Orthopedic disorders of the knee in hemophilia: A current concept review

Abstract

The knee is frequently affected by severe orthopedic changes known as hemophilic arthropathy (HA) in patients with deficiency of coagulation factor VIII or IX and thus this manuscript seeks to present a current perspective of the role of the orthopedic surgeon in the management of these problems. Lifelong factor replacement therapy (FRT) is optimal to prevent HA, however adherence to this regerous treatment is challenging leading to breakthrough bleeding. In patients with chronic hemophilic synovitis, the prelude to HA, radiosynovectomy (RS) is the optimal to ameliorate bleeding. Surgery in people with hemophilia (PWH) is associated with a high risk of bleeding and infection, and must be performed with FRT. A coordinated effort including orthopedic surgeons, hematologists, physical medicine and rehabilitation physicians, physiotherapists and other team members is key to optimal outcomes. Ideally, orthopedic procedures should be performed in specialized hospitals with experienced teams. Until we are able to prevent orthopedic problems of the knee in PWH will have to continue performing orthopedic procedures (arthrocentesis, RS, arthroscopic synovectomy, hamstring release, arthroscopic debridement, alignment osteotomy, and total knee arthroplasty). By using the aforementioned procedures, the quality of life of PWH will be improved.

Keywords: Hemophilia; Knee; Orthopedic problems; Prevention; Surgical treatment

Core tip: Hemophilia is an inherited bleeding disorder due to deficiency of factor VIII (hemophilia A) or factor IX (hemophilia B) resulting in insufficient thrombin generation leading to recurrent intra-articular hemorrhages (hemarthroses). Prevention of hemarthroses with intravenous infusions of the deficient protein from infancy to adulthood (primary prophylaxis) should be considered to achieve optimal outcomes. If factor replacement therapy (FRT) is insufficient, or if patients are not adherent to the prescribed regimen, recurrent hemarthroses results in chondrocyte apoptosis (cartilage degeneration) and hypertrophy of the synovium (synovitis). Many surgical interventions are available for the knee joint. For example, to treat synovitis recalcitrant to FRT, there are two primary orthopedic modalities: Radiosynovectomy and arthroscopic synovectomy. This article reviews the pathogenesis, diagnosis and treatment of hemophilic arthropathy of the knee.