Bruit/pulsation 
Radiolucency multilocular - honeycomb
Large lesion can cause chronic bony expansion with sunburst appearance

Question 9

Sturge webber syndrome

Answer 9

Portwine stain
- dermal capillary hemangioma usually affecting trigeminal nerve distribution

Leptomeningeal system - seizures, hemiplegia

Ocular - retinal detachment, glaucoma, blindness

Question 10

What is cystic hygromas?

Answer 10

Lymphatic malformations - macrocystic type

Often occur in neck due to loose connective tissue (posterior triangle)

Soft fluctuant mass

Mx:
Becos they are easily infected - antibiotic
Large lesion -combination therapy
Intralesional injection of sclerotic agents (ethanol, bleomycin)
Surgery
Trachy if causing airway obstruction

Recurrence os highhhh!!!!

Question 11

What is Schobinger staging of AVM

Answer 11

4 stages

1. Quiescent stage - asymptomatic
2. Expansion stage - asymptomatic expansion
3. Destructive expansion - pain, bleeding, tissue necrosis
4. Decompensation - due to cardiac failure