Susan Nicholson, MD, Dept. of Anesthesia; and Kelli Deering and Paige Kaplan, MD, Division of Genetics, Children's Hospital of Philadelphia, Univ. of PA School of Medicine


Children and adults with Williams Syndrome (WS), a contiguous gene deletion syndrome, have medical issues that require procedures that should be done under general anesthesia. There is a perception that anesthesia carries greater risks in WS patients, arising from a number of isolated case reports published between 1961 and 1996 describing sudden death in 19 children with WS and congenital heart disease (CHD) who had undergone procedures both with and without anesthesia. The mechanism of death is incompletely understood.


A retrospective chart review of all the WS patients in this tertiary care children's hospital was done to evaluate the patients who had received an anesthetic in this hospital. 26 WS patients with CHD, ranging from mild to severe, between 6 months and 19 years of age underwent 60 surgical and cardiac catheterization procedures during a 13 year period (1987 to 2000).


There was a single adverse patient occurrence in an 18 month old boy with severe distal branch pulmonary stenosis and pulmonary hypertension. On emergence from anesthesia and tracheal extubation, his SpO2 fell, resulting in bradycardia. Resuscitative efforts, including chest compressions, administration of pharinacologic agents and reintubation were instituted with prompt return to baseline and full recovery.


Anesthesia can be safely administered to children with WS and CHD. A preoperative assessment to delineate the physiologic implications of the CHD, anesthetic care provided by a pediatric cardiac anesthesiologist who maintains the physiologic parameters within a narrow range, availability of resuscitative drugs/equipment, expeditious performance of the procedure with vigilance extending into the postoperative period enable children and adults with WS to benefit from anesthesia for their procedures.