Evaluation of Perioperative Anesthetic Outcomes in Patients with Williams Syndrome Based on the Risk Stratification

INTRODUCTION: Williams syndrome (WS) is associated with morbidity and mortality during surgical and nonsurgical procedures. The primary objective of the study was to assess the incidence of perioperative mortality in patients with WS among the risk groups. The secondary objectives were to analyze the mortality cause and study the various perioperative outcomes among the WS risk groups. MATERIALS AND METHODS: In this retrospective observational study, we included patients with WS who underwent anesthesia for elective corrective surgical repair and nonoperative procedures like radiological imaging and cardiac catheterization procedures in our institute from January 2009 to April 2024. The patients were stratified into high-, medium-, and low-risk groups based on the risk factors recorded from the hospital medical records. We analyzed the incidence of perioperative mortality and associated outcomes among the three risk groups of patients with WS. RESULTS: Medical records of 30 patients with WS were studied. The incidence of mortality in the high-risk group was 11.8%, whereas there was no mortality in the other risk groups. The mortality occurred in high-risk group patients who underwent sedation with inhalational anesthesia for a radiological imaging procedure. There was no significant difference in the duration of mechanical ventilation, vasoactive inotropic score, or length of intensive care unit and hospital stay among the three risk groups. CONCLUSION: Mortalities were observed in the high-risk group who underwent sedation with inhalational anesthesia for radiological imaging. Preoperative risk stratification is needed to identify the risk category and alleviate the major perioperative cardiac events in patients with WS.