Mukhtar Iftikhar A, Letts Merv
Division of Orthopedic Surgery, Children's Hospital of Eastern Ontario, Ottawa, Canada.
J Pediatr Orthop. 2004 Jan-Feb;24(1):87-91.
The Kasabach-Merritt syndrome of consumptive coagulopathy associated with massive hemangiomas is a potentially life-threatening problem in patients with a pathologic fracture of the osseous lesion. This can result in massive bleeding even after minor trauma. In such patients, operative management of long bone hemangiomatous lesions, including percutaneous needle aspirations, may be contraindicated, and nonoperative management may be preferable. Kasabach-Merritt syndrome must be suspected in patients with large hemangiomas with associated bone lesions, and appropriate coagulation studies should be obtained before any operative management. Review of the world English literature on Kasabach-Merritt syndrome has revealed that the most common pathologic fractures occur in the vertebral bodies. An additional case report of a child with a pathologic fracture and deformity of his radius has been documented. Decreased hematocrit and fibrinogen levels associated with thrombocytopenia and a prolonged prothrombin time and partial thromboplastin time in association with bone hemangioma should alert the orthopaedist to the possibility of Kasabach-Merritt syndrome.
与巨大血管瘤相关的消耗性凝血病的卡萨巴赫-梅里特综合征，对于患有骨病变病理性骨折的患者来说是一个潜在的危及生命的问题。这甚至在轻微创伤后都可能导致大量出血。在此类患者中，长骨血管瘤性病变的手术治疗，包括经皮针吸，可能是禁忌的，非手术治疗可能更可取。对于患有巨大血管瘤并伴有骨病变的患者，必须怀疑卡萨巴赫-梅里特综合征，并且在任何手术治疗前都应进行适当的凝血研究。对世界英文文献中关于卡萨巴赫-梅里特综合征的综述表明，最常见的病理性骨折发生在椎体。已有一份关于一名儿童桡骨病理性骨折和畸形的病例报告。与骨血管瘤相关的血细胞比容和纤维蛋白原水平降低，伴有血小板减少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。
