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Beckwith-Wiedemann syndrome

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Beckwith-Wiedemann syndrome

Definition:

Beckwith-Wiedemann syndrome is characterized by a consistent set of symptoms including a large tongue (macroglossia), large organs (visceromegaly), large body size (macrosomia), umbilical hernia or omphalocele (hernia of the navel), and low blood sugar in the newborn (neonatal hypoglycemia). The cause is unknown.

Causes, incidence, and risk factors:

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

Affected children are often large at birth. Many have an abdominal wall defect, such as an umbilical hernia or omphalocele. They have a characteristic facial appearance with a gaping mouth and large tongue.

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 being most common).

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