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Anaesthetic management of a child with stone man syndrome: Look before you leap!

Stone Man syndrome or fibrodysplasia ossificans progressiva (FOP) is an extremely rare (1 in 2 million) genetic disorder characterised by ectopic ossification of the skeletal and connective tissues leading to progressive fusion of axial and appendicular skeleton. Surgery and anaesthesia-induced trau...

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Autores principales: Kamal, Geeta, Gupta, Anju, Batla, Sapna, Gupta, Nishkarsh
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/PMC5372410/
https://www.ncbi.nlm.nih.gov/pubmed/28405043
http://dx.doi.org/10.4103/0019-5049.202168
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author Kamal, Geeta
Gupta, Anju
Batla, Sapna
Gupta, Nishkarsh
author_facet Kamal, Geeta
Gupta, Anju
Batla, Sapna
Gupta, Nishkarsh
author_sort Kamal, Geeta
collection PubMed
description Stone Man syndrome or fibrodysplasia ossificans progressiva (FOP) is an extremely rare (1 in 2 million) genetic disorder characterised by ectopic ossification of the skeletal and connective tissues leading to progressive fusion of axial and appendicular skeleton. Surgery and anaesthesia-induced trauma can lead to disease flare-up if due precautions are not taken and disable the patient further. However, rarity of the disease may lead to its common misdiagnosis and anaesthesiologist may be caught unaware. There is relative paucity of literature regarding anaesthetic management of children with FOP. Videolaryngoscopes (VLs) provide a non-line-of-sight view and require less anterior force to visualise the glottis, may provide an alternative to fibreoptic intubation for airway management in such cases. Use of VL has only been reported once in an adult with FOP for nasotracheal intubation. We describe the successful anaesthetic management of an 11-year-old child with FOP and anticipated difficult airway.
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spelling pubmed-53724102017-04-12 Anaesthetic management of a child with stone man syndrome: Look before you leap! Kamal, Geeta Gupta, Anju Batla, Sapna Gupta, Nishkarsh Indian J Anaesth Case Report Stone Man syndrome or fibrodysplasia ossificans progressiva (FOP) is an extremely rare (1 in 2 million) genetic disorder characterised by ectopic ossification of the skeletal and connective tissues leading to progressive fusion of axial and appendicular skeleton. Surgery and anaesthesia-induced trauma can lead to disease flare-up if due precautions are not taken and disable the patient further. However, rarity of the disease may lead to its common misdiagnosis and anaesthesiologist may be caught unaware. There is relative paucity of literature regarding anaesthetic management of children with FOP. Videolaryngoscopes (VLs) provide a non-line-of-sight view and require less anterior force to visualise the glottis, may provide an alternative to fibreoptic intubation for airway management in such cases. Use of VL has only been reported once in an adult with FOP for nasotracheal intubation. We describe the successful anaesthetic management of an 11-year-old child with FOP and anticipated difficult airway. Medknow Publications & Media Pvt Ltd 2017-03 /pmc/articles/PMC5372410/ /pubmed/28405043 http://dx.doi.org/10.4103/0019-5049.202168 Text en Copyright: © 2017 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 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
Kamal, Geeta
Gupta, Anju
Batla, Sapna
Gupta, Nishkarsh
Anaesthetic management of a child with stone man syndrome: Look before you leap!
title Anaesthetic management of a child with stone man syndrome: Look before you leap!
title_full Anaesthetic management of a child with stone man syndrome: Look before you leap!
title_fullStr Anaesthetic management of a child with stone man syndrome: Look before you leap!
title_full_unstemmed Anaesthetic management of a child with stone man syndrome: Look before you leap!
title_short Anaesthetic management of a child with stone man syndrome: Look before you leap!
title_sort anaesthetic management of a child with stone man syndrome: look before you leap!
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5372410/
https://www.ncbi.nlm.nih.gov/pubmed/28405043
http://dx.doi.org/10.4103/0019-5049.202168
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