title: Management of musculoskeletal complications of hemophilia.
abstract: Prophylactic treatment from ages 2 to 18 years could prevent the development of hemophilic arthropathy if the concentration of the patient's deficient factor is kept from falling below 1% of normal. Early treatment is of paramount importance because the immature skeleton is very sensitive to the complications of hemophilia. Major hemarthrosis and chronic hemophilic synovitis should be treated aggressively to prevent hemophilic arthropathy. When advanced hemophilic arthropathy is present with severe disability, the aim should be to restore function while minimizing the risk to the patient. Joint debridement is an effective method to achieve this goal, especially around the elbow or ankle, and can be considered an alternative to knee replacement in the younger age groups. Proximal tibial valgus osteotomy is a reliable treatment method for painful genu varum of the mobile hemophilic knee. Supramalleolar tibial varus osteotomy is an attractive alternative to the more commonly used surgical option of arthrodesis. Finally, joint replacement can usually be relied on to restore both mobility and function in a diseased joint. The potential benefits of joint replacement must always be weighed against the long-term sequelae, however, especially loosening and consequent revision surgery.
authors.affiliation: Service of Traumatology, La Paz University Hospital, Madrid, Spain. rmerchan@arrakis.es
authors.forename: E Carlos
authors.initials: EC
authors.lastname: Rodriguez-Merchan
mesh_terms: D000293:Adolescent; D000328:Adult; D001178:Arthroplasty; D002648:Child; D002675:Child, Preschool; D015942:Factor VIIa / Q000627:therapeutic use; D005260:Female; D015658:HIV Infections / Q000150:complications; D006467:Hemophilia A / Q000150:complications* / Q000628:therapy; D006801:Humans; D006820:Hyaluronic Acid / Q000627:therapeutic use; D007223:Infant; D007592:Joint Diseases / Q000517:prevention & control*; D008297:Male; D009140:Musculoskeletal Diseases / Q000150:complications / Q000628:therapy*; D011994:Recombinant Proteins / Q000627:therapeutic use
chemical_list: D011994:Recombinant Proteins; D006820:Hyaluronic Acid; D015942:Factor VIIa