血友病合并骨折患者的特点及围手术期治疗

摘要

Objective:To investigate the characteristics and perioperative management of hemophilia patients with fracture.Methods:Retrospectively, we analyzed 8 patients with hemophilia combined with fracture, who were admittted to our department from 2005 to 2013.Six patients were with hemophilia A and two with hemophilia B;Based on the severity of hemophilia, 2 cases were light, 3 moderate and 3 severe;Based on the location of fracture, 4 cases were femoral neck fractures, 1 femoral intertrochanteric fracture, 1 bilateral distal femur fractures, 1 tibiofibula fracture, and 1 humerus intercondylar fracture. Blood coagulation factor replacement therapy was conducted preoperatively, intraoperatively and postoper-atively, All the patients underwent closed or open reduction and internal fixation or joint replacement. Also, we analyzed the perioperative complications and observed whether the fracture healed.Results:The average age was 33.5 years (14 to 47 years); In 6 cases, fractures occurred at femur, accounting for 75%of all the fractures; Femoral neck fracture was treated by closed reduction and hollow screws fixation;Femoral intertrochanteric fracture, distal femur fracture, and tibiofibula fracture were treated by open reduction and internal fixation with plate;Humerus intercondylar fracture was treated by elbow joint replacement.Intraoperative bleeding was from 50 to 600 mL, an average of 262 mL;Perioperatively, the average use of FⅧ/activated prothrombin complex concentrates ( APCC) was 358 U/kg (125 to 554 U/kg) .Postoperatively, poor wound healing was observed in 2 patients, and the condition improved after symptomatic treatment; In patients with internal fixation, all the fractures united, and the average hea ling time was 14 weeks.No complications such as fixation loosening or rupture occurred after internal fixation.Conclusion:Hemophilia combined with fracture mainly occurred in the young, and the site of fracture was given priority to femur.With perfect preoperative preparation, on the basis of the replace-ment therapy, hemophilia combined with fractures was safe for surgical treatment, and postoperative frac-tures healing wasgood.But the risk of poor wound healing was high.%目的：探讨血友病合并骨折患者的特点及围手术期治疗方法。方法：回顾性分析2005年至2013年在北京大学人民医院创伤骨科住院治疗的8例血友病合并骨折患者，甲型血友病6例，乙型血友病2例；其中轻型2例，中型3例，重型3例；按照骨折部位不同分为股骨颈骨折4例，股骨粗隆间骨折1例，双侧股骨远端陈旧性骨折1例，胫腓骨骨折1例，肱骨髁间骨折1例；术前、术中、术后进行凝血因子替代治疗；所有患者均行闭合／切开复位内固定或者关节置换术。分析围手术期并发症并随访骨折愈合情况。结果：患者平均年龄为33．5岁（14～47岁）；6例骨折发生于股骨；股骨颈骨折行闭合复位空心钉内固定，股骨粗隆间骨折、股骨远端骨折、胫腓骨骨折行切开复位钢板内固定，肱骨髁间骨折行肘关节置换术，术中出血50～600 mL，平均262 mL；围手术期FⅧ／凝血酶原复合物平均使用量为358 U／kg（125～554 U／kg）；术后2例患者出现伤口愈合不良，经对症处理后好转；行内固定治疗的患者，术后均出现骨性愈合，平均愈合时间为14周，术后无内固定松动及断裂等并发症发生。结论：血友病合并骨折主要发生于青年，骨折部位以股骨为主；完善术前准备，在替代治疗的基础上，可以安全地进行手术治疗，术后骨折愈合良好；术后出现伤口愈合不良的风险大。