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Definition Return to top
Beckwith-Wiedemann syndrome is a congenital (present from birth) growth disorder that causes large body size, large organs, and other symptoms.
Causes Return to top
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.
Symptoms Return to top
Exams and Tests Return to top
The signs of Beckwith-Wiedemann syndrome include:
Tests for Beckwith-Wiedemann syndrome include:
Treatment Return to top
Infants with low blood sugar may be treated fluids given through a vein ( intravenous solutions).
Defects in the abdominal wall may need to be repaired. The child must be watched closely for the development of tumors.
Outlook (Prognosis) Return to top
Children with Beckwith-Wiedemann syndrome who survive infancy do well, although no long-term follow-up information is available. Mental development appears to be normal to very slightly decreased. Swelling of the tongue can cause problems with feeding and sleeping.
Possible Complications Return to top
When to Contact a Medical Professional Return to top
If you have a child with Beckwith-Wiedemann syndrome and worrisome symptoms develop, call your pediatrician immediately.
Prevention Return to top
There is no known prevention for Beckwith-Wiedemann syndrome. Genetic counseling may be of value for families who would like to have additional children.Update Date: 5/12/2009 Updated by: Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.