Beckwith-Wiedemann syndrome : Overview, Causes, & Risk Factors

Definition

Beckwith-Wiedemann syndrome is a congenital (present from birth) growth disorder that causes large body size, large organs, and other symptoms.

Overview, Causes, & Risk Factors

The cause of Beckwith-Wiedemann syndrome is unknown, but it may be genetic. Most cases are associated with a defect in chromosome number 11.

Infancy can be a critical period because of low blood sugar (hypoglycemia), omphalocele (when present), and an increased rate of tumor development. Wilm’s tumor and adrenal carcinoma are the most common tumor’s in patients with this syndrome.

Pictures & Images

Beckwith-Wiedemann syndrome

Beckwith-Wiedemann syndrome in the newborn is a consistent group of findings of unknown cause and is characterized by large tongue (macroglossia), large organs (visceromegaly), large body size (macrosomia), hernia of the navel (omphalocele) and small head (microcephaly).

Metopic ridge

Metopic ridge

A metopic ridge is a ridge of bone or suture line on the forehead between the two halves of the frontal bone. The ridging is caused when the two halves close prematurely.


Review Date : 10/31/2009
Reviewed By : Linda J. Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington, School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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