Perioperative care of the patient with Williams syndrome

Williams syndrome, initially described by Williams, Barratt-Boyes, and Lowe in 1961, consists of characteristic dysmorphic features, congenital heart disease, and distinctive behavioral and emotional traits. In addition to acquired and congenital heart disease, manifestations in the renal, endocrine, musculoskeletal, and central nervous system may have implications during the perioperative period. Congenital and acquired heart disease can be a significant issue as sudden death, related to abnormalities of the coronary arteries, has been reported perioperatively in these patients. The authors present a 7-month-old infant, previously diagnosed with Williams syndrome, who required anesthetic care for repair of subaortic and supravalvular aortic stenosis. The potential perioperative implications of Williams syndrome are discussed.