Center for Benign Haematology, Thrombosis and Haemostasis, University, Medical Center Utrecht, University Utrecht, Van Creveldkliniek, Utrecht, The Netherlands.
Department, of Rheumatology & Clinical Immunology, University Medical Center Utrecht, University Utrecht, Utrecht, The Netherlands.
Haemophilia. 2022 Nov;28(6):1044-1053. doi: 10.1111/hae.14633. Epub 2022 Aug 3.
Haemophilic ankle arthropathy (HAA) causes major morbidity. When conservative treatment fails, major surgical interventions are indicated. An alternative treatment to maintain joint mobility and postpone these interventions is desired.
To gather prospective data on clinical/structural changes after ankle joint distraction (AJD) in HAA.
This study includes patients with severe HAA insufficiently responding to conservative treatment. AJD was performed during 8-10 weeks by use of an external frame. Questionnaires, physical examination and radiology were used to evaluate pain, function and structural changes before and 6, 12, 24 and 36 months after distraction. Mixed effect models were used for analysis.
This study includes eight cases (21-53 years). The fixed effects estimates of the visual analogue score (0-10) improved from 7.5 at baseline to 3.4 (p = .023) 3 years after distraction. The Haemophilia Activities List (HAL, 0-100) for basic/complex lower extremities functions improved from respectively 29.6 and 31.5 to 54.3 (p = .015) and 50.7 (p = .031). Joint mobility was maintained. Magnetic resonance imaging (MRI) showed thickened cartilage and reduced bone marrow oedema and subchondral cysts. Pin tract infections (n = 6) were effectively treated and no adverse bleeding events occurred. At 3-year follow-up, in none of the patients the originally indicated arthrodesis was performed.
This first prospective study showed that AJD in HAA results in decreased pain, improved function and decreased arthropathy-related MRI findings in the majority of patients for prolonged time. Although the study population is small and follow-up is relatively short, AJD may be promising to postpone invalidating interventions and might be a breakthrough treatment.
血友病性踝关节病（HAA）会导致严重的发病。当保守治疗失败时，需要进行主要的手术干预。需要一种替代治疗方法来维持关节活动度并推迟这些干预措施。
收集踝关节牵张术（AJD）治疗血友病性踝关节病后临床/结构变化的前瞻性数据。
本研究纳入对保守治疗反应不佳的严重 HAA 患者。AJD 通过使用外固定架在 8-10 周内进行。在牵张前、6、12、24 和 36 个月时使用问卷、体格检查和影像学评估疼痛、功能和结构变化。使用混合效应模型进行分析。
本研究纳入 8 例患者（21-53 岁）。视觉模拟评分（0-10）的固定效应估计值从基线时的 7.5 改善到牵张后 3 年时的 3.4（p=0.023）。基本/复杂下肢功能的血友病活动清单（HAL，0-100）分别从 29.6 和 31.5 改善至 54.3（p=0.015）和 50.7（p=0.031）。关节活动度得到维持。磁共振成像（MRI）显示软骨增厚，骨髓水肿和软骨下囊肿减少。（n=6）的针道感染得到有效治疗，且无不良出血事件发生。在 3 年随访时，没有患者进行了最初指示的关节融合术。
这项前瞻性研究首次表明，在大多数患者中，AJD 治疗 HAA 可减轻疼痛、改善功能并减少与关节炎相关的 MRI 发现，且持续时间较长。尽管研究人群较小，随访时间相对较短，但 AJD 可能有望推迟无效的干预措施，并且可能是一种突破性的治疗方法。
