Kasabach-Merritt syndrome complicating treatment of a closed femoral fracture.
The Kasabach-Merritt syndrome of consumptive coagulopathy associated with massive hemangiomas is a potentially life-threatening problem in patients who sustain long bone fractures of the involved extremities. In this syndrome, platelet consumption is caused by their sequestration in the sinusoids and epitheloid chambers of large hemangiomas. Secondary fibrinolysis then occurs with resulting thrombocytopenia, hypofibrinogenemia, and increased fibrin degradation products that can lead to disseminated intravascular coagulation. This can result in massive bleeding even after minor trauma. In such patients, operative management of long bone fractures, including the placement of cutaneous pins for skeletal traction, may be contraindicated; nonoperative management may be necessary. Kasabach-Merritt syndrome must be suspected in patients with large hemangiomas and associated long bone fractures, and appropriate coagulation studies should be obtained before operative management or placement of percutaneous skeletal pins. Decreased hematocrit and fibrinogen levels associated with thrombocytopenia and prolonged prothrombin time and partial thromboplastin time should alert the orthopaedist to the possibility of Kasabach-Merritt syndrome, and prompt hematologic consultation should be obtained. If surgical treatment is deemed too dangerous because of the possibility of uncontrollable disseminated intravascular coagulation, the only prudent option may be a closed reduction and cast application after appropriate medical management of coagulation parameters.
Menendez
L R
LR
Department of Orthopaedic Surgery, University of Southern California, Los Angeles 90033, USA.
Thommen
V D
VD
D001858:Bone Nails; D000075202:Contraindications; D004211:Disseminated Intravascular Coagulation / Q000150:complications*; D005260:Female; D005264:Femoral Fractures / Q000150:complications* / Q000601:surgery*; D005593:Fracture Fixation, Internal; D005596:Fractures, Closed / Q000150:complications* / Q000601:surgery*; D006391:Hemangioma / Q000150:complications*; D006801:Humans; D008875:Middle Aged; D013577:Syndrome