Characterizing Periprocedural Care for Pediatric Patients With Williams Syndrome Undergoing General Anesthesia at a Tertiary Pediatric Hospital.

Abstract

Williams syndrome is a rare congenital disorder affecting connective tissue and the cardiovascular and central nervous systems. Pediatric patients diagnosed with Williams syndrome face significant risk for cardiac collapse and death when undergoing anesthesia. We sought to evaluate our institution's historical practices, evaluate individual risk stratification, and create detailed standardized perianesthesia guidelines for management of this population, particularly during noncardiac procedures. The study included a retrospective chart review of pediatric patients with Williams syndrome who received anesthesia over a 10-year period at a single institution. A total of 23 patients underwent 46 procedures. At time of procedure, median age was 5.8 years (range, 0.4-17.6 yr), and the majority (n = 19) had a "low" Williams syndrome risk category and required anesthesia for noncardiac procedures. Most (61%) had no cardiac involvement beyond mild supravalvar or branch pulmonary artery stenosis. No intraprocedure adverse cardiac events were identified. One patient experienced an adverse cardiac event approximately 60 minutes postanesthesia emergence which included ST segment depression and development of refractory ventricular fibrillation, necessitating deployment of venoarterial extracorporeal membrane oxygenation. This patient was eventually stabilized and was taken to the operating room 3 days later for definitive severe supravalvar aortic stenosis repair. Patients with Williams syndrome have a reported risk for sudden cardiac death that is 25 to 100 times greater than that of the age-matched general population. The incidence of adverse cardiac events in our cohort of patients with Williams syndrome undergoing anesthesia was lower compared with previous studies, which reported rates ranging from 4.2% to 11%. These findings support the idea that risk stratification and institutional practice guidelines can aid anesthesia providers in making informed decisions, and standardization of pre-, intra-, and postprocedural care according to existing guidelines may further reduce risks. Our review offers valuable insight into historical anesthesia management and contributes to a broader interdisciplinary understanding of care provision for this high-risk group.