血友病性骨关节病变的外科治疗策略

Surgical treatment in heamophilic patients with musculoskeletal disorder

目的探讨血友病性骨关节病变的外科治疗方法及策略。方法1996年1月至2015年6月，手术治疗血友病性骨关节病变患者120例，年龄6-61岁，平均（29．7±12）岁。甲型血友病109例，乙型血友病11例。表现为关节内出血及血友病关节炎87例（膝关节受累63例、髋关节受累29例、踝关节受累11例），血友病假瘤21例，血友病并发肌肉及肌腱出血15例。根据手术创伤大小，选择凝血因子替代治疗方案。记录血友病性骨关节病变的临床表现、手术种类、术后疗效及并发症发生情况。结果120患者共接受166例次手术，其中41例（34．2％，41／120）同时接受了多部位手术。手术包括关节置换103例次（62．0％，103／166）、假瘤切除及重建手术21例次（12．7％，21／166）、跟腱及腓肠肌膜松解15例次（9．0％，15／166）、踝关节融合12例次（7．2％，12／166）、膝屈曲畸形矫形术13例次（7．8％，13／166）、其他2例次（1．2％，2／166）。围手术期共出现30例次并发症（18．1％，30／166），包括凝血功能相关并发症8例次（4．8％，8／166）、手术相关并发症12例次（7．2％，12／166）、伤口相关并发症10例次（6．0％，10／166）。9例因并发症行再手术治疗（7．5％，9／120）。结论血友病性骨关节病变最常累及膝关节、髋关节及肌肉系统。在合理补充凝血因子的条件下，对血友病关节炎、血友病假瘤进行手术治疗能有效缓解症状，获得满意的临床疗效，但血友病患者围手术期并发症发生率较高。

Objective To study the surgical treatment strategy for heamophilic arthropathy and musculoskeletal apparatus. Methods A total of 120 heamophilic patients underwent 166 primary operations from January 1996 to June 2015 in Peking Union Medical College Hospital, with the average age of 29.7± 12 years （from 6 to 61 years）. Hemophilic type A accounted for 109 patients and hemophilic type B accounted for 11. Eighty-seven patients presented with bleeds within the joints, with 63 cases for knee involved, 29 cases for hip, 11 cases for ankle. Fifteen patients presented with intramuscular bleeds, 21 patients with heamophilic pseudotumors. Strategy of clotting factor replacement therapy was designed according to the different level of operation procedure. The clinical manifestation, operative strategy, clinical outcome and complications were retrospectively recorded. Results 41 cases （34.2%） of patients underwent surgeries for more than one location. Totally, 166 procedures were performed for 120 patients. There were 103 procedures of joint arthroplasty （62.0%, 103/166）, 21 procedures of pseudotumor resection （12.7%, 21/166）, 15 procedures of tendon lengthening, 12 procedures of ankle arthrodesis, 13 procedures for knee flexion contracture. There were 30 cases of postoperative complications, with the rate of 18.1% （30/166）. The coagulation related complication was 4.8% in this group （8/166）. The surgical procedure related complication was 7.2% （12/166）. All the preoperative symptoms were relieved during the follow-up. Conclusion Surgical treatment was effective for the heamophilic arthropathy and lesion of museuloskeletal apparatus, under the reasonable clotting factor replacement therapy. The incidence of perioperative complication in heamophilic patients is higher than that in non-hemophilic patients. Closely inspection was inevitable for perioperative treatment in hemophilic patients.