Graft-Free Surgical Technique for Treating Hand Deformities and Pseudosyndactyly in Patients With Epidermolysis Bullosa.

Abstract

Epidermolysis bullosa is a rare inherited skin disorder that leads to extreme sensitivity and blistering from even slight injuries. Frequent blistering on the hands can result in deformities, and in severe cases, the hands may become enclosed in epithelial tissue, a condition referred to as "mitten deformity." In this study, we aimed to develop a modified surgical technique for the treatment of hand deformities and pseudosyndactyly in patients with epidermolysis bullosa without the aid of any skin grafts or allografts. We performed a retrospective review of 14 hands affected by dystrophic epidermolysis bullosa who underwent reconstructive surgery for mitten hand deformity. The patients' quality of life, functional outcomes, web creep, and deformity were evaluated using appropriate questionnaires 1 year after surgery. Careful precautions were implemented in the operating room before surgery to avoid further damage. We began by detaching the syndactyly between the fingers, and then the flexion deformity of the fingers was corrected to the extent possible. Finger abduction and extension movements were achieved using a suitable half-ring Ilizarov apparatus and K-wires. Postoperative care included hand therapy, bracing, and dressing. No revision surgery or complications occurred within 1 year after surgery. Both unimanual and bimanual functions showed improvement. At the 1-year follow-up, there was a notable improvement in finger flexion deformity and web creep. In this study, we reported a modified surgical technique for the treatment of hand deformities in patients with epidermolysis bullosa. Improvements in both functional scores and deformity resulted from meticulous preoperative planning and appropriate anesthesia, the use of Ilizarov lengthening without skin grafts, careful postoperative dressing changes, and continued hand therapy at home. Therapeutic V.