Standardized Ultrasonography (HEAD-US) of Joints and First Correlation with Function in Haemophilic Arthropathy: Results of a Clinical Trial and Potential Importance of Joint-Ultrasound for an Individualized Prophylaxis in Haemophiliacs

Background: Sonography is applied in patients with bleeding disorders. By ultrasonography the extent of synovitis is detected as a sign of the activity of an existing hemophilic arthropathy, as a sign of progression are defects of articular cartilage and bony structures quantifiable. In 2013 an easy-to-use standardized ultrasound protocol (HEAD-US) for examination of early joint changes in Hemophilic Arthropathy was published. Correlation between functional and structural changes in Haemophilic Arthropathy was not published before.

Aims: The sonographic quantification of the arthropathy by haemophilia-treaters could be the basis for better individualized haemophilia therapy in young patients. 

Methods: For this purpose, highly reproducible sonographic standard section planes and an examiner-independent ultrasound score (HEAD -US) were published by C. Martinoli in 2013. After a preliminary study of 27 patients now we have included 177 young german patients (children and young adults) with haemophilia A, haemophilia B or von-Willebrand- disease from different German hemophilia treatment centers in the HaemarthroSonoPilot study (DRKS00004483).
After informed consent sonography of the elbow, knee and ankle joints with a Zonare " z.one ultra " ultrasound device (Linear probe L14 -5w ) was performed. Simultaneously an orthopedic clinical examination with clinical scoring and 3D motion analysis of the lower limbs for detecting early function defects were performed with an ultrasonic topometer (developed by the University of Bonn, Germany). 

Results: Already in the previous investigation of the pilot study on a small cohort yielded promising results depending on age. The investigations in the presented pilot study showed correlation of the sonographic diagnostics with the measurement of a clinical orthopedic examination in haemophilic arthropathy. Through the joint sonography changes were even partially already seen before that stood out in the clinical investigation. 

Conclusion: It may be useful if haemophilia treaters in future apply this method to individualize the therapy under close control and evaluation of joint changes.