Objective: To study the orthopedic treatment strategy for hemophilia complicated with musculoskeletal disorders as well as the peri-operative consumption of clotting factor. Methods: Total 338 orthopedic surgeries were performed for 261 patients, average age of 30.6 y (6-65 y) , with hemophilia between January 1996 and December 2019 at our institute. Two hundred and twenty-six patients presented with bleeds within the joints. Sixty-one patients presented with intramuscular bleeds, 45 presented with hemophilic pseudotumors, and six presented with miscellaneous complaints. Strategy of clotting factor replacement therapy was designed as per differences in the level of the operation procedure. Information regarding clinical manifestation, operative strategy, clotting factor consumption, and re-operation for complications was retrospectively recorded. The costs for multiple joint procedure and single joint procedure were studied. Results: We found that 270 of the 338 surgical procedures were major surgical procedures (79.9%) . There were 203 procedures of joint arthroplasty (60%) . Fourteen patients underwent reoperations for local recurrence (4.2%) . The average factor Ⅷ consumption before the surgery was 44.4 ± 8.1 IU/kg. The average FⅧ consumption within postoperative 2 weeks was 40 962 IU (647±177 IU/kg) . Seven type A hemophilic patients developed F Ⅷ inhibitor following the surgical procedure, with an average level of 13.7±11.2 BU/mL. Sixty-eight patients underwent multiple joint procedures under one anesthesia session (26%) . There was no significant difference in the factor consumption between the multiple joint procedure and single joint procedure. Conclusions: Surgical treatment was found to be effective for hemophilic arthropathy and lesion of the musculoskeletal apparatus, with the clotting factor replacement therapy. Multiple joint procedures under one anesthesia were more cost effective for patients with hemophilia, with less factor consumption than staged single joint procedure.
目的： 探讨血友病性骨骼肌肉病变的外科手术治疗方法及围手术期凝血因子消耗情况。 方法： 回顾性分析1996年1月至2019年12月在中国医学科学院北京协和医院骨科因骨骼肌肉病变接受手术治疗的261例血友病患者。根据手术创伤大小选择凝血因子替代治疗方案。记录病变的临床表现、手术种类、围手术期凝血因子消耗、输血率及再手术情况，对比单次麻醉下单部位手术与多部位手术的凝血因子消耗及费用。 结果： 261例患者均为男性，平均年龄为30.6（6~65）岁。血友病A 235例，血友病B 26例；重型（因子活性<1%）141例，中间型（因子活性1%~5%）91例，轻型（因子活性>5%）29例。共施行手术338例次（关节内出血及关节炎226例次、血友病并发肌腱肌膜出血挛缩61例次、血友病假瘤45例次、其他6例次）。338例手术中，大型手术270例（79.9%），关节置换203例（60.1%）。因手术部位病变复发再手术14例，再致残率为4.2%。共55例（21.1%）患者在围手术期接受红细胞输注。血友病A患者术前FⅧ的平均用量为（44.4±8.1）IU/kg，术后2周内FⅧ总用量平均为（647±177）IU/kg（40 962 IU）。术后3个月康复期内，6例（2.3%）患者发生手术部位再出血。7例患者术后检出FⅧ抑制物，平均抑制物水平为（13.7±11.2）BU/ml。单次麻醉下多关节手术组（68例）与单关节手术组凝血因子消耗量及费用差异无统计学意义。 结论： 针对血友病性骨骼肌肉病变，在合理补充凝血因子条件下，根据病变的具体情况选择合适的手术治疗方案是有效的缓解症状的方法。单次麻醉及凝血因子替代治疗下进行多关节手术不增加凝血因子消耗，具有更佳的成本效益。.
Keywords: Hemophilia; Multiple joint procedure; Muscularskeletal disorder; Plotting factor; Surgical treatment.