What is Beckwith-Wiedemann Syndrome?
Beckwith-Wiedemann Syndrome (BWS) is a condition that affects many parts of the body. It is classified as an overgrowth syndrome, which means that affected infants are considerably larger than normal and continue to grow and gain weight at an unusual rate during childhood. Growth begins to slow by about age 8 and adults with this condition are not always unusually tall.
In some children with Beckwith-Wiedemann Syndrome specific parts of the body may grow abnormally large, leading to an asymmetric or uneven appearance. This unusual growth pattern is known as hemihyperplasia.
Beckwith-Wiedemann Syndrome affects an estimated 1 in 11,000 newborns. The condition may actually be more common than this estimate because some people with mild or unusual symptoms are never diagnosed.
Signs and Symptoms of BWS
Outlook
Most children with Beckwith-Wiedemann syndrome and isolated hemihypertrophy grow up to be healthy adults.
The physical features of Beckwith-Wiedemann syndrome often become less noticeable as children grow. They are often larger than their peers during childhood, but their growth slows as they get older. By adolescence, growth tends to normalize and cancer risk decreases. They generally grow up to be adults of above average height.
Adults with these disorders can lead a normal life and have healthy children. They typically have normal intelligence and normal lifespans. Some of the visible, physical signs of Beckwith-Wiedemann syndrome, such as a disparity in leg length or an enlarged tongue, may require surgical correction, but most of the characteristics become less apparent with time.
Cancer Risks
Children with Beckwith-Wiedemann syndrome and isolated hemihypertrophy are at an increased risk of developing certain cancers during childhood. The risk of developing cancer is estimated to be 5 to 10 percent, but that risk may vary depending on the specific genetic cause of the child’s condition.
- Wilms' tumor, a cancer of the kidney, is the most common cancer found in these children. Ninety five percent of Wilms' tumors occur in Beckwith-Wiedemann syndrome or hemihypertrophy patients by age 7.
- Hepatoblastoma, a cancer of the liver, is the second-most common cancer in patients with Beckwith-Wiedemann syndrome or isolated hemihypertrophy. This cancer usually develops by 2 years of age.
- Rarely, adrenocortical carcinoma, neuroblastoma or rhabdomyosarcoma.