Ambulatory General Anesthesia for Dental Treatment in a Patient With Williams Syndrome and Supravalvular Aortic...

Williams syndrome (WS) is a genetic disorder marked by intellectual disability, distinctive craniofacial features, dental abnormalities, and congenital heart defects, particularly supravalvular aortic stenosis-a narrowing above the aortic valve. Patients with WS are at increased risk of perioperative sudden cardiac death, necessitating thorough cardiovascular assessment. Additionally, craniofacial anomalies such as micrognathia and retrognathia may lead to difficult airway management and often require awake intubation. However, awake intubation and hospitalization can be distressing for patients with WS due to their developmental challenges. These facts indicate the importance of identifying during the preoperative assessment for ambulatory general anesthesia those at low risk for cardiovascular complications and airway difficulties. This case report details the successful anesthetic management of a 9-year-old boy with WS undergoing ambulatory general anesthesia for dental treatment by maintaining a balance between myocardial oxygen supply and demand to minimize cardiovascular fluctuations and by paying careful attention to managing his airway. Our case highlights the feasibility and safety of ambulatory general anesthesia for patients with WS, minimizing hospitalization stress and ensuring patient safety.