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Anesthetic Considerations for Angelman Syndrome: Case Series and Review of the Literature

BACKGROUND: Angelman syndrome is a rare neurodevelopmental disorder characterized by intellectual disability, severe speech impairment, ataxia, seizures, happy demeanor, distinctive craniofacial features, high vagal tone, and gamma-amino butyric acid receptor abnormalities. The aim of this report is...

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Autores principales: Warner, Mary Ellen, Martin, David P, Warner, Mark A, Gavrilova, Ralitza H, Sprung, Juraj, Weingarten, Toby N
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Kowsar 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5903253/
https://www.ncbi.nlm.nih.gov/pubmed/29696118
http://dx.doi.org/10.5812/aapm.57826
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author Warner, Mary Ellen
Martin, David P
Warner, Mark A
Gavrilova, Ralitza H
Sprung, Juraj
Weingarten, Toby N
author_facet Warner, Mary Ellen
Martin, David P
Warner, Mark A
Gavrilova, Ralitza H
Sprung, Juraj
Weingarten, Toby N
author_sort Warner, Mary Ellen
collection PubMed
description BACKGROUND: Angelman syndrome is a rare neurodevelopmental disorder characterized by intellectual disability, severe speech impairment, ataxia, seizures, happy demeanor, distinctive craniofacial features, high vagal tone, and gamma-amino butyric acid receptor abnormalities. The aim of this report is to review our experience of patients with Angelman syndrome undergoing anesthetic management. METHODS: We retrospectively reviewed perioperative course of patients with Angelman syndrome who underwent procedures under anesthesia from 2000 to 2016. RESULTS: Six patients with Angelman syndrome underwent 18 procedures; 14 performed under general anesthesia, and 4 with monitored anesthetic care, many for minor procedures (e.g., dental and diagnostic). Five patients had profound developmental delay and were nonverbal and 4 of them had epilepsy. The perioperative courses were uncomplicated except a 2 year-old girl having an intraoperative bronchospasm, a 16 year-old girl requiring flumazenil administration, and 28 year-old man who was electively intubated with a videolaryngoscope because of airway management concerns. No patients were documented as having postoperative pain. CONCLUSIONS: Angelman syndrome patients often require anesthesia for relatively innocuous procedures, and their speech impairment and happy demeanor can confound postoperative pain assessment. Patients can have atypical responses to benzodiazepines. Craniofacial abnormalities can complicate airway management. Although not encountered in this series, anesthesiologists need to be aware that Angelman syndrome patients have developed malignant bradydysrhythmias while anesthetized.
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spelling pubmed-59032532018-04-25 Anesthetic Considerations for Angelman Syndrome: Case Series and Review of the Literature Warner, Mary Ellen Martin, David P Warner, Mark A Gavrilova, Ralitza H Sprung, Juraj Weingarten, Toby N Anesth Pain Med Research Article BACKGROUND: Angelman syndrome is a rare neurodevelopmental disorder characterized by intellectual disability, severe speech impairment, ataxia, seizures, happy demeanor, distinctive craniofacial features, high vagal tone, and gamma-amino butyric acid receptor abnormalities. The aim of this report is to review our experience of patients with Angelman syndrome undergoing anesthetic management. METHODS: We retrospectively reviewed perioperative course of patients with Angelman syndrome who underwent procedures under anesthesia from 2000 to 2016. RESULTS: Six patients with Angelman syndrome underwent 18 procedures; 14 performed under general anesthesia, and 4 with monitored anesthetic care, many for minor procedures (e.g., dental and diagnostic). Five patients had profound developmental delay and were nonverbal and 4 of them had epilepsy. The perioperative courses were uncomplicated except a 2 year-old girl having an intraoperative bronchospasm, a 16 year-old girl requiring flumazenil administration, and 28 year-old man who was electively intubated with a videolaryngoscope because of airway management concerns. No patients were documented as having postoperative pain. CONCLUSIONS: Angelman syndrome patients often require anesthesia for relatively innocuous procedures, and their speech impairment and happy demeanor can confound postoperative pain assessment. Patients can have atypical responses to benzodiazepines. Craniofacial abnormalities can complicate airway management. Although not encountered in this series, anesthesiologists need to be aware that Angelman syndrome patients have developed malignant bradydysrhythmias while anesthetized. Kowsar 2017-07-26 /pmc/articles/PMC5903253/ /pubmed/29696118 http://dx.doi.org/10.5812/aapm.57826 Text en Copyright © 2017, Anesthesiology and Pain Medicine http://creativecommons.org/licenses/by-nc/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.
spellingShingle Research Article
Warner, Mary Ellen
Martin, David P
Warner, Mark A
Gavrilova, Ralitza H
Sprung, Juraj
Weingarten, Toby N
Anesthetic Considerations for Angelman Syndrome: Case Series and Review of the Literature
title Anesthetic Considerations for Angelman Syndrome: Case Series and Review of the Literature
title_full Anesthetic Considerations for Angelman Syndrome: Case Series and Review of the Literature
title_fullStr Anesthetic Considerations for Angelman Syndrome: Case Series and Review of the Literature
title_full_unstemmed Anesthetic Considerations for Angelman Syndrome: Case Series and Review of the Literature
title_short Anesthetic Considerations for Angelman Syndrome: Case Series and Review of the Literature
title_sort anesthetic considerations for angelman syndrome: case series and review of the literature
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5903253/
https://www.ncbi.nlm.nih.gov/pubmed/29696118
http://dx.doi.org/10.5812/aapm.57826
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