Aim of study: In the final stages of haemophilic arthropathy of the knee jo
int, the preservation of walking ability is only possible by joint replacem
ent. Fibrotic ancylosis and severe deformities, being mostly bilaterally, m
ake the joint reconstruction difficult and impair the results. The purpose
of this study was to evaluate the results of total knee replacement (TKR) i
n haemophilia. Methods: From 1990 to 1998, 14 TKR in 7 patients with severe
haemophilia were performed. The mean age at operation was 47.3 years (rang
e, 27-62 years). The mean follow-up period was 3.7 years (range, 1 - 7 year
s). The TKR was performed bilaterally at the same time in 6 cases. In 1 cas
e, the bilateral TKR was done one after another with 6 months interval, in
10 knee joints, an unconstrained or semi-constrained surface replacement sy
stem was used. In 4 joints, a hinged prosthesis was required. Results: The
range of motion (extension-flexion) was improved from 0-23-69 degrees preop
eratively to 0-4-88 degrees at follow-up. The mean HSS Score increased from
34.5 points preoperatively to 77.9 points at follow-up. Perioperatively, n
o haemorrhages or early infections were observed. In 1 case, 6 years postop
eratively a late infection of the hinged knee prosthesis occurred. A two-st
age-exchange of the prosthesis was performed. Aseptic loosenings of prosthe
tic components were not observed. Conclusion: The TKR in haemophilla is tec
hnically demanding and requires a consequent perioperative F-VIII or F-IX s
ubstitution. A bilateral simultaneous implantation is useful. The indicatio
n for TKR has to be strict because of the higher risks and requires a close
cooperation with the haemostaseologist.