EXPERIENCE IN THE SURGICAL-MANAGEMENT OF THE HAND IN DYSTROPHIC EPIDERMOLYSIS-BULLOSA

Between 1981 and 1990, 45 patients (80 hands) underwent 122 hand operations, involving extensive release of contractures and pseudosyndactyly, with split skin grafting of secondary defects. Other procedures included the use of Kirschner wires, tendon release and arthroplasty. A postoperative programme of long term night splintage was employed. Finger extension was significantly improved for up to 5 years postoperatively, with a mean extension deficit preoperatively of 139-degrees, at 1 year postoperatively 60-degrees (p < 0.0001) and 4 years 84-degrees (p < 0.05). Preoperatively, pseudosyndactyly was present in 64.3 % of web spaces, in comparison to 13.3 % at 1 year, 37 % at 4 years (p < 0.01) and 66.6% at 5 years (N.S.) postoperatively. Adduction contracture of the thumb was significantly improved for 2 years postoperatively. Functional assessment of 18 hands showed significant improvement postoperatively.