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