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Recognition and Management of the Infant With BeckwithWiedemann Syndrome

Spivey, Pamela S. MSN, RN, CNS; Bradshaw, Wanda T. MSN, RN, NNP-BC

Section Editor(s): MERRITT, LINDA RNC, MSN

doi: 10.1097/ANC.0b013e3181c2003f
Focus on the Physical: CE 2.3 HOURS Continuing Education

Beckwith–Wiedemann Syndrome (BWS) is the most common overgrowth syndrome in infancy. The characteristic findings are macroglossia, abdominal wall defects, and macrosomia. Genetic studies in infants with BWS demonstrate 3 major subgroups of patients: familial, sporadic, or chromosomally abnormal. Recognition in the neonatal period is important because of the high incidence of childhood malignant tumors associated with BWS. This article provides an overview of the syndrome and discusses its etiology, physical findings, and diagnostic evaluation. Management and clinical implications including family support will also be discussed.

Levine Children's Hospital, Department of Neonatology, Charlotte, North Carolina.

Address correspondence to Pamela S. Spivey, MSN, RN, CNS, Levine Children's Hospital, Charlotte, NC 28204; pamspivey43@yahoo.com.

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© 2009 National Association of Neonatal Nurses