Knee Problems in Severe Hemophilia: Orthopedic Management

Introduction

It is well known that in hemophilia, the knees tend to bleed beginning at an early age of 2–5 years. The synovium is only able to reabsorb a small amount of intra-articular blood; if the amount of blood is excessive, the synovium will hypertrophy as a compensating mechamism, so that eventually the affected joint will show an increase in the size of the synovium: so-called hypertrophic chronic hemophilic synovitis () The hypertrophic synovium is very richly vascularized, so that small injuries will easily make the joint rebleed. The final result will be the classic vicious cycle of hemarthrosis-synovitis-hemarthrosis 1, 2.

Abstract

Contemporary knowledge appears to demonstrate that radiosynovectomy is a very effective procedure, which decreases both the frequency and the intensity of recurrent intra-articular bleeds related to knee synovitis. The procedure should be performed as soon as possible to minimize the degree of articular cartilage damage. It can also be used in patients with inhibitors and with a minimal risk of complications. No damage has been reported in relation to the radioactive materials. Radiosynovectomy is currently the preferred procedure when radioactive materials are available; however, chemical synovectomy is an effective alternative method if radioactive materials are not available. Radiosynovectomy is the best choice for patients with persistent synovitis. Personal experience and the general recommendation is that when three early consecutive radiosynovectomies (repeated every 3 to 6 months) fail to halt synovitis, an arthroscopic synovectomy should be immediately considered. For advanced hemophilic arthropathy of the knee, the best solution is a total knee replacement. Other surgical and nonsurgical procedures are less commonly needed for the hemophilic knee.

Keywords

hemophilia, knee, management, review