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Beckwith-Wiedemann Syndrome
Definition 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).
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Overview, Causes, & Risk Factors
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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• Definition
• Overview, Causes, & Risk Factors
• Symptoms & Signs
• Prevention
• Diagnosis & Tests
• Treatment
• Prognosis
• Complications
• Support Groups
• Calling Your Health Care Provider
• Pictures & Images
Topics that might be of interest to you Diseases & Conditions
• Cushing's Syndrome - Adrenal Tumor
• Diastasis Recti
• Hypoglycemia
• Omphalocele
• Tumor
• Umbilical Hernia
• Undescended Testicle
• Wilms' Tumor
Tests & Exams
• Abdominal CT Scan
• Abdominal Film
• Bone X-Ray
• MRI
Other Topics
• Anterior
• Chromosome
• Etiology
• Fatigue
• Gestational Age
• Heimlich Maneuver
• Intravenous
• Long Bones
• Macroglossia
• Macrosomia
• Metopic Ridge
• Pinna Abnormalities and Low-Set Ears
• Poor Feeding in Infants
• Seizures
• Testes
• Visceromegaly
Review Date : 12/30/2002
Reviewed 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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