Prolonged intubation in a child with undiagnosed Williams syndrome following anesthesia

Williams syndrome is a rare genetic disorder characterized by supravalvular aortic stenosis and pulmonary artery stenosis. We report a previously healthy 52 day-old male with a loud systolic murmur undergoing revision inguinal herniorrhaphy after an uneventful anesthetic for the initial repair. Upon extubation, he developed labored breathing, which progressed to apnea and required reintubation. After treatment and observation overnight, he was successfully extubated without complication. Additional work-up revealed severe branch pulmonary artery stenosis bilaterally, leading to a Williams syndrome diagnosis. To our knowledge, this is the first reported case of undiagnosed Williams syndrome as a cause of prolonged intubation postoperatively.