Prune Belly (Eagle-Barrett) Syndrome

Also known as: Eagle-Barrett syndrome, triad syndrome

What is prune belly syndrome?

Prune belly syndrome is a rare birth defect occurring usually in boys where infants have a triad (three) of abnormalities. These include absence/poor development of abdominal muscles, undescended testicles and a big bladder with problems with their urinary tract which makes it difficult for them to empty their bladders. Infants in addition may have many other birth defects (skeletal, lungs, intestines and heart with girls having abnormalities in their external genitalia).
 

What causes prune belly syndrome? 

While the cause is unknown, there may be a genetic abnormality as other children in a family may have the same problem. Blockage in fetal life of the tube that drains the bladder may cause some of the bladder and urinary tract abnormalities.
 

What are the signs/symptoms of prune belly syndrome?

Along with the three signs/symptoms of an undeveloped wrinkly abdominal wall, urinary tract problems and undescended testicles, older children with prune belly syndrome may have recurrent urinary tract infection. Other birth defects that may occur with prune belly syndrome may cause a variety of other symptoms. Some infants may be stillborn or  not survive for more than a few months.
 

What are prune belly syndrome care options? 

Treatment varies and depends on the severity of the symptoms. Antibiotics and a number of different surgical options are available. 

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 3/23/2018 2:19:07 PM