Hemangioma and vascular malformations

TypeHemangiomaVascular malformations
1. DefinitionHamartoma endothelial cell proliferationErrors in blood vessel morphogenesis
2. ElementsIncreased number of capillaries/ sinusoidal spacesMix of arteries, veins and capillaries
3. Classification– Superficial (capillary)
– Deep (cavernous)
– Compound (both)
1. Simple lesion
a) Low flow:
– Capillary malformation
– Venous malformation
– Lymphatic malformation (Lymphangioma)
b) High flow:
– Arterial malformation

2. Combined lesions
– AV malformation
– Lymphovenous malformation
4. GrowthRapid congenital growthGrows with patient
5. BoundariesCircumscribed, rarely affecting bonePoorly circumscribed, may affect bone
6. Thrill & bruitNoMay have
7. Involution (shrink)Spontaneously
Responds to oral steroids
Does not involute
8. ResectionPersistent lesions resectableDifficult – surgical hemorrhage
9. RecurrenceUncommonCommon
Table comparing hemangioma with vascular malformation

Hemangioma

Clinical:

  • Purple, flat/nodular lesion – blanch on pressure
  • Painless swelling
  • Resorb overlying bone
  • Bleeds
  • Loosens teeth

Associated syndromes:

Diagnosis: Angiogram hemangioma – in head and neck, inject radiopaque material into external carotid artery

Radiology:

  • Well circumscribed radiolucency
  • Unilocular or multilocular (honeycomb appearance)

Histology:

  • Fibrous connective tissue network – contains endothelial lined capillaries containing RCB
  • Sinusoids with endothelial cells

NB:

1. Infantile hemangioma

  • Superficial overlying patch of redness
  • Appears weeks/months after birth
  • Natural course:
    • 1. Proliferate – first year
    • 2. Involuting – few years
    • 3. Involuted – most resolved by age 10

2. Congenital hemangioma

  • Present at birth – blue/gray hue with pale halo
  • RICH – Rapidly involuting congenital hemangioma
    • Reach maximum size by birth and involute within 12-18 months
  • NICH – Non involuting congenital hemangioma
    • Continue to grow in size in proportion to patient and do not involute
  • PICH – Partially involuting congenital hemangioma
    • Evolve from RICH to persistent NICH like lesions
  • Complications: Ulceration, bleeding, infection, obstruct/displace organs

Management:

  1. Watchful neglect – if congenital, most involute
  2. Surgical excision – contraindicated in large lesions
    • Need for surgery: Trauma, hemorrhage, ulceration, 2ry infection, psychosocial effect
  3. Laser therapy
  4. Pharmacologic – steroids, propranolol, vincristine
  5. Sclerotherapy – induction of fibrosis to control bleeding with absolute (95%) alcohol and then surgical removal
    • Sclerosants: Doxycycline, bleomycin, OK-432, sodium tetradecyl sulphate
  6. Embolic therapy
  7. Cryotherapy

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