Outcomes After Tonsillectomy in Children With Angelman Syndrome

ImportanceAngelman syndrome is a rare genetic disorder characterized by developmental delay, sleep disturbances, and a happy demeanor. Tonsillectomies are common procedures for individuals with Angelman syndrome, and their postoperative recovery may be complicated by challenging pain assessments, respiratory complications, or feeding difficulties. Inappropriate laughing may mask perioperative pain and anxiety.ObjectiveThe objective of this study is to review postoperative outcomes and complications of children with Angelman syndrome undergoing tonsillectomy.Methods (Design, Setting, Participants, Intervention, Measures)We conducted a retrospective review of patients with Angelman syndrome undergoing tonsillectomies from 2000 to 2024 in a quaternary pediatric hospital. Demographic, clinical, and surgical outcome variables were collected.ResultsTwelve children with Angelman syndrome underwent tonsillectomy: 7 for sleep-disordered breathing, 4 for sialorrhea, and 1 for recurrent tonsillitis. Median (IQR) duration of stay was 4.0 (3.0-5.3) days. The most common reason for prolonged hospital stay was inadequate oral intake. Nine (75.0%) children experienced postoperative complications, most frequently pooling of secretions and oxygen desaturations. Three children (25.0%) experienced severe postoperative complications, including 1 opioid overdose, 1 respiratory distress, and 1 aspiration pneumonia. Two patients were readmitted to the hospital: 1 for irregular breathing and poor pain control, and 1 for epistaxis.ConclusionThe postoperative course following tonsillectomy in children with Angelman syndrome can be complicated by a prolonged recovery, inadequate pain control, opioid toxicity, respiratory complications, and poor oral intake. Caregiver input on pain behavior is critical to develop an effective postoperative management strategy.RelevanceBased on our results and a literature review, we have created recommendations for post-tonsillectomy care in children with Angelman syndrome.