Dental management of a pediatric patient with angelman syndrome under general anesthesia

Abstract

Angelman syndrome (AS) is a genetic disorder characterized by cognitive impairment, locomotor difficulties, distinctive behavioural patterns, and speech impairment. The syndrome is associated with a partial deletion of chromosome 15, which affects the subunit of gamma-aminobutyric acid (GABA) receptor. Many central nervous system drugs used in anesthesia operate through these receptors. Consequently, patients with AS, who have impaired GABA receptors, often require general anesthesia even for minor procedures. The anesthetic management of these patients can be challenging due to potential complications, including seizures, vagal nerve hyperfunction, craniofacial malformations, and peripheral muscular atrophy. In this report, we describe the administration of general anesthesia with sevoflurane for a nine-year-old boy with AS who was undergoing treatment for tooth decay.