Korean J Anesthesiol.  2009 Dec;57(6):785-788. 10.4097/kjae.2009.57.6.785.

Anesthetic management in pediatric patient with Angelman syndrome: A case report

• Ok SJ
• Kim JH
• Son JH
• Kim WY
• Park YC

• KMID: 2239803
• DOI: http://doi.org/10.4097/kjae.2009.57.6.785

Abstract

The Angelman syndrome is characterized by an abnormality of chromosome 15, where a subunit of the gamma amino-butyric acid receptor is coded. The clinical features are developmental delay, microcephaly, wide mouth, prognathia which usually do not have problem with intubation. But, muscular atrophy may induce delayed recovery from neuromuscular blockade. Moreover, there are case reports that vagal hypertonia such as severe bardycardia or asystole occurred during anesthesia. We present a 5-year-9-month-old male Angelman syndrome patient who underwent a left and right rectus ophthalmicus muscle recession. We gave him prophylactic glycopyrrolate before anesthetic induction and induced and maintained anesthesia with sevoflurane and oxygen. After that we monitored train-of-four ratio for evaluation of neuromuscular blockade. There is no complication during operation and recovery from anesthesia.

Keyword

Angelman syndrome; Prophylactic glycopyrrolate; Vagal hypertonia

MeSH Terms

Anesthesia
Angelman Syndrome
Chromosomes, Human, Pair 15
Glycopyrrolate
Heart Arrest
Humans
Intubation
Male
Methyl Ethers
Microcephaly
Mouth
Muscles
Muscular Atrophy
Neuromuscular Blockade
Oxygen
Glycopyrrolate
Methyl Ethers
Oxygen