title: Surgical management of hands in children with recessive dystrophic epidermolysis bullosa: use of allogeneic composite cultured skin grafts.
abstract: Recessive dystrophic epidermolysis bullosa (RDEB) is characterised by progressive childhood hand syndactyly and flexion contractures, which can be managed surgically but require split thickness autografts to facilitate satisfactory postoperative healing. We report on the partial substitution, for autografts, of improved composite cultured skin (CCS) allografts. The structure and preparation of these CCSs is outlined and their application in the course of 16 operations performed on 7 RDEB children with syndactyly and flexor contractures of fingers is described. Hand contractures were released and web spaces were covered with local flaps and split thickness autografts, while adjacent sides of the digits and other areas, as well as donor sites were generally grafted with CCS. Morphologic and functional results with CCS were judged to be good to excellent, the average time to recurrence was increased approximately 2-fold and smaller autografts needed to be used. In addition, healed CCS-treated donor sites could provide superior donor sites for further surgery.
authors: Sydney Children's Hospital, Randwick, NSW, Australia.
authors: M
authors: M
authors: Eisenberg
authors: D
authors: D
authors: Llewelyn
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003286:Contracture / Q000209:etiology / Q000601:surgery; D046508:Culture Techniques / Q000379:methods; D016108:Epidermolysis Bullosa Dystrophica / Q000150:complications / Q000601:surgery*; D005260:Female; D005347:Fibroblasts / Q000166:cytology; D006227:Hand Deformities, Acquired / Q000209:etiology / Q000601:surgery*; D006801:Humans; D015603:Keratinocytes / Q000166:cytology; D008297:Male; D012867:Skin / Q000648:ultrastructure; D016038:Skin Transplantation / Q000379:methods*; D017768:Skin, Artificial; D016896:Treatment Outcome