Musculoskeletal complications of hemophilia

Abstract

The most important clinical strategy for management of patients with hemophilia is the avoidance of recurrent hemarthroses by means of continuous, intravenous hematological prophylaxis. When only intravenous on-demand hematological treatment is available, frequent evaluations are necessary for the early diagnosis and treatment of episodes of intra-articular bleeding. The natural history of the disease in patients with poorly controlled intra-articular bleeding is the development of chronic synovitis and, later, multi-articular hemophilic arthropathy. Once arthropathy develops, the functional prognosis is poor. Treatment of these patients should be conducted through a comprehensive program by a multidisciplinary hemophilia unit. Although continuous prophylaxis can avoid the development of the orthopedic complications of hemophilia still seen in the twenty-first century, such a goal has not, so far, been achieved even in developed countries. Therefore, many different surgical procedures such as arthrocentesis, radiosynoviorthesis (radiosynovectomy) (yttrium-90, rhenium-186), tendon lengthenings, alignment osteotomies, joint arthroplasties, removal of pseudotumours, and fixation of fractures are still frequently needed in the care of these patients.

Fig. 1

A photograph demonstrating chronic hemophilic synovitis of the knee

Fig. 2

Radiographs of a 36-year-old patient with bilateral hemophilic arthropathy of the knee

Fig. 3

a A radiograph illustrating a posterior thigh pseudotumor which developed six years following a spontaneous thigh hematoma. b This photograph demonstrates the surgical specimen which resulted from the removal of the pseudotumor