Rodriguez-Merchan E Carlos
Department of Orthopaedic Surgery, La Paz University Hospital, Madrid. Spain.
Cardiovasc Hematol Disord Drug Targets. 2017;17(2):111-118. doi: 10.2174/1871529X17666170908110758.
Hemophilic arthropathy (HA) of the ankle is prevalent in people with hemophilia (PWH). It is frequently severe and incapacitating, due to recurrent bleeding into the ankle articulation during infancy. Around 50% of hemophilic patients suffer from ankle pain and radiological signs of HA.
To review current treatment of HA of the ankle in PWH.
A literature review of hemophilic ankle arthropathy in PWH was performed utilizing MEDLINE (PubMed) and the Cochrane Library.
Primary hematologic prophylaxis could keep away from the development of ankle HA if the level of the patient's deficient factor is prevented from dropping below 1% of normal. Recurrent articular bleeding can be prevented by the intravenous infusion of clotting factor concentrates (prophylaxis). Major articular bleeds and chronic hemophilic synovitis should be managed fiercely to prevent ankle HA. In the circumstance of advancing articular involvement, some noninvasive and invasive procedures can procure symptomatic mitigation and ameliorate the patient's function and quality of life.
The ideal treatment for the hemophilic ankle when hematologic prophylaxis fails includes physical medicine and rehabilitation, orthoses, radiosynovectomy, arthroscopic ankle debridement (in the initial stages of cartilage degeneration), and ankle distraction, ankle fusion or total ankle replacement (in advanced stages of cartilage degeneration).
踝关节血友病性关节病（HA）在血友病患者（PWH）中很常见。由于婴儿期踝关节反复出血，该病通常较为严重且会导致功能丧失。约50%的血友病患者患有踝关节疼痛及HA的影像学表现。
综述PWH中踝关节HA的当前治疗方法。
利用MEDLINE（PubMed）和Cochrane图书馆对PWH中的血友病性踝关节病进行文献综述。
如果能防止患者缺乏的凝血因子水平降至正常水平的1%以下，原发性血液学预防可避免踝关节HA的发生。通过静脉输注凝血因子浓缩物（预防）可预防反复的关节出血。应积极处理主要关节出血和慢性血友病性滑膜炎以预防踝关节HA。在关节受累进展的情况下，一些非侵入性和侵入性手术可缓解症状并改善患者的功能和生活质量。
当血液学预防失败时，血友病性踝关节的理想治疗方法包括物理医学与康复、矫形器、放射性滑膜切除术、关节镜下踝关节清创术（在软骨退变的初始阶段）以及踝关节撑开、踝关节融合或全踝关节置换（在软骨退变的晚期阶段）。
