SPR Unknown case #44: FINAL
Publication Date: 2010-12-08
MRI left ankle: Focal subchondral cysts in the left tibial epiphysis are associated with focal areas of edema in the distal tibial epiphysis and metaphysis. Erosions of the articular surfaces of the distal tibia and the talar dome. Thinning of the articular cartilage of the tibio-talar joint. Small amount of tibio-talar joint fluid, as well as in some other tarsal joints. Thickening with decreased signal in the synovium of the tibio-talar joint, likely related to hemosiderin deposition and nodular synovial hypertrophy.
• Hemophilic Arthritis
• Pigmented Villonodular Synovitis (PVNS)
• Hemophilia is a defect in coagulation caused by a deficiency clotting factor or clotting that is nonfunctional.
• Deficiencies of factor VIII (antihemophilic factor) lead to classic hemophilia (hemophilia A), and deficiencies offactor IX (plasma prothromboplastin component) lead to Christmas disease (hemophilia B).
• These genetic disorders are X-Iinked recessive. They primarily affect males (1:5000), but are transmitted by females.
• The clinical and radiologic features of patients with classic hemophilia and Christmas disease are virtually identical.
• Hemorrhage most often occurs in the synovial joints. In descending order, the knee, ankle, elbow, shoulder, and hip are involved. The knee is the most commonly affected joint in Hemophilia and dense joint effusions are common. The knee damage may progress to fixed flexion deformity and subluxation.
• Reflect the presence of hemarthrosis (joint effusion), synovial inflammation and hyperemia (osteoporosis and epiphyseal overgrowth), chondral erosions and subchondral resorption (osseous erosions and cysts), cartilaginous denudation (joint space narrowing), intraosseous or subperiosteal hemorrhage (pseudotumors), and osseous proliferation (sclerosis and osteophytes).
• Some abnormalities of osseous shape, such as widening of the intercondylar notch, flattening of the condylar surface, or squaring of the patella, are very characteristic of chronic hemarthrosis of the knee.
XR Findings (underestimate disease)
MRI Findings (more sensitive)
- Hermann, G, Gilbert, MS, Abdelwahab, IF Hemophilia: evaluation of musculoskeletal involvement with CT, sonography, and MR imaging. Am. J. Roentgenol. 1992 158: 119-123.
- Jaume Llauger, Jaume Palmer, Núria Rosón, Sílvia Bagué, Àngels Camins, and Rosa Cremades. Nonseptic Monoarthritis: Imaging Features with Clinical and Histopathologic Correlation. RadioGraphics 2000 20: 263S-278S.
- Jelbert A, Vaidya S, Fotiadis N. Imaging and staging of haemophilic arthropathy. Clin Rad (2009) 65: 1119-1128.