血友病性跟腱挛缩症的外科治疗

Surgical treatment for hemophilia induced contracture of Achilles tendon

背景：血友病性跟腱挛缩症是血友病的常见下肢合并症,可导致下肢不等长、活动受限等症状。在全面的围手术期凝血因子替代治疗下手术矫正跟腱挛缩可极大改善患者的生活质量。目的：探讨外科治疗血友病性跟腱挛缩症的围术期处理方法、术式选择及疗效。方法：回顾性分析1998年6月至2014年2月收治的血友病性跟腱挛缩患者15例（15足）,均为男性;年龄18~41岁,平均24.6岁;左侧7例,右侧8例。其中甲型血友病11例,乙型血友病4例。病程11~84个月,平均30.2个月。术前根据美国矫形足踝协会（AOFAS）评分为（41.2±20.1）分,简明健康调查量表（SF-36量表）评分总分为（41.4±17.7）分。入院后行凝血因子预试验,制定围术期凝血因子替代治疗方案,均行跟腱Z型延长术。结果：手术时间55~75 min,平均61 min;术中使用止血带,出血量均〈20 ml,围术期均未输异体血。术后14例手术切口Ⅰ期愈合,1例伤口裂开,经换药后3周愈合。术后无感染、下肢深静脉血栓形成、神经损伤等并发症发生。患者均获得随访,随访时间为6个月~15年5个月,平均21个月。术后3个月及6个月随访时,AOFAS评分分别为（77.8±16.7）分和（79.8±12.5）分,SF-36量表评分分别为（73.6±13.8）分和（77.7±11.2）分,与术前相比均有统计学差异（P〈0.05）;术后3个月与术后6个月比较均无统计学差异（P〉0.05）。结论：手术治疗可以纠正血友病性跟腱挛缩患者的马蹄足畸形,改善足部负重及行走功能。术前行凝血因子预试验、围手术期行凝血因子替代治疗可降低术后大出血的发生率。

Background： Contracture of Achilles tendon is one of the common complications of hemophilia. It may result in leg length discrepancy and limitation of motion. The quality of life can effectively be improved by surgical treatment under periopera- tive clotting factor substitution therapy in patients with hemophilia induced contracture of Achilles tendon. Objective： To explore perioperative management and outcome of hemophilia induced contraeture of Achilles tendon. Methods： Between June 1998 and February 2014, 15 cases （15 feet） of hemophilia induced contracture of Achilles tendon underwent Achilles tendon lengthening, including 11 cases of hemophilia A and 4 cases of hemophilia B. Left feet was in- volved in 7 cases and right feet in 4 cases. All were males aged from 18 to 41 years （mean, 24.6 years）. Disease duration ranged from 11 to 84 months （mean, 30.2 months）. Preoperative American Orthopaedic Foot and Ankle Society （AOFAS） score was 41.24±20.1, and Short Form 36 Health Survey Scale （SF-36） score was 41.44±17.7. All patients were given clotting factors for preliminary experiment and clotting factor substitution therapy was performed perioperatively. Results： The operation time was 55-75 min （mean, 61 min）. Tourniquet was used in all cases and blood loss was less than 20 ml. Wounds healing was achieved in 14 patients and wound dehiscence occurred in one patient. No postoperative infection, deep vein thrombosis, or nerve injury occurred. The follow-up period ranged from 6 to 185 months in all the patients （mean, 21 months）. AOFAS scores were 77.84±16.7 and 79.8±12.5 at 3 and 6 months after surgery, and SF-36 scores were 73.64±13.8 and 77.7±11.2, respectively, which were significantly different from preoperative scores （P〈0.05）, but there was no significant difference between postoperative 3-month and 6-month scores （P〉 0.05）. Conclusions： For patients with hemophilia induced contracture of Achilles tendon, surgical treatment can correct deformity, release pain and improve function. Clotting factor preliminary experiment before surgery and substitution therapy in periop- erative period can reduce the risk of severe postoperative hemorrhage.