Orthopaedic problems about the ankle in hemophilia.
Hemophilia is an inherited recessive, sex-linked bleeding disorder. The lack of sufficient coagulation factor VIII produces hemophilia A, and the lack of factor IX causes hemophilia B. The prevention and treatment of the disease requires intravenous infusion of the deficient factor. Hemophilic patients present with multiarticular joint degeneration (hemophilic arthropathy), secondary to recurrent hemarthroses. With the availability of deficient factors, hemophilic patients requiring elective ankle surgery can undergo such surgery with a high expectation of success. A thorough analysis of each case by a multidisciplinary team will increase the likelihood of successful surgical intervention in the hemophilic patient. Radiosynovectomy decreases both the frequency and the intensity of recurrent ankle bleeding episodes related to ankle synovitis. The general recommendation is that when 3 early consecutive radiosynovectomies (repeated every 6 months) fail to halt synovitis, arthroscopic synovectomy should be considered. For advanced hemophilic arthropathy of the ankle, the first alternative for treatment, in our opinion, is arthroscopic ankle debridement. In the most severe cases, we recommend either ankle arthrodesis or total ankle replacement. In every other case, we feel that the best therapy is prophylaxis and radiosynovectomy in order to avoid hemophilic synovitis and ankle arthropathy.
La Paz University Hospital, Madrid, Spain. ecrmerchan@gmx.es
E Carlos
EC
Rodriguez-Merchan
D000843:Ankle Joint; D001174:Arthrodesis; D019643:Arthroplasty, Replacement; D001182:Arthroscopy; D003646:Debridement; D006395:Hemarthrosis / Q000209:etiology / Q000517:prevention & control / Q000532:radiotherapy / Q000601:surgery*; D006467:Hemophilia A / Q000150:complications*; D006801:Humans; D019637:Orthopedic Procedures / Q000379:methods*; D010396:Penicillamine / Q000008:administration & dosage / Q000627:therapeutic use; D000074402:Synovectomy; D013585:Synovitis / Q000209:etiology / Q000517:prevention & control
D010396:Penicillamine