Cartilage damage in the haemophilic joints: pathophysiology, diagnosis and management

Intra-articular bleeding affects the metabolism and repair of articular cartilage. Biomechanical data have shown that blood causes harmful effects on overall cartilage function under loading conditions. Therefore, haemophilic patients suffering a haemarthrosis should be subjected to blood aspiration (arthrocentesis) to prevent cartilage damage. MRI and ultrasonography have shown themselves to be excellent noninvasive tools for the evaluation of early cartilage damage that remains undetectable by conventional radiography in haemophilic patients. Prophylaxis with the deficient factor can prevent cartilage deterioration and reduce the incidence of joint haemorrhage in children with haemophilia. Radiosynovectomy has proved to be a highly effective procedure to decrease both the frequency and the severity of recurrent intra-articular bleeding episodes. Nowadays, the most usual surgical procedures for treating articular cartilage defects (cartilage repair) include abrasion chondroplasty, microfracture, mosaicplasty, autologous chondrocyte implantation (ACI), and matrix-induced ACI. In small defects (<2-4 cm(2)), ostechondral autograft or microfracture are the recommended options. In large defects (>2-4 cm(2)), ACI or osteochondral allograft are indicated. However, these techniques have not been applied in haemophilic patients because inflammatory conditions and advanced degenerative change (>50% joint space narrowing) are contraindications for cartilage repair. Thus, prevention of cartilage damage is paramount in haemophilia. The definitive remedy for advanced cartilage damage is either (knee or hip) replacement or (ankle) arthrodesis. Primary prophylaxis and radiosynovectomy are the best alternatives at our disposal to protect our patients against cartilage damage and arthropathy in haemophilic joints.