New Guidance Issued for Care of Children With Williams Syndrome

In an American Academy of Pediatrics clinical report, published online Jan. 21 in Pediatrics, recommendations are presented for the management of patients with Williams syndrome (WS).

Colleen A. Morris, M.D., from the University of Nevada in Reno, and Stephen R. Braddock, M.D., from Saint Louis University, reviewed the current literature and developed recommendations for health care supervision for children with WS.

The authors note that most children with WS have cardiovascular anomalies secondary to elastin arteriopathy; individuals with WS are at increased risk for myocardial ischemia, acute hemodynamic deterioration, and sudden death due to these abnormalities. Fifty percent of people with WS have hypertension, which can occur at any age; blood pressure measurement is recommended at well-child visits. In most patients, antihypertensive therapy successfully controls hypertension and improves vascular stiffness. When feasible, children with WS should be evaluated by an experienced pediatric cardiologist. When hypercalcemia is suspected, serum calcium determination should be obtained every four to six months up to age 2 years and every two years thereafter. Parents should be educated about the signs and symptoms of hypercalcemia. Developmental milestones may be delayed in WS, and children should be referred for early interventions for physical, occupational, and speech therapy evaluation and treatment. Because children with WS have a unique cognitive and behavioral profile, a psychoeducational evaluation is important for school-aged children.

“The pediatrician can use knowledge of the clinical manifestations and the natural history of WS to anticipate medical problems and educate the family,” the authors write.

Abstract/Full Text

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