Soothing the soul
Beckwith-Wiedemann syndrome (BWS) is a growth disorder that's congenital, or present from birth. BWS is variable, meaning not all children have all the physical characteristics of the syndrome. Characteristics can include:
- Large tongue
- Large body or asymmetrical body growth
- Incomplete closure of the abdominal wall
- Low blood sugar, called hypoglycemia
- Increased frequency of tumors in the liver or kidneys
- Large lower jaw, prominent eyes and a small midface
Many children with BWS have a genetic abnormality on chromosome number 11. In some cases, however, no cause for the syndrome can be found.
A pediatrician or a medical geneticist — a doctor specializing in genetic conditions — may diagnose Beckwith-Wiedemann based on a physical examination.
Treatment varies from child to child, depending on what characteristics the baby has and how severe they are. Treatments may include:
- In newborns, treatment for low blood sugar, or hypoglycemia, is important to reduce the risk of central nervous system complications, which can result from insufficient nutrition to the brain.
- Newborns with defects in the abdominal wall will have the defect repaired.
- Infants with an insufficient airway (due to a large tongue) may need a breathing tube.
- Since large tongues can interfere with feeding, some children may need to be fed with special nipples or given tube feedings.
- Children should be screened for tumors, using abdominal ultrasound and serum alpha fetoprotein (AFP), every three months until 8 years of age, and tumors should be treated as needed.
- Children with very enlarged tongues may benefit from surgery to reduce the size of the tongue at 2 to 4 years of age. This procedure can lead to improved feeding and speech.
- Some children benefit from speech therapy.
- Surgery may be performed during puberty to equalize different leg lengths. Surgery may help with facial growth differences as well; the age when these surgeries are performed depends on the child's specific case.
- Orthodontic treatment is usually needed in adolescence.
UCSF Benioff Children's Hospitals medical specialists have reviewed this information. It is for educational purposes only and is not intended to replace the advice of your child's doctor or other health care provider. We encourage you to discuss any questions or concerns you may have with your child's provider.
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