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Definition Return to top
Beckwith-Wiedemann syndrome is a consistent grouping of findings of unknown etiology (cause) and characterized by a large tongue (macroglossia), large organs (visceromegaly) and large body size (macrosomia), umbilical hernia or omphalocele (hernia of the navel), and low blood sugar in the newborn (neonatal hypoglycemia).Causes, incidence, and risk factors Return to top
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).
Symptoms Return to top
Signs and tests Return to top
The signs for Beckwith-Wiedemann syndrome are as follows:
The tests for Beckwith-Wiedemann syndrome are as follows:
Treatment Return to top
In infancy, hypoglycemia may be treated with intravenous solutions. Enlargement of the tongue can cause problems with feeding and sleeping. Defects of the abdominal wall may need to be repaired. In addition, affected children must be followed carefully for the appearance of tumors.
Support Groups Return to top
A support group for this syndrome can be found at www.beckwith-wiedemann.org.
Expectations (prognosis) Return to top
Children who survive infancy do well, although there is no long-term follow-up information available. Mental function appears to be normal to very slightly decreased.
Complications Return to top
Calling your health care provider Return to top
If you have a child with Beckwith-Wiedemann syndrome at home, any worrisome symptoms develop, call your health care provider immediately.
Prevention Return to top
There is no known prevention at this time. Genetic counseling may be of value for families.
Update Date: 12/30/2002 Updated by: A.D.A.M. editorial. Previous review: Adam Ratner, M.D., Children's Hospital of Philadelphia, Philadelphia, PA. Review provided by VeriMed Healthcare Network (2/1/2002).
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Page last updated: 28 October 2004 |