Cargando…
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...
Autores principales: | , , , |
---|---|
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 |
_version_ | 1782518610021842944 |
---|---|
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. |
format | Online Article Text |
id | pubmed-5372410 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Medknow Publications & Media Pvt Ltd |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT kamalgeeta anaestheticmanagementofachildwithstonemansyndromelookbeforeyouleap AT guptaanju anaestheticmanagementofachildwithstonemansyndromelookbeforeyouleap AT batlasapna anaestheticmanagementofachildwithstonemansyndromelookbeforeyouleap AT guptanishkarsh anaestheticmanagementofachildwithstonemansyndromelookbeforeyouleap |