Musculoskeletal ultrasound in hemophilia: Results and recommendations from a global survey and consensus meeting

Abstract

Introduction For persons with hemophilia, optimization of joint outcomes is an important unmet need. The aim of this initiative was to determine use of ultrasound in evaluating arthropathy in persons with hemophilia, and to move toward consensus among hemophilia care providers regarding the preferred ultrasound protocols for global adaptation. Methods A global survey of hemophilia treatment centers was conducted that focused on understanding how and why ultrasound was being used and endeavored to move toward consensus definitions of both point‐of‐care musculoskeletal ultrasound (POC‐MSKUS) and full diagnostic ultrasound, terminology to describe structures being assessed by ultrasound, and how these assessments should be interpreted. Next, an in‐person meeting of an international group of hemophilia health care professionals and patient representatives was held, with the objective of achieving consensus regarding the acquisition and interpretation of POC‐MSKUS and full diagnostic ultrasound for use in the assessment of musculoskeletal (MSK) pathologies in persons with hemophilia. Results The recommendations were that clear definitions of the types of ultrasound examinations should be adopted and that a standardized ultrasound scoring/measurement system should be developed, tested, and implemented. The scoring/measurement system should be tiered to allow for a range of complexity yet maintain the ability for comparison across levels. Conclusion Ultrasound is an evolving technology increasingly used for the assessment of MSK outcomes in persons with hemophilia. As adoption increases globally for clinical care and research, it will become increasingly important to establish clear guidelines for image acquisition, interpretation, and reporting to ensure accuracy, consistency, and comparability across groups.

Diagnosis and treatment of chronic synovitis in patients with haemophilia: consensus statements from the Italian Association of Haemophilia Centres

“…This strategy of detection and grading for synovial hypertrophy has been used in some studies, 35,51,52 consistently suggesting that US is highly sensitive (>92%) for detecting synovial abnormalities with results comparable to those obtained with MRI 35,51,52 . Although some people may believe that joint screening with ultrasound would be highly demanding and time‐consuming in the routine clinical practice, the advent of simplified point‐of‐care protocols is allowing clinicians to perform the examination of the six joints of interest in a very short time 53 . The use of ultrasound couldv not be considered a time‐wasting activity if it is fully integrated with the physical exam to improve the sensitivity in the detection of early‐phase joint alterations.…”

The value of ultrasonography in detecting early arthropathic changes and contribution to the clinical approach in patients of hemophilia the value of ultrasonography in patients of hemophilia

“…Ultrasound has been shown to detect and quantify disease activity (joint effusion and synovial hypertrophy) 9 and degenerative damage (osteochondral changes). Ultrasound is widespread throughout the territory, is inexpensive, quick to perform and has the possibility of “point‐of‐care” use in patients suffering from hemophilia 10 …”

A roadmap for better management for synovitis in haemophilia

“…There must be standardization to allow Haemophilia Treatment Centers to bring this innovation to the bedside. 6 Lastly, regarding interventions for the management of synovitis, there was consensus recommendation to escalate or institute a prophylaxis regimen to prevent bleeding. Escalation of increasing frequency or using pharmacokinetic measures to refine dosing are recommended with clotting factor concentrate, but there is no recommendation on integrating or adjusting the novel therapeutics, such as subcutaneous non-factor therapy (Emicizumab).…”

“…Again there was no agreement reached for the role of CT or MRI imaging, but there was a consensus recognition of role of ultrasound imaging to investigate synovitis: Musculoskeletal Ultrasound (MSKUS), either through full diagnostic ultrasound, performed by a radiologist with visualization of anatomical structures, or point of care (POC)-MSKUS a limited ultrasound targeted for identification of effusion and synovial hypertrophy performed by a trained health care provider at the bedside or clinic. 6 While MRI is acknowledged as the gold standard, the limitations of time, expense accessibility and sedation requirements make it inaccessible for PwH, especially young children. 7 Joint ultrasound, which does not require ionizing radiation or sedation, is less expensive, and has good correlation with MRI imaging for detection of effusion and synovial hypertrophy, 8 has an increasingly important role in comprehensive care for PwH.…”

“…The lack of consensus in the paper represents the lack of current consensus in integrating joint ultrasound evaluations into the clinic. There must be standardization to allow Haemophilia Treatment Centers to bring this innovation to the bedside 6 …”