Haemophilia imaging: a review

Abstract

Haemophilia disorders are characterised by a blood coagulation anomaly leading to prolonged and excessive bleeding. Imaging provides an essential role in the investigation of both the musculoskeletal and the non-musculoskeletal complications of haemophilia. Our institution is home to a large tertiary referral centre for haemophilia treatment. Using our broad experience, we present a multi-modality pictorial review of the musculoskeletal manifestations of haemophilia, including haemophilic arthropathy, intra-muscular haemorrhage and haemophilic pseudotumour. The main imaging features of haemophilic arthropathy are described, including synovial hypertrophy, haemosiderin deposition, sub-chondral cyst formation and loss of joint space.

Tumours and tumour mimics in the olecranon

“…43 Radiographically, they are welldefined, lobulated, expansile, lytic lesions, which may demonstrate endosteal scalloping, cortical thinning, and pathological fractures (Fig 10). 44 Trabeculae frequently extend across the osteolytic lesions (Fig 10b). Radiographic findings vary with extent, location, and different stages of haemorrhages, and hence cause confusion with other tumourous or infectious conditions.…”

“…40 Haemosiderin deposits can be identified with gradient-echo sequences, showing markedly low-signal. 44 Geodes Geodes (Fig 11) are subarticular cystic lesions, often found during the course of rheumatoid arthritis. The sites most commonly affected are the tibial plateau and femoral neck.…”

Arthritis

“…However, up to 30 % of patients produce antibodies to these clotting factors, leading to increased risk of uncontrolled bleeding [ 27 ]. Affected joints may be treated with radionuclide or open synovectomy [ 25 ].…”

“…MRI allows more precise evaluation of joint involvement in early as well as advanced disease. Scoring systems (such as the semiquantitative Denver scale) facilitate assessment of treatment response [ 27 ]. Early MRI fi nding in joints affected by hemophilia include effusions with fl uid-fl uid levels, synovial hypertrophy, and focal areas of cartilage erosion.…”

Paediatric musculoskeletal US beyond the hip joint

“…These structures can be distinguished based on subtle echotextural differences. In the bottom part of the image, drawings illustrate the extension of cartilage (white), synovial pannus (transparent) and joint effusion (light grey) in each of the areas (1-4) marked in the ultrasonogram Corresponding colour Doppler image reveals marked hyperaemia around the joint space and the abscess deposition, which is possible with susceptibility-sensitive MRI [44]. US seems particularly helpful in detection of subclinical joint involvement and early signs of joint damage, thus guiding patient management more appropriately.…”