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Division of Urology

Pediatric Urology Conditions

Prune Belly Syndrome

What is prune belly syndrome?

Prune belly syndrome, also known as triad syndrome or Eagle-Barrett syndrome, is characterized by three abnormalities:

The urinary tract is significantly affected in virtually all children with prune belly syndrome. The bladder usually does not empty very well, and children must be monitored for the development of chronic kidney disease.

A child with prune belly syndrome may also have other birth defects. The skeletal system, lungs, intestines and heart are most commonly affected. Girls may have abnormalities in their external genitalia as well.

What causes prune belly syndrome?

Prune belly syndrome is uncommon, occurring in approximately 1 in 30,000 to 40,000 live births. Boys make up the majority of the affected population, accounting for 95 percent of the cases.

The cause of prune belly syndrome is unknown; however, some cases have been reported in siblings, suggesting there may be a genetic component.

Prune belly syndrome develops as the fetus is growing before birth. One theory is that if a blockage develops in the urethra (the tube that drains urine from the bladder to the outside of the body) urine can build up in the bladder, causing pressure and forcing the bladder to stretch to the point where it does not work as well.  

What are the symptoms of prune belly syndrome?

Although children with prune belly syndrome often share common features, the severity of the symptoms can vary from mild to severe. The most common symptoms include:

How is prune belly syndrome diagnosed?

Often prune belly syndrome is diagnosed during routine fetal ultrasounds. If not diagnosed prenatally, the distinctive appearance of the abdomen and the scrotal exam will usually lead to a quick diagnosis after birth. For children who do not have all of the common outward features of prune belly syndrome, the first sign of concern may be a urinary tract infection which would prompt further testing by the child's physician. Additional diagnostic procedures may include the following:

Treatment for prune belly syndrome

Treatment for prune belly syndrome will vary from one child to another depending on the severity of symptoms. If a child with prune belly syndrome has other birth defects, he will need to be followed by several specialties who will work together to provide the best care. We frequently work closely with our colleagues in Nephrology to preserve bladder and kidney drainage and function and to prevent urinary tract infections. Some common interventions include:

Antibiotic prophylaxis: A low, once-per-day dose of an antibiotic may be ordered to prevent a urinary tract infection.

Improve urinary drainage:

Preserving testicular function:

Abdominal wall reconstruction: Reconstruction of the abdominal wall involves removal of the “prune-like” excess folds of skin and tightening of the abnormal muscles to create a smoother abdominal wall appearance.

Children with prune belly syndrome require careful observation throughout their lives. We will monitor your child’s bladder and kidney development and function on a regular basis.


Reviewed by: Jennifer Kirk, BSN, MSN, CPNP
Date: July 2012
 

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