How to assess, detect, and manage joint involvement in the era of transformational therapies: Role of point‐of‐care ultrasound

Minno, Matteo Nicola Dario Di; Martinoli, Carlo; Pasta, Gianluigi; Corte‐Rodríguez, Hortensia De la; Samy, Islam; Stephensen, David; Timmer, Merel A.; Winburn, Ian

doi:10.1111/hae.14657

Cited by 8 publications

(9 citation statements)

References 99 publications

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“…According to Ray et al [8] radiological evaluation is crucial in the assessment and follow-up of musculoskeletal complications of hemophilia (hemophilic arthropathy, intramuscular hematomas, hemophilic pseudotumors) and magnetic resonance imaging (MRI) is the gold standard. It has been recently reported that POCUS (point-of-care ultrasound) can ease the early discovery of articular damage and can monitor illness advancement while rendering insights into the effectiveness of treatment regimens, and should be deemed as a vital evaluation tool for managing the care of PWH [9]. In adults with hemophilia, physical examination and POCUS study render complementary information on their joint illness.…”

Section: Diagnostic Methodsmentioning

confidence: 99%

Orthopedic surgical procedures in people with hemophilia

Encinas-Ullán¹,

Corte‐Rodríguez²,

Gómez-Cardero³

et al. 2023

Blood Coagulation &Amp; Fibrinolysis

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People with hemophilia tend to develop joint lesions secondary to the recurrent hemarthroses typical of their condition. These usually include chronic synovitis and arthropathy chiefly affecting their ankles, knees, and elbows. In addition, muscular hematomas, albeit less frequently, may also result in complications such as acute compartment syndrome, pseudotumors, bone cysts and peripheral nerve compression. Joint lesions may require some of the following surgical interventions: arthroscopic synovectomy (in cases of synovitis), arthroscopic joint debridement, radial head resection, opening-wedge tibial osteotomy, arthrodesis, arthrodiastasis (of the ankle), tendon lengthening (hamstrings, Achilles tendon), progressive extension of the knee by placing an external fixator in cases of flexion contracture of the knee, supracondylar femoral extension osteotomy in cases of knee flexion contracture and, eventually, a total joint arthroplasty when the affected joint has been destroyed and the patient experiences severe joint pain. Total knee arthroplasty in hemophilic patients is associated with a high infection risk (7% on average). As regards the complications following muscle hematomas, acute compartment syndrome requires urgent performance of a fasciotomy when hematological treatment is incapable of resolving the problem. Surgical resection of hemophilic pseudotumors is the best solution, with those affecting the pelvis (secondary to iliopsoas hematomas) being particularly difficult to resolve. Peripheral nerve lesions can often be effectively addressed with hematological treatment, although a surgical neurolysis of the ulnar nerve is indicated if nonoperative treatment fails.

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Section: Diagnostic Methodsmentioning

confidence: 99%

Orthopedic surgical procedures in people with hemophilia

Encinas-Ullán¹,

Corte‐Rodríguez²,

Gómez-Cardero³

et al. 2023

Blood Coagulation &Amp; Fibrinolysis

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“…It is particularly helpful in the evaluation of synovial proliferation, a surrogate of disease activity, and in more accurate evaluation of painful musculoskeletal episodes. 18,19 Compared to MRI, US is more sensitive in distinguishing bloody from non-bloody effusions 20 and has high to excellent correlation with MRI for detecting pathological soft tissue. 21 US can also provide a general idea of the osteochondral surface, revealing marginal bone erosions, superficial subchondral cysts, and peripheral articular cartilage defect.…”

Section: Joint Assessmentmentioning

confidence: 99%

“…X-rays and clinical exam are less sensitive to early changes. POC-MSKUS is a safe, cost-effective, readily available, and sensitive tool that allows for accurate diagnosis of acute haemarthrosis 19,26,33,34 and serial evalua-tion of synovial proliferation (active vs. inactive) 18,28,29 and the overall condition of osteochondral surfaces of index joints in PwH. 22,26 It is crucial to point out that while early diagnosis and management of haemophilic arthropathy is becoming more relevant for clinical management and individualised patient regimens 29 evidence to conclude that US-detectable findings are sensitive to or could inform changes or intensification of prophylactic therapy in PwH remains insufficient.…”

Section: Magnetic Resonance Imaging (Mri)mentioning

confidence: 99%

Haemophilic arthropathy: Diagnosis, management, and aging patient considerations

Bakeer,

Saied,

Gavrilovski

et al. 2024

Haemophilia

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Gene therapy and universal use of safer, more effective, and personalised prophylactic regimens (factor, and nonfactor) are expected to prevent joint bleeding and promote joint health in persons with haemophilia (PwH). Growing evidence suggests that subclinical bleeding, with active and inactive synovial proliferation, continues and haemophilic arthropathy remains a major morbidity in PwH despite early institution of joint prophylaxis. Joint health assessment is evolving with physical examination scores complementing imaging scores. Point‐of‐care ultrasound is emerging as a safe, cost‐effective, and readily available tool for acute determination of musculoskeletal abnormalities, serial evaluation of joints for sonographic markers of haemophilic arthropathy, and in providing objective insight into the efficacy of new therapies. In acute haemarthrosis, arthrocentesis expedites recovery and prevent the vicious cycle of bleed‐synovitis‐rebleed. When synovial proliferation develops, a multidisciplinary team approach is critical with haematology, orthopaedics, and physiotherapy involvement. Synovectomy is considered for patients with chronic synovitis that fail conservative management. Non‐surgical and minimally invasive procedures should always be offered and considered first. Careful patient selection, screening and early intervention increase the success of these interventions in reducing bleeding, pain, and improving joint function and quality of life. Chemical synovectomy is practical in developing countries, but radioactive synovectomy appears to be more effective. When surgical synovectomy is considered, arthroscopic/minimally invasive approach should be attempted first. In advanced haemophilic arthropathy, joint replacement and arthrodesis can be considered. While excited about the future of haemophilia management, navigating musculoskeletal challenges in the aging haemophilia population is equally important.

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“…the Denver Score and the International Prophylaxis Study Group) and ultrasound (e.g. Haemophilia Early Arthropathy Detection with Ultrasound [HEAD‐US], Joint Tissue Activity and Damage Exam [JADE] and the Universal Simplified Ultrasound [US‐US] protocol) 8 …”

Section: Joint Health Assessment Revisited In the Era Of New Treatmentsmentioning

confidence: 99%

Outcomes and outcome measures

Castaman,

Jimenez‐Yuste,

Gouw

et al. 2024

Haemophilia

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IntroductionAdvances in haemophilia treatment have resulted in a near‐normal life expectancy, lower burden of bleeding and treatment, and improved quality of life in high‐income countries. Bleeding rate is approaching zero and novel parameters should be evaluated to assess the efficacy of treatment not only from the clinical point of view by using new methodologies (e.g. joint health assessment), but also from the patient's perspective (e.g. pain, quality of life, treatment satisfaction).Methods and resultsThis approach should be aimed at combining objective clinical methodologies and patient‐reported outcomes (PROs). However, some instruments used for assessing PROs are still suboptimal and not properly validated. Recent evidence suggests that these tools can take advantage from a more personalized designed approach and could be effectively improved and serve to facilitate the patient's self‐evaluation. For other congenital bleeding disorders (BDs), a set of patient‐relevant outcomes has been also defined that overlap substantially those of haemophilia, including bleeding, side effects and complications, and PROs, such as pain, physical functioning, impact on daily life including school and work and mental health. There is a growing focus on addressing women‐specific outcomes in BDs, reflecting an increased awareness of the unique challenges faced by women in this context. However, the development of tailored tools is imperative to further advance the progress in managing women with BDs, ensuring more accurate monitoring and personalized care.ConclusionsHow incorporating these outcome measures in the process of approval of novel treatments for these disorders by regulatory authorities remains to be established.

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How to assess, detect, and manage joint involvement in the era of transformational therapies: Role of point‐of‐care ultrasound

Cited by 8 publications

References 99 publications

Orthopedic surgical procedures in people with hemophilia

Orthopedic surgical procedures in people with hemophilia

Haemophilic arthropathy: Diagnosis, management, and aging patient considerations

Outcomes and outcome measures

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