Musculo-Skeletal Complications of Hemophilia

Abstract

Prophylactic treatment from ages 2 to 18 years could avoid the development of hemophilic arthropathy if the concentration of the patient's deficient factor is prevented from falling below 1% of normal. A review of the literature on the musculo-skeletal complications of hemophilia has been performed. Early treatment is of paramount importance because the immature skeleton is very sensitive to the complications of hemophilia; severe structural deficiencies may develop quickly. Major hemarthrosis (including joint aspiration) and chronic hemophilic synovitis should be treated aggressively to prevent hemophilic arthropathy. For the treatment of chronic haemophilic synovitis, radiosynovectomy should always be indicated as the first procedure. If, after three procedures with 6-month interval, radiosynovectomy fails, an arthroscopic synovectomy must be indicated. When advanced hemophilic arthropathy is present with severe disability, the aim should be to restore function. Arthroscopic joint debridement is an effective method to achieve this goal and can be considered to be an alternative to total joint arthroplasty in the younger age groups. Finally total joint arthroplasty can usually be relied upon to restore both mobility and function in a diseased joint. The potential benefits of joint arthroplasty must though always be weighed up against the long term sequelae, especially loosening and consequent revision surgery. A correct surgical haemostasis must be achieved by the infusion of factor concentrate at the right dose. Prophylactic treatment from ages 2 to 18 years could avoid the development of hemophilic arthropathy if the concentration of the patient's deficient factor is prevented from falling below 1% of normal. Major hemarthrosis, chronic hemophilic synovitis and the initial stages of hemophilic arthropathy should be treated aggressively to prevent hemophilic arthropathy. Finally total joint arthroplasty can usually be relied upon to restore both mobility and function in a diseased joint. A correct surgical haemostasis must be achieved by the infusion of factor concentrate at the right dose.


doi:10.4021/jh18e