血友病性踝关节病患者的围术期管理策略

Perioperative management of hemophilic ankle disorders

背景:血友病是一种凝血障碍的遗传性疾病,出血累及小腿三头肌时可引起肌肉痉挛,并发跟腱挛缩,引起马蹄足畸形,严重影响关节功能和生活质量。目的:探讨血友病性踝关节病患者围术期的管理方法。方法:总结1996年1月至2018年3月收治的血友病性踝关节病患者31例。术前与血液内科合作,完成预实验,即监测凝血因子活性、活化部分凝血活酶时间(activated partial thromboplastin time,APTT)和凝血因子抑制物,制定围术期凝血因子替代治疗方案。术后动态监测凝血因子水平,予凝血因子替代治疗,根据病情动态观察,酌情处理。做好伤口管理、感染预防、痛疼管理、深静脉血栓预防、功能锻炼、心理疏导等。结果:本研究共31例血友病患者,行腓肠肌肌腱延长术7例次,跟腱延长术24例次,踝关节融合3例次,术后效果满意,患者生活质量显著提高。结论:血友病性踝关节病作为一种出血性疾病,手术风险大,围术期管理难度高,控制出血是关键。围术期给予周密护理,做好凝血因子替代治疗,密切观察病情,做好疼痛管理、功能锻炼、心理疏导等可以有效地帮助患者度过围术期。

Background： Hemophilia is a kind of hereditary disease of clotting disorder, which can cause muscle spasm, concurrence of Achilles tendon contracture and malformation of horseshoe. It affects the function of joint and quality of life seriously. Objective： To explore perioperative management methods of hemophilic ankle disorders. Methods： A total of 31 patients with hemophilic ankle disorders treated surgically between January 1996 and March 2018 were retrospectively analyzed in this study. Before surgery, preliminary experiment was performed by hematologists, including the measurement of coagulation factor activity, activated partial thromboplastin time（APTT）, coagulation factor inhibitors. Then the individual replacement therapy of coagulation factors was made. The level of coagulation factor was dynamically monitored and replacement therapy was applied after surgery. According to the condition, we should deal with it appropriately, such as wound management, infection prevention, pain management, deep vein thrombosis（DVT） prevention, functional exercise and mental care.Results： Those patients underwent 7 gastrocnemius tendon lengthening, 24 achilles tendon lengthening and 3 arthrodesis. Clinical outcomes were satisfactory and the quality of life was significantly improved. Conclusions： Hemophilic ankle arthropathy is a hemorrhagic disease with high risk for operation and high requirement for perioperative nursing. Controlling bleeding is the key point during perioperative period. Careful nursing（pain management, functional exercise, mental care） and appropriate replacement therapy of coagulation factor can effectively help patients smoothly go through perioperative period.