Menendez L R, Thommen V D
Department of Orthopaedic Surgery, University of Southern California, Los Angeles 90033, USA.
Clin Orthop Relat Res. 1995 Jul(316):185-8.
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.
与巨大血管瘤相关的消耗性凝血病性卡萨巴赫-梅里特综合征，对于发生受累肢体长骨骨折的患者来说是一个潜在的危及生命的问题。在这种综合征中，血小板消耗是由于它们被截留在大血管瘤的血窦和上皮样腔隙中。继而发生继发性纤维蛋白溶解，导致血小板减少、纤维蛋白原血症降低以及纤维蛋白降解产物增加，这可导致弥散性血管内凝血。即使是轻微创伤后也可能导致大量出血。在此类患者中，长骨骨折的手术治疗，包括用于骨牵引的皮牵引针置入，可能是禁忌的；可能需要非手术治疗。对于患有巨大血管瘤并伴有长骨骨折的患者，必须怀疑卡萨巴赫-梅里特综合征，在进行手术治疗或经皮骨牵引针置入之前，应进行适当的凝血研究。与血小板减少相关的血细胞比容和纤维蛋白原水平降低以及凝血酶原时间和部分凝血活酶时间延长，应提醒骨科医生注意卡萨巴赫-梅里特综合征的可能性，并应立即进行血液学会诊。如果由于存在无法控制的弥散性血管内凝血的可能性而认为手术治疗过于危险，唯一谨慎的选择可能是在对凝血参数进行适当的药物治疗后进行闭合复位和石膏固定。
