Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Singh, Suyash, Das, Kuntal K., Kumar, Raj
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2017
Materias:
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
_version_ 1783312886199746560
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
work_keys_str_mv AT singhsuyash lipomeningomyelocelewithfattyfilumterminaleinapatientwithjarcholevinsyndromearareassociationrequiringspecialattention
AT daskuntalk lipomeningomyelocelewithfattyfilumterminaleinapatientwithjarcholevinsyndromearareassociationrequiringspecialattention
AT kumarraj lipomeningomyelocelewithfattyfilumterminaleinapatientwithjarcholevinsyndromearareassociationrequiringspecialattention