Microsurgical Release of Pseudosyndactyly in Patients Affected by the Epidermolisis Bullosa (E.B.)

摘要

E.B. is a rare genodermatosis characterized by the presence of extremely fragile skin and recurrent blister formation due to minor traumas. Patients usually reach the 2nd-3rd decades of life: exitus is related to the cachettic state tied to the M.O.F. Authors present their experience in the surgical treatment of EB in the hand. From January 2001 to January 2005, 13 patients observed (7M, 6F). Range 10-40 years old. All with l-to-2 previous deglov-ing procedure elsewhere performed. Operated: 9, programmed: 2. All operations were performed in election, in local anesthesia and sedation, with micro-surgical technique. The complete wound healing of the hand was obtained within the 3rd week post op. Our protocol has shown the following advantages: minor op- and post-op anesthesiological risks; joints protection from anchilosis; a reduced hospital stay with a better compliance of the patient and parents; elimination of the risk of jatrogenic lesions; possibility to perform several surgical treatment; partial functional recovery of the hand (mean follow up 1,8 years).