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A 10-year follow-up of asymptomatic Charcot hip joints caused by CIPA syndrome (congenital insensitivity to pain with anhidrosis) with failure of any surgical reconstructive treatment

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare genetic disorder. Patients with CIPA lack among other things the protective sensation of pain. Orthopaedic manifestations are fractures, infections and hip joint dislocation. We report a 10-year follow-up of late developing hip dyspla...

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Autores principales: Delniotis, Ioannis, Leidinger, Benedikt
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6537910/
https://www.ncbi.nlm.nih.gov/pubmed/31186831
http://dx.doi.org/10.1093/jscr/rjz154
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author Delniotis, Ioannis
Leidinger, Benedikt
author_facet Delniotis, Ioannis
Leidinger, Benedikt
author_sort Delniotis, Ioannis
collection PubMed
description Congenital insensitivity to pain with anhidrosis (CIPA) is a rare genetic disorder. Patients with CIPA lack among other things the protective sensation of pain. Orthopaedic manifestations are fractures, infections and hip joint dislocation. We report a 10-year follow-up of late developing hip dysplasia with CIPA and the result of several reconstructive surgical procedures. The final outcome was asymptomatic Charcot hip joints with superior migration and dislocation of both hips without pain. In conformance with the literature standard operative techniques in these rare cases usually seem to fail. A multidisciplinary conservative approach with close observation and activity modification is the proposed treatment of choice.
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spelling pubmed-65379102019-06-11 A 10-year follow-up of asymptomatic Charcot hip joints caused by CIPA syndrome (congenital insensitivity to pain with anhidrosis) with failure of any surgical reconstructive treatment Delniotis, Ioannis Leidinger, Benedikt J Surg Case Rep Case Report Congenital insensitivity to pain with anhidrosis (CIPA) is a rare genetic disorder. Patients with CIPA lack among other things the protective sensation of pain. Orthopaedic manifestations are fractures, infections and hip joint dislocation. We report a 10-year follow-up of late developing hip dysplasia with CIPA and the result of several reconstructive surgical procedures. The final outcome was asymptomatic Charcot hip joints with superior migration and dislocation of both hips without pain. In conformance with the literature standard operative techniques in these rare cases usually seem to fail. A multidisciplinary conservative approach with close observation and activity modification is the proposed treatment of choice. Oxford University Press 2019-05-28 /pmc/articles/PMC6537910/ /pubmed/31186831 http://dx.doi.org/10.1093/jscr/rjz154 Text en Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2019. http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Case Report
Delniotis, Ioannis
Leidinger, Benedikt
A 10-year follow-up of asymptomatic Charcot hip joints caused by CIPA syndrome (congenital insensitivity to pain with anhidrosis) with failure of any surgical reconstructive treatment
title A 10-year follow-up of asymptomatic Charcot hip joints caused by CIPA syndrome (congenital insensitivity to pain with anhidrosis) with failure of any surgical reconstructive treatment
title_full A 10-year follow-up of asymptomatic Charcot hip joints caused by CIPA syndrome (congenital insensitivity to pain with anhidrosis) with failure of any surgical reconstructive treatment
title_fullStr A 10-year follow-up of asymptomatic Charcot hip joints caused by CIPA syndrome (congenital insensitivity to pain with anhidrosis) with failure of any surgical reconstructive treatment
title_full_unstemmed A 10-year follow-up of asymptomatic Charcot hip joints caused by CIPA syndrome (congenital insensitivity to pain with anhidrosis) with failure of any surgical reconstructive treatment
title_short A 10-year follow-up of asymptomatic Charcot hip joints caused by CIPA syndrome (congenital insensitivity to pain with anhidrosis) with failure of any surgical reconstructive treatment
title_sort 10-year follow-up of asymptomatic charcot hip joints caused by cipa syndrome (congenital insensitivity to pain with anhidrosis) with failure of any surgical reconstructive treatment
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6537910/
https://www.ncbi.nlm.nih.gov/pubmed/31186831
http://dx.doi.org/10.1093/jscr/rjz154
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