Perioperative Care of a Child With Williams Syndrome

Karen M. Miller, Vidya Raman, Peter Winch, Joseph D. Tobias


The classical clinical and physical findings of Williams syndrome include 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. Of primary concern during perioperative care is congenital and acquired heart disease as perioperative sudden death related to abnormalities of the coronary arteries has been reported. We present a 15-month-old child with Williams syndrome, who required anesthetic care for an emergent laparotomy due to an acute abdomen. The potential perioperative implications of Williams syndrome are discussed and previous published reports are reviewed.

J Med Cases. 2016;7(10):420-425


Williams syndrome; Perioperative care; Chromosome; 7q11.23

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