By Disease Name > Blue Rubber Bleb Nevus Syndrome

Hemangioma

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Strawberry Hemangiomas

•at birth the major cutaneous vasculature is already formed, but the microvasculature continues to reorganize for the first four months of life
•the proliferative phase of hemangioma neoplasms coincides with the postnatal period of reorganization
•

treatment:

•prednisone (2mg/kg/day) often long course (8 to 10 months) (slow taper to avoid rebound)
•interferon alpha (adverse effect = spastic diplegia)
•ulcerating – Regranex gel Qam (under dressing or barrier cream); metrogel Qpm  (healing ≈ 21days)
•ILK 10 to 20/cc (small volumes)
•propranolol

 

 

NEONATAL HEMANGIOMATOSIS

•AKA Diffuse Congenital Hemangiomatosis
•sporadic; multiple 0.2cm – 2cm hemangiomas
•visceral involvement varies form none to disseminated; most commonly involves the liver (64%)
•widespread visceral involvement may lead to death by high-output cardiac failure
•lesions begin to involute in second half of first year of life (90% have involuted by age 9)
•treatment:  PO steroid, interferon-alpha (but 10% incidence of spastic diplegia)

 

see Kasabach-Merritt Phenomenon

 

Cavernous Hemangioma

•two rare conditions in which numerous cavernous hemangiomas occur (both sporadic):

Maffucci syndrome

•sporadic;  pathogenesis unknown
•enchondromas (= benign cartilaginous tumors):
•secondary fractures
•cause severe dysformity
•may develop into chondrosarcomas
•numerous cavernous hemangiomas (esp. distal extremities)
•ddx:  Proteus syndrome,  blue rubber bleb nevus syndrome

 

blue rubber bleb nevus syndrome

•sporadic;  pathogenesis unknown
•cavernous hemangiomas of skin and GI tract
•GI bleeding and anemia, therefore CBC, stool guiacs, endoscopy
•ddx:  Maffucci syndrome, multiple glomus tumors, diffuse congenital Hemangiomatosis