Role of Musculoskeletal Ultrasound in Detection of Hemophilic Arthropathy

Abstract

Abstract Background Hemophilia is an X-linked congenital bleeding disorder, with hemophilia A comprising the majority of cases and hemophilia B. Von Willebrand disease (VWD) is a bleeding disorder of variable severity, caused by deficiency of von Willebrand factor (VWF) especially type III. The severe form of the bleeding disorders is characterized by spontaneous hemoarthrosis which occurs mainly in large synovial joints and results in hemophilic arthropathy (HA) that is characterized by soft tissue changes such as synovial hypertrophy and effusion as well as cartilage and bony damage., MRI is the gold standard, however ultrasound (US) was proven to be a sensitive tool in detecting synovial hypertrophy with results comparable to MRI, as well as differentiate between hemoarthrosis and arthritis-metiated joint pain, together with having more advantage to the MRI, making it an excellent tool in assessing disease activity. Objective To describe the value of MSK ultrasound in assessment of intra-articular findings in hemophilic arthropathy (HA) in the pediatric age group. Methods A descriptive, cross-sectional study that included 120 joints in 20 patients with bleeding disorders, (moderate or severe hemophilia A/B or vWD). Evaluated joints included elbows, knees and ankle joints on both sides for every patient. One additional patient was evaluated for a hip joint affection. The abnormal US findings among the involved joints were compared to the sonographically-normal contralateral side. We excluded patients with recent joint surgeries, those who have any other rheumatological disease. Results Out of the 120 joints examined, 28 joints showed US abnormal findings. The knee was the most common involved joint (75%), followed by the ankle (30%) then the elbow (15%). The most frequent US lesion was synovial hypertrophy (82%), followed by joint effusion (78.6%), synovial hyperemia (53%) and lastly comes the ostochondral lesions (7.1%). Other juxta-articular findings were reported as myositis, tenosynovitis and intra-/intermuscular hematoma. Hemoarthrosis was found in 6 out of 16 knee joints, 2 out of 7 ankles, yet no elbow nor hip joint bleedings were reported. There was a significant correlation between the incidence of synovial thickening, bone and cartlage damage and bleeding incidence and increasing age. A high statistical significant difference was found between the normal and abnormal joints (by US) and especially the knee and ankle joints. Conclusion MSK US has now emerged as a promising imaging modality for the early detection and management of HA, and for the evaluation of hemarthrosis and painful MSK episodes in hemophilic patients. It has a high efficacy in assessment of the soft tissue abnormalities such as synovial hypertrophy, vascularity and joint effusion, as well as late-onset degenerative changes. Global standardization of the different US scoring systems and clear definitions of the US pathologies in HA are needed with high-level MSK US training practice as well, for accurate assessment of HA using US.