Happy Puppet Syndrome: A Case Report of Anesthetic Management

Angelman syndrome (AS) is a rare neurogenetic disorder that results from an abnormality of the maternal chromosome 15. Clinical presentations for AS include developmental delays, seizure disorders, ataxia, truncal hypotonia, scoliosis, structural cardiac abnormalities, hyperactive tendon reflexes, absent speech, and craniofacial anomalies. Patients with AS also may have vagal hypertonia, which can result in refractory bradycardia in the perioperative setting. Mutations of chromosome 15 can lead to abnormalities in γ-aminobutyric acid A (GABA_A) receptors, through which numerous anesthesia agents mediate their actions. Defects in GABA_A receptors may result in unpredictable reactions or a resistance to anesthetics. Because of high genetic heterogeneity, no conclusive evidence exists for the most appropriate anesthetic approach for patients with AS. Depending on the patient's AS classification and severity, current literature suggests minimizing the doses of all anesthetic agents. This case report details the anesthesia management of a pediatric patient with AS presenting for otolaryngologic surgery. The 4-year-old boy received general anesthesia with typical anesthetic agents administered at standard pediatric doses without incident. Despite the lack of adverse events in this case, clinicians must be aware of potential anesthetic complications that are unique to patients with AS and should proceed with caution.