Surgical treatment and postoperative splinting of recessive dystrophic epidermolysis bullosa

Digital contractures and pseudosyndactyly, common manifestations in recessive dystrophic epidermolysis bullosa, cause significant functional impairment. The deformities progress with time, although surgery may delay the progression. The role of surgical intervention, hand therapy, and the use of prolonged splinting was examined in seven children (nine hands) with recessive dystrophic epidermolysis bullosa with an average age of 5 years, 8 months (range, 1 year, 10 months to 16 years, 4 months). The technique of surgery, postoperative regimen, and splinting differ from those previously reported. Surgery includes "de-cocooning" the hand and fingers, manipulating contracted joints, and full-thickness skin grafting to dermal defects. Surgery and the postoperative regimen of rigid night splints and web-retaining gloves for day wear has allowed arrest or minimal progression of contractures in complaint patients in short-term follow-up study of an average of 17 months (range, 12-28 months). An interdisciplinary team of physicians and surgeons, therapists, and nurses makes this care regimen possible and influences family compliance.