A Case of Kasabach-Merritt syndrome with generalized hemorrhagic diathesis

ABSTRACT

Kasabach-Merritt syndrome is the association of thrombocytopenia, disorder of coagulation, spontaneous bleeding and enlargement of a hemangima or extensive hemangiomatosis, which can be often life threatening. We experienced a three years-old girl with suddenly enlarging hemangioma of right cheek, complicated by severe thrombocytopenia, consumptive coagulopathy and hemorrhagic cystitis. Generalized petechia and ecchymosis were noted on the overlying skin of hemangioma, trunk and extremities. But the study of MRI and angiography, hypevascular was located within the right masseter muscle extending to temporalis muscle and anterior to parotid gland. So selective embolization with PVA (polyvinyl alcohol) was performed through the distal branch of internal maxillary artery and facial artery. She was medicated with oral corticosterioid for 3 weeks. After embolizationn, blood flow to hemangioma and facial swelling were markedly decreased. Patient was followed up for 7 years and she showed normal contour of face and no bleeding diathesis.