What is prune belly syndrome?

Prune belly syndrome is a rare condition that is present at birth. It is known by its three main features:

  • There is a lack of or severe weakness of the muscles of the stomach. As a result, the baby’s stomach skin is wrinkled like a prune.
  • A baby boy’s testicles fail to drop from inside the body into the scrotum (the delicate sack of skin behind the penis).
  • There are problems with how the urinary system (urine flow system) has formed.

The syndrome can also affect other parts of the body. These include the heart, lungs, intestines and skeletal system. Other names for prune belly syndrome are triad syndrome and Eagle-Barrett syndrome.

Who gets prune belly syndrome?

Prune belly syndrome is rare. It happens only in about one in every 30,000 to 40,000 babies born. It mostly happens in boys (about 95% of the cases).

What causes prune belly syndrome?

Prune belly syndrome starts in a baby while it is still in its mother’s womb. No one knows the exact cause of prune belly syndrome. A baby might get it if there is a buildup of urine in the bladder, where it is stored. This may be caused by a block in the urethra, the tube that carries urine from the bladder out of the body.

If there is a block, the urine can flow backward instead of out, causing the bladder to get bigger. A large bladder can get in the way of the stomach muscles. This keeps the muscles from forming the right way. It also causes problems with the urinary system and testicles.

Sometimes, brothers and sisters have prune belly syndrome, which may mean the problem is genetic (runs in the family).

How is prune belly syndrome diagnosed?

This syndrome can often be found when the mother is still pregnant. This can be done using a routine test called a fetal ultrasound. The doctor can usually tell by looking at the baby after it’s born that it has prune belly syndrome. If the signs are mild, the doctor will use other tests to confirm or help find this condition. These tests include blood tests, X-rays, ultrasound and special tests called VCUG and IVP.

How is prune belly syndrome treated?

The doctor plans the treatment based on each patient’s own needs. He or she will consider many factors when making the plan. These include:

  • The health of the baby at the time
  • How bad the baby’s symptoms are
  • If the baby can handle certain treatments
  • The child’s outlook for the future
  • The wishes of the baby’s caregiver

Some babies with prune belly syndrome have symptoms that are very mild. They may be treated only with drugs that can keep germs from infecting the urinary system.

Other babies have symptoms that are more severe. They may need to see an urologist, a doctor who works with the urinary system and its problems. The child may need a small procedure to fix any issues. Or, he or she may need intense surgical repair. Baby boys may need a surgery to lower their testicles into the scrotum.

What is the outlook for a baby with prune belly syndrome?

The outlook for children with prune belly syndrome depends on how bad their symptoms are. Some babies with prune belly syndrome are not born alive or do not live long after birth. Those with milder forms of the syndrome do live. They can grow and have fairly normal lives.

Some people with prune belly syndrome have medical problems throughout life. About 30% of those will need a kidney transplant later in life. People living with prune belly syndrome need life-long follow-up care to make sure there are no problems with their urinary system. Follow-up care also helps reduce the possibility of any kidney damage.

When should I call my child’s doctor?

Infections in the urinary system, called urinary tract infections or UTIs, are bad for people with prune belly syndrome. Call your child’s healthcare provider at the first sign of a UTI. These signs include:

  • Fever or chills
  • Feeling of having to go to the bathroom more than usual
  • Strong feeling of having to go, but little comes out
  • Burning when going to the bathroom
  • Pain or pressure in the lower back or stomach
  • Cloudy or dark urine
  • Strong, bad-smelling urine

References

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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 7/8/2016…#16231