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血友病性假肿瘤合并破坏性骨关节炎的手术治疗

Surgical management of hemophilic pseudotumor complicated by destructive osteoarthropathy.

作者信息

Zhai Jiliang, Weng Xisheng, Zhang Baozhong, Peng Hui-ming, Bian Yan-yan, Zhou Lei

机构信息

Department of Orthopaedic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.

出版信息

Blood Coagul Fibrinolysis. 2015 Jun;26(4):373-7. doi: 10.1097/MBC.0000000000000260.

Abstract

Hemophilic pseudotumor gradually erodes bone and induces fracture or deformity, causing joint dysfunction or destructive osteoarthropathy. Reports about surgery for hemophilic pseudotumor complicated by destructive osteoarthropathy are scarce. The object of this study was to evaluate the results and complications of surgical management for patients of pseudotumor complicated by destructive osteoarthropathy. We retrospectively reviewed records from July 1996 to July 2013, and found eight patients with pseudotumor complicated by destructive osteoarthropathy. We recorded their demographic data, time of surgery, amount of blood loss and transfusion, bone union, and complications. Seven patients were diagnosed with hemophilia A and one with hemophilia B. The mean age at surgery was 31.9 ± 8.3 years. Two of the eight underwent excision of the pseudotumor and metallic fixation, one had amputation, and five underwent autogenous or exogenous bone grafting and fixation with an absorbable screw. The median operating time was 170 min (135-315 min). The median amount of intraoperative blood loss was 1350 ml (100-4000 ml). The amount of red blood cells, plasma, and whole blood transfusion after surgery were 0-24 units, 0-2000 ml, and 0-4600 ml, respectively. After a median follow-up of 75 months, the numbers of pseudotumor recurrence, fracture nonunion, coagulation factor inhibitor formation, and wound complications were one, one, two, and four, respectively. Surgery is an effective treatment for hemophilic pseudotumor complicated by destructive osteoarthropathy. However, the incidences of wound infection, coagulation factor inhibitor formation, hemophilic pseudotumor recurrence, and fracture nonunion are high.

摘要

血友病性假肿瘤会逐渐侵蚀骨骼并导致骨折或畸形,进而引起关节功能障碍或破坏性骨关节炎。关于血友病性假肿瘤合并破坏性骨关节炎手术治疗的报道较少。本研究的目的是评估手术治疗血友病性假肿瘤合并破坏性骨关节炎患者的疗效及并发症。我们回顾性分析了1996年7月至2013年7月的记录,发现8例血友病性假肿瘤合并破坏性骨关节炎患者。我们记录了他们的人口统计学数据、手术时间、失血量和输血量、骨愈合情况及并发症。7例患者被诊断为甲型血友病,1例为乙型血友病。手术时的平均年龄为31.9±8.3岁。8例患者中,2例行假肿瘤切除及金属内固定,1例行截肢术,5例行自体或异体骨移植并用可吸收螺钉固定。中位手术时间为170分钟(135 - 315分钟)。术中中位失血量为1350毫升(100 - 4000毫升)。术后红细胞、血浆和全血的输血量分别为0 - 24单位、0 - 2000毫升和0 - 4600毫升。中位随访75个月后,假肿瘤复发、骨折不愈合、凝血因子抑制物形成及伤口并发症的例数分别为1例、1例、2例和4例。手术是治疗血友病性假肿瘤合并破坏性骨关节炎的有效方法。然而,伤口感染、凝血因子抑制物形成、血友病性假肿瘤复发及骨折不愈合的发生率较高。

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