Anesthetic considerations for Angelman syndrome: Case series and review of the literature

Mary Ellen Warner, David P. Martin, Mark A. Warner, Ralitza M Gavrilova, Juraj Sprung, Toby N. Weingarten

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

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. Theaimof this report is to review our experience of patients withAngelmansyndrome 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.

Original languageEnglish (US)
Article numbere57826
JournalAnesthesiology and Pain Medicine
Volume7
Issue number5
DOIs
StatePublished - Oct 1 2017

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Angelman Syndrome
Anesthetics
Airway Management
Postoperative Pain
Anesthesia
Craniofacial Abnormalities
Amino Acid Receptors
Flumazenil
Bronchial Spasm
Butyric Acid
Pain Measurement
Ataxia
Benzodiazepines
Intellectual Disability
General Anesthesia
Epilepsy
Tooth
Seizures

Keywords

  • Anesthesia
  • Angelman syndrome
  • Perioperative complications

ASJC Scopus subject areas

  • Anesthesiology and Pain Medicine

Cite this

Anesthetic considerations for Angelman syndrome : Case series and review of the literature. / Warner, Mary Ellen; Martin, David P.; Warner, Mark A.; Gavrilova, Ralitza M; Sprung, Juraj; Weingarten, Toby N.

In: Anesthesiology and Pain Medicine, Vol. 7, No. 5, e57826, 01.10.2017.

Research output: Contribution to journalArticle

Warner, Mary Ellen ; Martin, David P. ; Warner, Mark A. ; Gavrilova, Ralitza M ; Sprung, Juraj ; Weingarten, Toby N. / Anesthetic considerations for Angelman syndrome : Case series and review of the literature. In: Anesthesiology and Pain Medicine. 2017 ; Vol. 7, No. 5.
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