Anesthetic management of a patient with Williams syndrome

A 9-year-old boy with Williams syndrome was scheduled to receive dental treatment under general anesthesia. He had been diagnosed as having Williams syndrome based on the presence of characteristic features, including an elfin face, mental retardation, and congenital supravalvular aortic stenosis and hypercalcemia. Airway management using a mask technique was easily performed. Anesthesia was induced slowly with the inhalation of oxygen and sevoflurane. After obtaining an intravenous route, fentanyl and rocuronium were administered and the patient was intubated nasally. Tracheal intubation (using an Airway Scope®) was performed. The anesthesia was safely maintained using sevoflurane. Patients with Williams syndrome have a high risk of sudden death caused by arrhythmias or cardiac arrest. Therefore, special care to prevent heart failure during the perioperative period using a noninvasive hemodynamic monitor and appropriate management of the circulatory system, mental retardation, malignant hyperthermia, and hypercalcemia is needed in patients with this syndrome.