Abstract :
Kasabach-Merritt Syndrome (KMS) is a rare and life threatening complication presenting as thrombocytopenia with consumptive coagulopathy in patients with large capillary or cavernous hemangioma. It is associated with a mortality of 30-40%[1,2]. We report a case of a 16 year old boy with Klippel-Trenaunay Syndrome (KTS) who sustained a right supra condylar femur fracture complicated by KMS. His thrombocytopenia and coagulopathy was managed followed by rush rodding of femur fracture. The preoperative management of KMS was demanding and needed constant monitoring due to the risk of haemorrhage and thrombosis. This is the first case of surgical management of a long bone fracture complicated by KMS in children to be reported.
Keyword :
Kasabach-Merritt Syndrome, Klippel-Trenaunay Syndrome, Hemangioma, Coagulopathy