Prune Belly Syndrome in Children

What is prune belly syndrome in children?

Prune belly syndrome is a rare type of birth defect. The defect can range from mild to severe. It is also known as triad syndrome or Eagle-Barrett syndrome. It includes 3 (a triad) main problems:

• Missing or severe weakness of muscles in the belly (abdomen)
• One or both testicles not in the scrotal sac (undescended testicles)
• Abnormal, large bladder and problems with the kidneys and the tubes that lead from the kidneys to the bladder (ureters)

A child with prune belly syndrome often can't completely empty his or her bladder. This can cause serious bladder, ureter, and kidney problems.

A child with prune belly syndrome may also have other birth defects. Most often, these defects involve the skeletal system, intestines, lungs, and heart. Girls may have defects in their external genitals.

Some babies who have prune belly syndrome may die in the uterus at 20 weeks of pregnancy or later (are stillborn). Some babies with this condition die a few months after birth.

What causes prune belly syndrome in a child?

The cause of prune belly syndrome is not known. Some cases have occurred in siblings, which means a gene may be a cause.

It may occur if there is a blockage of the urethra during a baby’s growth in the uterus. The urethra is the tube that drains urine from the bladder to the outside of the body. A blockage prevents urine from flowing through the urinary tract. Urine can reverse flow and cause the bladder to become large.

Which children are at risk for prune belly syndrome?

Prune belly syndrome is not common. It occurs in about 1 in 30,000 to 40,000 births. Most cases occur in boys.

What are the symptoms of prune belly syndrome in a child?

Symptoms can occur a bit differently in each child, and may be mild to severe. They can include:

• Belly (abdomen) that looks wrinkly with multiple folds of skin
• Bladder causing a bulge in the abdomen above the pubic bones
• Urinary tract organs easy to feel through the abdomen
• Outline of the intestines seen through the abdomen
• No testes in the scrotum (baby boys)
• Trouble sitting upright or walking, because of weak muscles in the abdomen
• Frequent urinary tract infections if the urinary tract is blocked. This is uncommon in children younger than age 5 and unlikely in boys.

The symptoms of prune belly syndrome can be like other health conditions. Make sure your child sees his or her healthcare provider for a diagnosis.

How is prune belly syndrome diagnosed in a child?

Often prune belly syndrome is diagnosed by ultrasound while a woman is still pregnant. Or a healthcare provider can often make a diagnosis after a newborn baby’s first exam. Children who are diagnosed later often have urinary tract infections. The healthcare provider will ask about your child’s symptoms and health history. The provider will give your child a physical exam. Your child may also have tests such as:

• Intravenous pyelogram (IVP). This is an imaging test that uses an X-ray to view the urinary tract. A contrast dye is given so that the tract can be seen on film. An IVP also shows the rate and path of urine flow through the urinary tract.
• Voiding cystourethrogram (VCUG). This is a type of X-ray of the urinary tract. The healthcare provider puts a thin, flexible tube (catheter) into the tube that drains urine from the bladder to the outside of the body (the urethra). The provider fills the bladder with a liquid dye. The provider takes X-ray images as the bladder fills and empties. The images will show if there is any reverse flow of urine into the ureters and kidneys.
• Kidney (renal) ultrasound. This imaging test uses sound waves and a computer to create images of blood vessels, tissues, and organs. The test can show internal organs as they function, and help assess blood flow through vessels.
• Blood tests. These are done to see how well your child's kidneys function.

How is prune belly syndrome treated in a child?

Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is. Your child may see a urologist. This is a doctor who treats the urinary tract and the male genital tract. 

If your child has mild prune belly syndrome, he or she may take antibiotics to prevent urinary tract infections.

Some children may need surgery. A vesicostomy is surgery to make a small opening in the bladder through the abdomen to empty the bladder of urine. Other children may need to have surgery to rebuild the abdominal wall and urinary tract. In boys, a surgery to advance the testes into the scrotum may be done. This surgery is called orchiopexy.

Talk with your child’s healthcare providers about the risks, benefits, and possible side effects of all treatments.

What are possible complications of prune belly syndrome in a child?

Even with treatment, some children may develop kidney failure.

When should I call my child’s healthcare provider?

Call the healthcare provider if your child has:

• Symptoms that don’t get better, or get worse
• New symptoms

Key points about prune belly syndrome in children

• Prune belly syndrome is a rare type of birth defect.
• It includes 3 main problems. A child will have missing or severe weakness of muscles in the belly. One or both testicles are not in the scrotal sac (undescended testicles). And the bladder is large and there are problems with the kidneys and ureters.   
• A child with prune belly syndrome often can't completely empty his or her bladder. This can cause serious bladder, ureter, and kidney problems.
• If your child has mild prune belly syndrome, he or she may take antibiotics to prevent urinary tract infections. Some children may need surgery to fix the problems of prune belly syndrome.
• Even with treatment, some children may develop kidney failure.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

• Know the reason for the visit and what you want to happen.
• Before your visit, write down questions you want answered.
• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
• Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
• Ask if your child’s condition can be treated in other ways.
• Know why a test or procedure is recommended and what the results could mean.
• Know what to expect if your child does not take the medicine or have the test or procedure.
• If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
• Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.