Treatment of feet deformities in epidermolysis bullosa

Background Inherited epidermolysis bullosa is a rare disease characterised by mechanical fragility of the skin when under insignificant stress. The main consequences of epidermolysis bullosa, mainly the dystrophic type, despite pseudosyndactyly, are joint contractures and deformities in hands and fe...

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Veröffentlicht in:International orthopaedics 2016-07, Vol.40 (7), p.1361-1365
Hauptverfasser: Sternick, Marcelo Back, Formentini, Pierina Kaneno Ishida, de Souza, Gustavo Moreira Costa, Teixeira, Eduardo Cembranelli, de Almeida Filho, Ildeu Afonso, da Costa, Sérgio Moreira
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container_end_page 1365
container_issue 7
container_start_page 1361
container_title International orthopaedics
container_volume 40
creator Sternick, Marcelo Back
Formentini, Pierina Kaneno Ishida
de Souza, Gustavo Moreira Costa
Teixeira, Eduardo Cembranelli
de Almeida Filho, Ildeu Afonso
da Costa, Sérgio Moreira
description Background Inherited epidermolysis bullosa is a rare disease characterised by mechanical fragility of the skin when under insignificant stress. The main consequences of epidermolysis bullosa, mainly the dystrophic type, despite pseudosyndactyly, are joint contractures and deformities in hands and feet. In this study, we describe our experience treating patients suffering from epidermolysis bullosa, as far as feet deformities are concerned. Methods This is a retrospective analysis of a consecutive series of patients presenting feet deformities related to epidermolysis bullosa. Extension contractures of the toes, equinus and cavus deformities were treated with soft tissues surgery. Results Thirteen surgical procedures were done in six patients with feet deformities caused by epidermolysis bullosa. Of the feet operated 85.7 % extension contracture of the toes was asymptomatic at follow-up. However, 42.9 % developed hammertoe deformities. There were no recurrence or complications for other deformities. Subjectively, all patients declared themselves very satisfied with the results. Conclusion Foot deformities must be treated as early as possible, due to progressive disability for walking and pain symptoms. We considered that, despite long term complications, treatment was adequate and we recommend it. Level of Evidence Level IV.
doi_str_mv 10.1007/s00264-016-3135-1
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The main consequences of epidermolysis bullosa, mainly the dystrophic type, despite pseudosyndactyly, are joint contractures and deformities in hands and feet. In this study, we describe our experience treating patients suffering from epidermolysis bullosa, as far as feet deformities are concerned. Methods This is a retrospective analysis of a consecutive series of patients presenting feet deformities related to epidermolysis bullosa. Extension contractures of the toes, equinus and cavus deformities were treated with soft tissues surgery. Results Thirteen surgical procedures were done in six patients with feet deformities caused by epidermolysis bullosa. Of the feet operated 85.7 % extension contracture of the toes was asymptomatic at follow-up. However, 42.9 % developed hammertoe deformities. There were no recurrence or complications for other deformities. Subjectively, all patients declared themselves very satisfied with the results. Conclusion Foot deformities must be treated as early as possible, due to progressive disability for walking and pain symptoms. We considered that, despite long term complications, treatment was adequate and we recommend it. 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The main consequences of epidermolysis bullosa, mainly the dystrophic type, despite pseudosyndactyly, are joint contractures and deformities in hands and feet. In this study, we describe our experience treating patients suffering from epidermolysis bullosa, as far as feet deformities are concerned. Methods This is a retrospective analysis of a consecutive series of patients presenting feet deformities related to epidermolysis bullosa. Extension contractures of the toes, equinus and cavus deformities were treated with soft tissues surgery. Results Thirteen surgical procedures were done in six patients with feet deformities caused by epidermolysis bullosa. Of the feet operated 85.7 % extension contracture of the toes was asymptomatic at follow-up. However, 42.9 % developed hammertoe deformities. There were no recurrence or complications for other deformities. Subjectively, all patients declared themselves very satisfied with the results. Conclusion Foot deformities must be treated as early as possible, due to progressive disability for walking and pain symptoms. We considered that, despite long term complications, treatment was adequate and we recommend it. 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The main consequences of epidermolysis bullosa, mainly the dystrophic type, despite pseudosyndactyly, are joint contractures and deformities in hands and feet. In this study, we describe our experience treating patients suffering from epidermolysis bullosa, as far as feet deformities are concerned. Methods This is a retrospective analysis of a consecutive series of patients presenting feet deformities related to epidermolysis bullosa. Extension contractures of the toes, equinus and cavus deformities were treated with soft tissues surgery. Results Thirteen surgical procedures were done in six patients with feet deformities caused by epidermolysis bullosa. Of the feet operated 85.7 % extension contracture of the toes was asymptomatic at follow-up. However, 42.9 % developed hammertoe deformities. There were no recurrence or complications for other deformities. Subjectively, all patients declared themselves very satisfied with the results. 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source MEDLINE; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; Springer Nature - Complete Springer Journals
subjects Adolescent
Child
Child, Preschool
Contracture - surgery
Epidermolysis Bullosa - complications
Female
Foot Deformities - etiology
Foot Deformities - surgery
Humans
Male
Medicine
Medicine & Public Health
Original Paper
Orthopedics
Recurrence
Retrospective Studies
title Treatment of feet deformities in epidermolysis bullosa
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