Abstract Number: PB0593
Meeting: ISTH 2021 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical
Background: Hemophilic ankle arthropathy (HAA) causes morbidity in (young) hemophilia patients. If conservative treatment fails, major surgical interventions like arthrodesis are indicated. An alternative treatment to postpone these interventions and maintain joint mobility is desired. In osteoarthritis, joint distraction was a good joint-preserving alternative. Retrospective evaluation of three patients with HAA treated with ankle joint distraction (AJD) showed evident clinical and radiological improvements.
Aims: To gather prospective data on clinical efficacy and tissue structure changes of AJD in HAA.
Methods: This ongoing first prospective study included patients ≥18 and ≤55 years with severe complaints of HAA, insufficiently responding to conservative treatment. AJD was performed over ten weeks using an external frame, applying 5mm distraction. Data on clinical efficacy (questionnaires and performance tests) and tissue structure changes (X-ray and MRI) were evaluated.
Results: The AOS score is a valid instrument to measure pain related to ankle arthritis. Zero means ‘no pain’, hundred means ‘worst pain imaginable’. The HAL score is a valid instrument to measure the impact of hemophilia on self-perceived functional abilities. Zero represents ‘poor functional status’ and hundred represents ‘the best functional status’. Data over the course of three years is represented here as median scores. m=months after distraction.
Three-year follow-up data is available for eight patients. Over three years, ankle pain, as evaluated by the Ankle Osteoarthritis Scale (AOS) reduced significantly and a similar downward trend was seen in pain as evaluated by the Visual Analogue Scale (VAS). Functional status, assessed by the Hemophilia Activities List (HAL) and performance tests, significantly improved over time (figure 1). There were no significant changes in range of motion (ROM), indicating that joint mobility was retained among patients. MRI showed structural improvements in the ankle joints of these patients (figure 2). The main complication during treatment was pin tract infection (n=6), commonly seen with external frames and effectively treated with a short course of antibiotics. There were no adverse bleeding events.
Conclusions: This explorative study shows that AJD in HAA results in decreased pain, improved function and tissue repair for a prolonged time. This joint-preserving treatment may have a great impact, especially in the relatively young hemophilia population. Prolonged follow-up is needed to determine the duration of efficacy in HAA. Structural changes (decrease in subchondral cysts and edema) on MRI. Sagittal images at baseline and one and three year after AJD.
To cite this abstract in AMA style:
van Bergen E, van Vulpen L, Vogely H, de Kleijn P, Foppen W, van Roermund P, Schutgens R, Mastbergen S, Lafeber F. Promising Prospective Data on the Effect of Ankle Joint Distraction in Hemophilic Arthropathy: Three Year Follow-up [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/promising-prospective-data-on-the-effect-of-ankle-joint-distraction-in-hemophilic-arthropathy-three-year-follow-up/. Accessed October 1, 2023.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/promising-prospective-data-on-the-effect-of-ankle-joint-distraction-in-hemophilic-arthropathy-three-year-follow-up/