A new protocol for the treatment of hand deformities in recessive dystrophic epidermolysis bullosa (13 cases).
The recessive form of dystrophic epidermolysis bullosa creates severe hand deformities with disabling functional limitations in the main daily activities. Typically, the thumb is contracted in adduction, the first web space is obliterated, the palm and digits are contracted in flexion and interdigital spaces are lost (pseudo-syndactyly). In this paper, we present our experience with a protocol based on the association of various internationally developed techniques: brachial plexus anesthesia with ketamine sedation, dynamic splinting and coverage of the wounds with allogenic keratinocytes sheets. The overall results obtained in the first 13 patients showed a good tolerance of the procedure, no anesthesiologic complication and marked improvement of the hand deformities. The long-term follow-up revealed a recurrence before 2 years in 2 hands, between 2 and 4 years in 7 hands and after 4 years in 6 hands. The conclusion is that an aggressive surgical attitude, along with an adequate intra and post-operative rehabilitation, ensures a good restoration of hand function and a satisfying delay of inevitable recurrence.
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Pajardi
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Rafanelli
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D000203:Activities of Daily Living; D000771:Anesthesia, Intravenous; D000778:Anesthetics, Dissociative / Q000008:administration & dosage; D001917:Brachial Plexus; D002648:Child; D002675:Child, Preschool; D002985:Clinical Protocols; D003286:Contracture / Q000209:etiology / Q000601:surgery; D016108:Epidermolysis Bullosa Dystrophica / Q000235:genetics*; D005260:Female; D005500:Follow-Up Studies; D005808:Genes, Recessive; D006225:Hand / Q000503:physiopathology; D006227:Hand Deformities, Acquired / Q000209:etiology / Q000503:physiopathology / Q000534:rehabilitation / Q000601:surgery*; D006801:Humans; D015603:Keratinocytes / Q000637:transplantation; D007649:Ketamine / Q000008:administration & dosage; D008137:Longitudinal Studies; D008297:Male; D009407:Nerve Block; D026741:Physical Therapy Modalities; D011182:Postoperative Care; D012008:Recurrence; D013165:Splints; D013576:Syndactyly / Q000209:etiology / Q000601:surgery; D013933:Thumb / Q000473:pathology / Q000601:surgery; D014184:Transplantation, Homologous
D000778:Anesthetics, Dissociative; D007649:Ketamine