Anesthetic Management in Williams Syndrome

Preoperative Planning and Intraoperative Management

The American Society of Anesthesiologists recommends avoiding high concentrations of volatile anesthetics that cause excessive vasodilation and hypotension in patients with Williams syndrome, and instead using propofol or etomidate for induction with sevoflurane or isoflurane for maintenance, as these agents provide hemodynamic stability when carefully titrated 1
For muscle relaxation, the American Heart Association suggests using rocuronium or vecuronium, and avoiding succinylcholine due to risks in patients with connective tissue abnormalities, as well as avoiding atracurium and mivacurium due to histamine release that may trigger vasodilation and hypotension 1, 2

The American College of Cardiology recommends maintaining SpO2 ≥95% continuously to prevent hypoxemia-related myocardial stress, and considering central venous access for longer procedures or those with anticipated hemodynamic instability 1
The Society of Cardiovascular Anesthesiologists suggests maintaining strict normothermia throughout the procedure using forced-air warming devices and warmed intravenous fluids, as temperature extremes can trigger vasospasm and worsen coronary perfusion 1
All Williams syndrome patients require ICU or high-dependency unit admission for continuous cardiopulmonary monitoring for at least 24-48 hours, even after seemingly uncomplicated procedures, according to the American Society of Anesthesiologists 1