Korean J Anesthesiol.  2010 Feb;58(2):207-210. 10.4097/kjae.2010.58.2.207.

Anesthesia of a dental patient with Angelman syndrome: A case report

• Kim BS
• Yeo JS
• Kim SO

• KMID: 2092279
• DOI: http://doi.org/10.4097/kjae.2010.58.2.207

Abstract

Angelman syndrome is characterized by a partial deficit of paired autosomal chromosome 15, which contains a subunit of the GABA (Gamma-Amino Butyric Acid) receptor. Many drugs that act on the CNS (Central Nerve System) during anesthesia are believed to exert their effects via the GABA receptors. We describe the anesthesia of a 7 year-old female patient with Angelman syndrome who underwent surgery for dental caries. The basic factors that needed to be considered when administering anesthesia to this patient were epilepsy, significant dominance of the vagal tone, craniofacial abnormalities and peripheral muscular atrophy. Inhalational anesthetics (sevoflurane) were employed for this patient. The patient had an uneventful peri-operative period and was discharged home on the same day of the operation.

Keyword

Anesthesia; Angelman syndrome; Chromosome 15; GABA receptor; Outpatient surgery

MeSH Terms

Ambulatory Surgical Procedures
Anesthesia
Anesthetics
Angelman Syndrome
Chromosomes, Human, Pair 15
Craniofacial Abnormalities
Dental Caries
Epilepsy
Female
gamma-Aminobutyric Acid
Humans
Muscular Atrophy
Polyenes
Receptors, GABA
Anesthetics
Polyenes
Receptors, GABA
gamma-Aminobutyric Acid