Prune Belly Syndrome

Overview

What is prune belly syndrome?

Prune belly syndrome is a rare condition that is present at birth. Prune belly syndrome has three main features:

  • Your baby’s abdominal muscles are weak or absent. As a result, the skin on your baby’s belly is wrinkled like a prune.
  • Your baby boy’s testicles fail to drop from inside his body into his scrotum (the delicate sack of skin under the penis).
  • There are problems with how your baby’s urinary system (kidneys and bladder) has formed.

The syndrome can also affect other parts of the body including the heart, lungs, kidneys, intestines and skeletal system.

Prune belly syndrome has also been called 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).

Symptoms and Causes

What causes prune belly syndrome?

Prune belly syndrome is congenital (which means that they are born with it). No one knows the exact cause of prune belly syndrome.

Researchers have also seen prune belly syndrome in siblings, which may mean the problem is genetic (runs in the family). In addition, this condition has been seen in children with trisomy 18 and trisomy 21 (Down syndrome).

What are the signs of prune belly syndrome?

Prune belly syndrome has many signs -- over 50. Symptoms vary from baby to baby. Your healthcare provider will be able to make the diagnosis based on physical exam and tests.

The most common symptoms include:

  • Wrinkled or prune-like appearance of the abdominal wall. This is due to the absence or poor development of the abdominal muscles.
  • Large abdomen with loops of intestine that can be seen though the thin skin of the abdominal wall.
  • Testicles that fail to descend into the scrotum (undescended testicles, in males)
  • Urinary system problems including enlarged or blocked ureters, enlarged kidney, enlarged bladder, vesicoureteral reflux (backflow of urine up the urinary tract).
  • Stomach and intestinal problems.
  • Heart structural problems.
  • Musculoskeletal abnormalities, including scoliosis and club foot.

Diagnosis and Tests

How is prune belly syndrome diagnosed?

This syndrome can often be found during a fetal ultrasound while you are still pregnant. Your doctor can usually tell by looking at your baby after he’s born that he has prune belly syndrome.

Management and Treatment

How is prune belly syndrome treated?

Your baby’s treatment plan is based on their unique needs. Usually a team of specialists, depending on the organs affected, assist in developing the treatment plan. Many factors are considered, including:

  • Overall health of your baby at the time.
  • Age of your baby.
  • Extent of your baby’s medical problems.
  • Ability of your baby to tolerate/survive certain procedures and treatments.
  • Your child’s outlook for the future.
  • Your (the parent[s]) wishes .

In the mildest of cases, some babies with prune belly syndrome may only need antibiotics to treat or prevent urinary tract symptoms.

Most babies need several surgeries to repair the abdominal wall, genitals (to lower your boy’s testicles into the scrotum) and bladder and other urinary tract problems (urinary tract reconstruction).

Outlook / Prognosis

What can I expect if my child is diagnosed with prune belly syndrome?

The outlook for children with prune belly syndrome depends on the severity of their condition. Approximately 40% of children with prune belly syndrome will have normal kidney function despite other mild urinary tract problems and can grow to have fairly normal lives.

Some children with prune belly syndrome have medical problems throughout life. About 30% of those will need a kidney transplant in their lifetime. 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.

Living With

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 sensation when going to the bathroom.
  • Pain or pressure in the lower back or stomach.
  • Cloudy or dark urine.
  • Strong, bad-smelling urine.
  • Blood in urine.

A note from Cleveland Clinic

There’s a wide range of possible expectations if your baby is born with prune belly syndrome. Some babies do not survive while others live a relatively normal life. The care plan depends on the type and severity of body systems involved. Most patients need life-long follow up with their pediatrician, urologist, primary care provider, social worker/counselor and specialists including maternal-fetal medicine specialists, nephrologists, pulmonary and respiratory specialists.

Last reviewed by a Cleveland Clinic medical professional on 12/07/2020.

References

  • Genetic and Rare Diseases Information Center. Prune Belly Syndrome. (https://rarediseases.info.nih.gov/diseases/7479/prune-belly-syndrome)
  • Pomajzl AJ, Sankararaman S. . [Updated 2019 Nov 19]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Prune Belly Syndrome (https://www.ncbi.nlm.nih.gov/books/NBK544248/)
  • Accessed 6/8/2016.National Institutes of Health: Prune Belly Syndrome (http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3350267/)
  • National Organizations for Rare Disorders: Prune Belly Syndrome (http://rarediseases.org/rare-diseases/prune-belly-syndrome/)

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