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The outcome of Jarcho–Levin syndrome treated with a functional latissimus dorsi flap – A series of three cases

INTRODUCTION: Jarcho-Levin syndrome is manifested by vertebral body and rib malformations. Large rib defects with paradoxical chest motion lead to early deathdue to progressive respiratory insufficiency, hence it is a lethal syndrome. The only means of improving survival is early stabilisation of th...

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Autores principales: Gangurde, Bipin A., Raut, Binita, Mehta, Rujuta, Thatte, Mukund R.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3385396/
https://www.ncbi.nlm.nih.gov/pubmed/22754151
http://dx.doi.org/10.4103/0970-0358.96582
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author Gangurde, Bipin A.
Raut, Binita
Mehta, Rujuta
Thatte, Mukund R.
author_facet Gangurde, Bipin A.
Raut, Binita
Mehta, Rujuta
Thatte, Mukund R.
author_sort Gangurde, Bipin A.
collection PubMed
description INTRODUCTION: Jarcho-Levin syndrome is manifested by vertebral body and rib malformations. Large rib defects with paradoxical chest motion lead to early deathdue to progressive respiratory insufficiency, hence it is a lethal syndrome. The only means of improving survival is early stabilisation of the chest wall defect by containing the thoracic herniation. Nitcher et al. and Thatte et al. showed that reconstruction of the chest wall was life saving. Thatte et al. had postulated that early coverage of the lungs and thoracic contents with functional latissimus dorsi may prevent the visceral overgrowth and secondary pleural changes. MATERIALS AND METHODS: Our three cases which had medium- and long-term follow-up help to support this postulation. Three patients were assessed retrospectively. Their ages at surgery were 6 months, 8 months and 1 year, respectively. All had laboured breathing and paradoxical respiration. All of them were operated with ipsilateral latissimus dorsi flap. RESULTS: The results were evaluated clinically. The patients had reduced or no respiratory infections. The lung compliance improved and they had no tachypnoea on walking, running or playing. CONCLUSION: Hence, this can be used as a life-saving procedure for Jarcho-Levin syndrome on a long-term basis.
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spelling pubmed-33853962012-07-02 The outcome of Jarcho–Levin syndrome treated with a functional latissimus dorsi flap – A series of three cases Gangurde, Bipin A. Raut, Binita Mehta, Rujuta Thatte, Mukund R. Indian J Plast Surg Original Article INTRODUCTION: Jarcho-Levin syndrome is manifested by vertebral body and rib malformations. Large rib defects with paradoxical chest motion lead to early deathdue to progressive respiratory insufficiency, hence it is a lethal syndrome. The only means of improving survival is early stabilisation of the chest wall defect by containing the thoracic herniation. Nitcher et al. and Thatte et al. showed that reconstruction of the chest wall was life saving. Thatte et al. had postulated that early coverage of the lungs and thoracic contents with functional latissimus dorsi may prevent the visceral overgrowth and secondary pleural changes. MATERIALS AND METHODS: Our three cases which had medium- and long-term follow-up help to support this postulation. Three patients were assessed retrospectively. Their ages at surgery were 6 months, 8 months and 1 year, respectively. All had laboured breathing and paradoxical respiration. All of them were operated with ipsilateral latissimus dorsi flap. RESULTS: The results were evaluated clinically. The patients had reduced or no respiratory infections. The lung compliance improved and they had no tachypnoea on walking, running or playing. CONCLUSION: Hence, this can be used as a life-saving procedure for Jarcho-Levin syndrome on a long-term basis. Medknow Publications & Media Pvt Ltd 2012 /pmc/articles/PMC3385396/ /pubmed/22754151 http://dx.doi.org/10.4103/0970-0358.96582 Text en Copyright: © Indian Journal of Plastic Surgery http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Original Article
Gangurde, Bipin A.
Raut, Binita
Mehta, Rujuta
Thatte, Mukund R.
The outcome of Jarcho–Levin syndrome treated with a functional latissimus dorsi flap – A series of three cases
title The outcome of Jarcho–Levin syndrome treated with a functional latissimus dorsi flap – A series of three cases
title_full The outcome of Jarcho–Levin syndrome treated with a functional latissimus dorsi flap – A series of three cases
title_fullStr The outcome of Jarcho–Levin syndrome treated with a functional latissimus dorsi flap – A series of three cases
title_full_unstemmed The outcome of Jarcho–Levin syndrome treated with a functional latissimus dorsi flap – A series of three cases
title_short The outcome of Jarcho–Levin syndrome treated with a functional latissimus dorsi flap – A series of three cases
title_sort outcome of jarcho–levin syndrome treated with a functional latissimus dorsi flap – a series of three cases
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3385396/
https://www.ncbi.nlm.nih.gov/pubmed/22754151
http://dx.doi.org/10.4103/0970-0358.96582
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