PB1225 - Orthopedic assessment in patients with hemophilia and Von Willebrand disease in a mexican population

Background: The orthopedic assessment aims to identify and address musculoskeletal bleeds, manage pain and prevent complications in order to preserve the autonomy and quality of life in hemophilia patients.

Aims: Identify hemophilic arthropathy and quantify its severity according to the Hemophilia Joint Health Score (HJHS), radiological Pettersson score and Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) scales.

Methods: Observational, cross-sectional and descriptive study. Data were obtained from clinical records at Hospital General Regional No. 1 IMSS from January 2021 to October 2023.

Results: 86 patients were analyzed, 84% had hemophilia A, 15% hemophilia B and 1% von Willebrand disease type 3; of which only 7% had inhibitor. In terms of severity, 13% had a mild form, 15% moderate and 72% severe. The age range was 1 to 82 years, with a median of 32 years. Eighty-five percent of patients received prophylaxis. The main target joints reported were ankle (30.1%), elbow (27.9%) and knee (26.3%). The evaluation of HJHS showed a total score in range from 0 to 75, with a score of 0 in 36.8%. Petterson scale median scores were observed in elbows 7, right knee 3, left knee 8, and ankles 7. 61 patients underwent joint assessment with USG with the HEAD-US scale: mild synovitis being more frequent in bilateral ankle (54%) and right elbow (47.5%), chondral damage with a score ≥3 in elbows (right 57.3%, left 54.1%) and subchondral damage with a score ≥1 ankles (right 63.9%, left 62.2%). Only 25 joint bleeding events were reported with the most affected joint being the left knee (36%).

Conclusion(s): The use of osteoarticular assessment tools in orthopedic consultations, allows for the evaluation of disease progression and treatment outcomes. Prophylactic management significantly reduces spontaneous joint bleeding events in patients with moderate to severe hemophilia, as well as the development of target joints and premature joint deterioration.