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Lipomeningomyelocele with Fatty Filum Terminale in a Patient with Jarcho–Levin Syndrome: A Rare Association Requiring Special Attention
Lipomeningomyelocele (LMMC) with Jarcho–Levin syndrome (JLS) is extremely rare. The syndromic association is one of LMMC with JLS is one of phenotype of spondylocostal dysostosis (SCD). SCD is an autosomal-recessive disorder characterized by defects in the vertebrae and abnormalities of the ribs in...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Medknow Publications & Media Pvt Ltd
2017
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5890566/ https://www.ncbi.nlm.nih.gov/pubmed/29675085 http://dx.doi.org/10.4103/jpn.JPN_108_17 |
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author | Singh, Suyash Das, Kuntal K. Kumar, Raj |
author_facet | Singh, Suyash Das, Kuntal K. Kumar, Raj |
author_sort | Singh, Suyash |
collection | PubMed |
description | Lipomeningomyelocele (LMMC) with Jarcho–Levin syndrome (JLS) is extremely rare. The syndromic association is one of LMMC with JLS is one of phenotype of spondylocostal dysostosis (SCD). SCD is an autosomal-recessive disorder characterized by defects in the vertebrae and abnormalities of the ribs in the form of segmental fusion, malalignment, or absence of a few ribs. These patients are prone to pulmonary insufficiency and repeated infections. Close anesthetic vigil is also needed to maintain optimal ventilation during surgery. We are reporting a case of 11-month-old male child presented with LMMC swelling and absent left-side multiple ribs. On further evaluation, we found hemivertebrae and scoliosis. This report highlights the experience of operating a child with JLS in prone position. |
format | Online Article Text |
id | pubmed-5890566 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Medknow Publications & Media Pvt Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-58905662018-04-19 Lipomeningomyelocele with Fatty Filum Terminale in a Patient with Jarcho–Levin Syndrome: A Rare Association Requiring Special Attention Singh, Suyash Das, Kuntal K. Kumar, Raj J Pediatr Neurosci Case Report Lipomeningomyelocele (LMMC) with Jarcho–Levin syndrome (JLS) is extremely rare. The syndromic association is one of LMMC with JLS is one of phenotype of spondylocostal dysostosis (SCD). SCD is an autosomal-recessive disorder characterized by defects in the vertebrae and abnormalities of the ribs in the form of segmental fusion, malalignment, or absence of a few ribs. These patients are prone to pulmonary insufficiency and repeated infections. Close anesthetic vigil is also needed to maintain optimal ventilation during surgery. We are reporting a case of 11-month-old male child presented with LMMC swelling and absent left-side multiple ribs. On further evaluation, we found hemivertebrae and scoliosis. This report highlights the experience of operating a child with JLS in prone position. Medknow Publications & Media Pvt Ltd 2017 /pmc/articles/PMC5890566/ /pubmed/29675085 http://dx.doi.org/10.4103/jpn.JPN_108_17 Text en Copyright: © 2018 Journal of Pediatric Neurosciences 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-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms. |
spellingShingle | Case Report Singh, Suyash Das, Kuntal K. Kumar, Raj Lipomeningomyelocele with Fatty Filum Terminale in a Patient with Jarcho–Levin Syndrome: A Rare Association Requiring Special Attention |
title | Lipomeningomyelocele with Fatty Filum Terminale in a Patient with Jarcho–Levin Syndrome: A Rare Association Requiring Special Attention |
title_full | Lipomeningomyelocele with Fatty Filum Terminale in a Patient with Jarcho–Levin Syndrome: A Rare Association Requiring Special Attention |
title_fullStr | Lipomeningomyelocele with Fatty Filum Terminale in a Patient with Jarcho–Levin Syndrome: A Rare Association Requiring Special Attention |
title_full_unstemmed | Lipomeningomyelocele with Fatty Filum Terminale in a Patient with Jarcho–Levin Syndrome: A Rare Association Requiring Special Attention |
title_short | Lipomeningomyelocele with Fatty Filum Terminale in a Patient with Jarcho–Levin Syndrome: A Rare Association Requiring Special Attention |
title_sort | lipomeningomyelocele with fatty filum terminale in a patient with jarcho–levin syndrome: a rare association requiring special attention |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5890566/ https://www.ncbi.nlm.nih.gov/pubmed/29675085 http://dx.doi.org/10.4103/jpn.JPN_108_17 |
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