Indian Journal of Orthopaedics Surgery


Kasabach-Merritt syndrome complicating femur fracture in a 16 year old boy with Klippel Trenaunay Syndrome


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Author Details: Jayakrishnan Kelamangalathu Narayana Kurup, Imthias Vazhayil Kottamtavida, Hitesh Hasmukhlal Shah

Volume : 2

Issue : 3

Online ISSN : 2395-1362

Print ISSN : 2395-1354

Article First Page : 310

Article End Page : 312


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.

Keywords:
Kasabach-Merritt Syndrome, Klippel-Trenaunay Syndrome, Hemangioma, Coagulopathy