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Airway management in Escobar syndrome: A formidable challenge

Escobar syndrome is a rare autosomal recessive disorder characterized by flexion joint and digit contractures, skin webbing, cleft palate, deformity of spine and cervical spine fusion. Associated difficult airway is mainly due to micrognathia, retrognathia, webbing of neck and limitation of the mout...

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Autores principales: Mathew, Shaji, Chaudhuri, Souvik, Arun Kumar, HD, Joseph, Tim Thomas
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3883398/
https://www.ncbi.nlm.nih.gov/pubmed/24403623
http://dx.doi.org/10.4103/0019-5049.123336
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author Mathew, Shaji
Chaudhuri, Souvik
Arun Kumar, HD
Joseph, Tim Thomas
author_facet Mathew, Shaji
Chaudhuri, Souvik
Arun Kumar, HD
Joseph, Tim Thomas
author_sort Mathew, Shaji
collection PubMed
description Escobar syndrome is a rare autosomal recessive disorder characterized by flexion joint and digit contractures, skin webbing, cleft palate, deformity of spine and cervical spine fusion. Associated difficult airway is mainly due to micrognathia, retrognathia, webbing of neck and limitation of the mouth opening and neck extension. We report a case of a 1 year old child with Escobar syndrome posted for bilateral hamstrings to quadriceps transfer. The child had adequate mouth opening with no evidence of cervical spine fusion, yet we faced difficulty in intubation which was ultimately overcome by securing a proseal laryngeal mask airway (PLMA) and then by intubating with an endotracheal tube railroaded over a paediatric fibreoptic bronchoscope passed through the lumen of a PLMA.
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spelling pubmed-38833982014-01-08 Airway management in Escobar syndrome: A formidable challenge Mathew, Shaji Chaudhuri, Souvik Arun Kumar, HD Joseph, Tim Thomas Indian J Anaesth Case Report Escobar syndrome is a rare autosomal recessive disorder characterized by flexion joint and digit contractures, skin webbing, cleft palate, deformity of spine and cervical spine fusion. Associated difficult airway is mainly due to micrognathia, retrognathia, webbing of neck and limitation of the mouth opening and neck extension. We report a case of a 1 year old child with Escobar syndrome posted for bilateral hamstrings to quadriceps transfer. The child had adequate mouth opening with no evidence of cervical spine fusion, yet we faced difficulty in intubation which was ultimately overcome by securing a proseal laryngeal mask airway (PLMA) and then by intubating with an endotracheal tube railroaded over a paediatric fibreoptic bronchoscope passed through the lumen of a PLMA. Medknow Publications & Media Pvt Ltd 2013 /pmc/articles/PMC3883398/ /pubmed/24403623 http://dx.doi.org/10.4103/0019-5049.123336 Text en Copyright: © Indian Journal of Anaesthesia 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 Case Report
Mathew, Shaji
Chaudhuri, Souvik
Arun Kumar, HD
Joseph, Tim Thomas
Airway management in Escobar syndrome: A formidable challenge
title Airway management in Escobar syndrome: A formidable challenge
title_full Airway management in Escobar syndrome: A formidable challenge
title_fullStr Airway management in Escobar syndrome: A formidable challenge
title_full_unstemmed Airway management in Escobar syndrome: A formidable challenge
title_short Airway management in Escobar syndrome: A formidable challenge
title_sort airway management in escobar syndrome: a formidable challenge
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3883398/
https://www.ncbi.nlm.nih.gov/pubmed/24403623
http://dx.doi.org/10.4103/0019-5049.123336
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