Pediatric Radiology > Musculoskeletal > Metabolic Diseases > Hemophilia


Hemophilia

Hemophilia is a bleeding disorder which can have profound musculoskeletal sequelae. Roughly 90% of patients with hemophilia suffer from spontaneous hemarthroses, many of which are recurrent. Typically, these hemarthroses are monoarticular and occur most frequently in the knee > elbow > ankle. Hemosiderin is deposited within the synovium of the affected joint leading to synovial hypertrophy, which in turn can lead to destruction of the underlying articular cartilage.

Radiographic appearance:

Acute findings

  • joint effusion
  • periarticular osteopenia

Chronic findings

  • epiphyseal overgowth (e.g. widening of intracondylar notch in the knee) - may mimic findings of JRA
  • pseudotumor formation (hematoma with thick fibrous capsule) - usually in the soft tissues but may compress adjacent bone
  • osteoarthritic changes

 

Hemophilic arthropathy in a 7-year old male. AP and lateral radiographs of the right knee demonstrate joint irregularity and an effusion. There is widening of the intracondylar notch. Also note the squaring and epiphyseal overgrowth involving the tibia and femur.




© Copyright Rector and Visitors of the University of Virginia 2013