血友病足踝部病变的外科治疗

SURGICAL TREATMENT FOR HEMOPHILIA INDUCED LESIONS OF FOOT AND ANKLE

目的探讨外科治疗血友病足踝部病变围术期处理方法、术式选择及疗效。方法 1998年6月-2012年2月,收治血友病足踝部病变男性患者10例(12足)。年龄13～41岁,平均22.6岁。其中甲型血友病9例(11足),乙型血友病1例(1足)。单足受累8例,双足2例;左侧3足,右侧9足。病程5～84个月,平均32.2个月。术前根据美国矫形足踝协会(AOFAS)评分为(43.2±21.1)分,简明健康调查量表(SF-36量表)评分总分为(45.4±20.0)分。入院后给予凝血因子2 000～3 500 U行预试验,围术期行凝血因子替代方案治疗。行踝关节融合术4例(4足),单纯跟腱延长/肌腱转移术7例(8足);其中1例左足行单纯跟腱延长术,右足行踝关节融合术。结果手术时间65～265 min,平均141.1 min;除1例术中出血400 mL、术中回输200 mL自体血外,其余患者出血量均<50 mL,围术期均未输血。患者切口均Ⅰ期愈合,术后无感染、下肢深静脉血栓形成、神经损伤等并发症发生。患者均获随访,随访时间6个月～14年3个月,中位时间22个月。行踝关节融合术患者末次随访时X线片检查示踝关节完全融合。术后6个月及末次随访时,AOFAS评分分别为(78.8±14.7)分和(75.8±14.5)分,SF-36量表评分总分分别为(76.6±13.1)分和(75.5±13.2)分,两指标与术前比较,差异均有统计学意义(P<0.05);术后6个月与末次随访时比较,差异均无统计学意义(P>0.05)。结论对于血友病足踝部病变患者,手术治疗可以缓解足踝部疼痛,改善功能。术前行凝血因子预试验,围手术期行凝血因子替代方案,可降低术后大出血发生率。

Objective To explore perioperative management and postoperative effectiveness of hemophilia induced lesions of the foot and ankle. Methods Between June 1998 and February 2012, 10 cases （12 feet） of hemophilia induced lesions of the foot and ankle were treated with surgery, including 9 cases （11 feet） of hemophilia A and 1 case （1 foot） of hemophilia B. Single foot was involved in 8 cases and both feet in 2 cases, including 3 left feet and 9 right feet. All were males, aged from 13-41 years （mean, 22.6 years）. Disease duration was 5-84 months （mean, 32.2 months）. Preoperative American Orthopaedic Foot and Ankle Society （AOFAS） score was 43.2 ± 21.1. Short Form 36 Health Survey Scale （SF-36） score was 45.4 ±20.0. All patients were given clotting factors （2 000-3 500 U） for pre-experiment and clotting factors substitution therapy was performed perioperatively. Four cases （4 feet） underwent arthrodesis, and 7 cases （8 feet） underwent Achilles tendon lengthening/tendon transposition （1 patient underwent tendon lengthening on the left foot and arthrodesis on the right foot）. Results The operation time was 65-265 minutes （mean, 141.1 minutes）; 1 case had 400 mL blood loss and 200 mL autogenous blood transfusion, the other cases had less than 50 mL blood loss and no blood transfusion. Wounds healed by first intention in all patients, no postoperative infection, deep vein thrombosis, or other complications occurred. All cases were followed up 6 months to 14 years and 3 months （median, 22 months）. The X-ray films at last follow-up showed the patients undergoing arthrodesis obtained complete joint fusion. AOFAS scores at postoperative 6 months and last follow-up were 78.8 _ 14.7 and 75.8 ± 14.5, respectively; SF-36 scores were 76.6± 13.1 and 75.5± 13.2, respectively; and significant differences were found when compared with preoperative scores （P 〈 0.05）, but no significant difference between postoperative 6 months and last follow-up （P 〉 0.05）. Conclusion For patients with hemophilia induced lesions of the foot and ankle, surgical treatment could relieve foot and ankle pain and improve the function. Clotting factors pre-experiment at preoperation and substitution therapy at perioperation can reduce the risk of severe postoperative hemorrhage.