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 |
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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 |
format | Article |
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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.</description><identifier>ISSN: 0341-2695</identifier><identifier>EISSN: 1432-5195</identifier><identifier>DOI: 10.1007/s00264-016-3135-1</identifier><identifier>PMID: 26899603</identifier><language>eng</language><publisher>Berlin/Heidelberg: Springer Berlin Heidelberg</publisher><subject>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</subject><ispartof>International orthopaedics, 2016-07, Vol.40 (7), p.1361-1365</ispartof><rights>SICOT aisbl 2016</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c377t-bb8a2479d86c6a5bd662170fffeecc4583820c3e7b375b5d74b91701b8539c2a3</citedby><cites>FETCH-LOGICAL-c377t-bb8a2479d86c6a5bd662170fffeecc4583820c3e7b375b5d74b91701b8539c2a3</cites><orcidid>0000-0002-3631-7734</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s00264-016-3135-1$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s00264-016-3135-1$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,778,782,27907,27908,41471,42540,51302</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/26899603$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Sternick, Marcelo Back</creatorcontrib><creatorcontrib>Formentini, Pierina Kaneno Ishida</creatorcontrib><creatorcontrib>de Souza, Gustavo Moreira Costa</creatorcontrib><creatorcontrib>Teixeira, Eduardo Cembranelli</creatorcontrib><creatorcontrib>de Almeida Filho, Ildeu Afonso</creatorcontrib><creatorcontrib>da Costa, Sérgio Moreira</creatorcontrib><title>Treatment of feet deformities in epidermolysis bullosa</title><title>International orthopaedics</title><addtitle>International Orthopaedics (SICOT)</addtitle><addtitle>Int Orthop</addtitle><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.</description><subject>Adolescent</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Contracture - surgery</subject><subject>Epidermolysis Bullosa - complications</subject><subject>Female</subject><subject>Foot Deformities - etiology</subject><subject>Foot Deformities - surgery</subject><subject>Humans</subject><subject>Male</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Original Paper</subject><subject>Orthopedics</subject><subject>Recurrence</subject><subject>Retrospective Studies</subject><issn>0341-2695</issn><issn>1432-5195</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2016</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkLtOwzAUhi0EoqXwACwoI4vBl_iSEVXcpEosZbZs5wSlSuJiJ0PfHqMURsR0hvP9_9H5ELqm5I4Sou4TIUyWmFCJOeUC0xO0pCVnWNBKnKIl4SXFTFZigS5S2hFCldT0HC2Y1FUlCV8iuY1gxx6GsQhN0QCMRQ1NiH07tpCKdihg39YQ-9AdUpsKN3VdSPYSnTW2S3B1nCv0_vS4Xb_gzdvz6_phgz1XasTOactKVdVaemmFq6VkVJGmyYe8L4XmmhHPQTmuhBO1Kl2V99RpwSvPLF-h27l3H8PnBGk0fZs8dJ0dIEzJUC01J1xz-Q80_8-JyIJWiM6ojyGlCI3Zx7a38WAoMd9mzWzWZLPm26yhOXNzrJ9cD_Vv4kdlBtgMpLwaPiCaXZjikO380foF6-SCUg</recordid><startdate>20160701</startdate><enddate>20160701</enddate><creator>Sternick, Marcelo Back</creator><creator>Formentini, Pierina Kaneno Ishida</creator><creator>de Souza, Gustavo Moreira Costa</creator><creator>Teixeira, Eduardo Cembranelli</creator><creator>de Almeida Filho, Ildeu Afonso</creator><creator>da Costa, Sérgio Moreira</creator><general>Springer Berlin Heidelberg</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>7QP</scope><orcidid>https://orcid.org/0000-0002-3631-7734</orcidid></search><sort><creationdate>20160701</creationdate><title>Treatment of feet deformities in epidermolysis bullosa</title><author>Sternick, Marcelo Back ; Formentini, Pierina Kaneno Ishida ; de Souza, Gustavo Moreira Costa ; Teixeira, Eduardo Cembranelli ; de Almeida Filho, Ildeu Afonso ; da Costa, Sérgio Moreira</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c377t-bb8a2479d86c6a5bd662170fffeecc4583820c3e7b375b5d74b91701b8539c2a3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2016</creationdate><topic>Adolescent</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Contracture - surgery</topic><topic>Epidermolysis Bullosa - complications</topic><topic>Female</topic><topic>Foot Deformities - etiology</topic><topic>Foot Deformities - surgery</topic><topic>Humans</topic><topic>Male</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Original Paper</topic><topic>Orthopedics</topic><topic>Recurrence</topic><topic>Retrospective Studies</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Sternick, Marcelo Back</creatorcontrib><creatorcontrib>Formentini, Pierina Kaneno Ishida</creatorcontrib><creatorcontrib>de Souza, Gustavo Moreira Costa</creatorcontrib><creatorcontrib>Teixeira, Eduardo Cembranelli</creatorcontrib><creatorcontrib>de Almeida Filho, Ildeu Afonso</creatorcontrib><creatorcontrib>da Costa, Sérgio Moreira</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>Calcium & Calcified Tissue Abstracts</collection><jtitle>International orthopaedics</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Sternick, Marcelo Back</au><au>Formentini, Pierina Kaneno Ishida</au><au>de Souza, Gustavo Moreira Costa</au><au>Teixeira, Eduardo Cembranelli</au><au>de Almeida Filho, Ildeu Afonso</au><au>da Costa, Sérgio Moreira</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Treatment of feet deformities in epidermolysis bullosa</atitle><jtitle>International orthopaedics</jtitle><stitle>International Orthopaedics (SICOT)</stitle><addtitle>Int Orthop</addtitle><date>2016-07-01</date><risdate>2016</risdate><volume>40</volume><issue>7</issue><spage>1361</spage><epage>1365</epage><pages>1361-1365</pages><issn>0341-2695</issn><eissn>1432-5195</eissn><abstract>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.</abstract><cop>Berlin/Heidelberg</cop><pub>Springer Berlin Heidelberg</pub><pmid>26899603</pmid><doi>10.1007/s00264-016-3135-1</doi><tpages>5</tpages><orcidid>https://orcid.org/0000-0002-3631-7734</orcidid></addata></record> |
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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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