Beckwith-Wiedemann Syndrome

Also known as: BWS

What is Beckwith-Wiedemann Syndrome?

Beckwith-Wiedemann syndrome was first described by doctors Bruce Beckwith and Hans Rudolph Wiedemann in the 1960’s. The doctors identified children who had a number of symptoms including body overgrowth, external intestines, enlarged tongues, and low blood sugar. When a child exhibited all of these characteristics, they described them as having Beckwith-Wiedemann syndrome. Today, approximately 1 in 13,700 people have the condition.

What are the clinical characteristics of BWS?

  • Macroglossia (large tongue)
  • Macrosomia (large body size) and/or hemihypertrophy (one side of body is bigger than the other side)
  • Neonatal hypoglycemia (low blood sugar as newborn babies)  
  • Increased risk for certain types of tumors such as Wilm's tumor (kidney tumor) and hepatoblastoma (liver tumor) 
  • Omphalocele (born with intestines on the outside of the body) or umbilical hernia
  • Creases or pits on the ears
  • Normal intelligence
example of creases on ear
baby with enlarged tongue
baby with enlarged tongue

What causes Beckwith-Wiedemann in children?

Beckwith-Wiedemann is a result of abnormal activity in the genes that regulate the body’s cell growth. While there is no single cause for the change of these genes, a geneticist can determine the source with special testing and evaluation. Most individuals with Beckwith-Wiedemann are the first affected in the family and the risk for recurrence is low. However, there may be a hereditary connection depending on the underlying cause.  

What is the prognosis for my child with Beckwith-Wiedemann?

The prognosis for most children with this condition is generally positive, and treatment varies by child. It is important to monitor your child’s tumor development, but most children with Beckwith-Wiedemann will attend school, have friends, and enjoy life. With close follow-up by the craniofacial team and/or clinical geneticists at Nicklaus Children’s Hospital, your child can grow to become a healthy and happy adult.

Reviewed by: Chad Perlyn, MD

This page was last updated on: February 06, 2020 02:42 PM

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