Background Recurrent clinical and subclinical joint bleeding almost inevitably causes arthropathy with affected function up to severe loss of motion of elbows, knees and ankles. Diagnosis of hemophilia is essentially clinical and laboratory based, however, imaging is an important tool for evaluation of complications and therapeutic follow-up. Musculoskeletal Ultrasound (MSUS) is a useful method for frequent follow-up in pediatric patients being non-invasive, easy repeatable, painless, without ionizing radiation, relatively inexpensive, with no need for sedation.
Objectives To investigate the role of MSUS as a tool for evaluation of joint affection in hemophilic arthropathy compared to clinical and functional assessment as well as conventional x-ray.
Methods This study included 20 male children (a total of 120 joints) with severe hemophilia type A (6–15ys). Twenty age and sex matched non-hemophilic children served as controls. Patients underwent clinical examination, assessment of the Hemophilic Joint Health Score, functional assessment according to Functional Independence Score for Hemophilia, radiological evaluation using conventional X-ray according to Pettersson classification as well as qualitative ultrasongraphic scoring according to Klukowska et al., 2001, and Muca-perja et al., 2012. For more accurate quantitative values, we designed an MSUS index based on measurements of the cartilage thickness and joint space dimensions. All above was done at the knees, ankles and elbow joints.
Results The knee joint was the principal joint affected among hemophilic patients. Cartilage thinning was the predominant pathological disorder with subsequent joint space narrowing especially at the medial condylar side of the knee. Using the designed quantitative score the cartilage thickness among patients, as well as the joint space, showed a highly significant negative correlation with age, clinical scores and the radiological score while no statistically significant relation was detected with the functional scoring. The ultrasonography qualitative scores failed to show statistically significant relation on correlating with clinical, functional and radiological scores. The prevalence of cartilage damage among patients according to Klukowska et al., score was 75% of the total 40 knee joints, 47.5% with synovial hypertrophy, 30% with synovial effusion and 20% with increased synovial vascularity. The prevalence of cartilage damage among patients according to Muca-perja et al., 2012 was 90% of the total 40 knee joints, 47.5% with synovial hypertrophy, 27.5% with bone erosions and 20% with synovial hyperemia.
Conclusions MSUS successfully detected changes in the hemophilic joints even before functional affection started. MSUS provides accurate assessment, allowing early detection of hemophilic arthropathy as well as precise follow up of progression of damage by standardizing and simplifying the planes and landmarks for acquisition of the sonographic images using quantitative measurements in haemophilic joints
Klukowska A, Czyrny Z, Laguna P and Brzewski M: Correlation between clinical, radiological and ultrasonographical image of knee joints in children with hemophilia, Haemophilia; 2001: 7(3):286–92.
Muca-Perja, M, Riva, S, Grochowska, B, et al. Ultrasonography of haemophilic arthropathy. Haemophilia. 2012;18(3):364–368
Disclosure of Interest None declared
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