JOINT REPLACEMENT IN MORQUIO-BRAILSFORDS DISEASE

Morquio-Brailsford's disease belongs to the mucopolysaccharidoses, it shows growth-retarding with dysproportional dwarfism. Due to bone defo rmations and joint dysplasias very early severe arthritic changes arri se with serious functional disabilities, especially in the loaded lowe r extremities; this may lead to early alloplastic joint replacement su rgery. By means of two patients with respective bilateral THR and sing ular total knee replacement intraoperative complications and postopera tive follow up (up to 12 years) are represented casuistically. Unfavou rable anatomic properties (dysplasia, size of joints) were the main te chnical surgery problem; the use of individually manufactured implants might be recommended in some of these cases. The achieved subjective and objective functional results of our cases were - compared to the p reoperative situation - altogether quite satisfactory.