Pathophysiology-Driven Anesthetic Management of Williams Syndrome for Combined Oral Surgery: A Case Report

Williams syndrome is a rare multisystem genetic disorder associated with developmental delay, characteristic dysmorphic features, congenital cardiovascular lesions, and increased perioperative anesthetic risk, particularly when supravalvular aortic stenosis or other vasculopathy is present. Children with Williams syndrome may also present practical perioperative challenges related to anxiety, poor cooperation, and potential airway difficulty, making individualized anesthetic planning essential. We report a 12-year-old boy with Williams syndrome who underwent ranula excision and complete oral rehabilitation under general anesthesia. Although preoperative cardiac evaluation revealed no severe structural anomalies, he was managed as high risk due to the inherent vulnerability of coronary perfusion in Williams syndrome. The perioperative strategy combined tight hemodynamic control with pharmacologic and nonpharmacologic measures to minimize sympathetic activation. This case highlights that apparently mild structural cardiac findings may not fully reflect perioperative risk in Williams syndrome and supports a pathophysiology-driven, individualized anesthetic approach even for seemingly minor noncardiac procedures.