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Definition:

Prune belly syndrome is a group of birth abnormalities marked by three major findings:

  • Lack of development of abdominal muscles, causing the skin of the belly area to wrinkle like a prune
  • Undescended testicles
  • Urinary tract problems


Alternative Names:

Eagle-Barrett syndrome



Causes, incidence, and risk factors:

The underlying causes of prune belly syndrome are unknown. It affects mostly boys.

When in the womb, the developing baby's abdomen swells with fluid. That fluid disappears after birth, leading to a wrinkled abdomen that looks like a prune. The appearance is more noticeable because of the lack of enough abdominal muscles.



Symptoms:
  • Club foot or abnormal limbs (rare)
  • Undescended testicles
  • Wrinkled skin on belly area


Signs and tests:

Expectant mothers carrying affected infants may have varying degrees of oligohydramnios (not enough amniotic fluid), which make the infant likely to have lung problems.

An ultrasound done during pregnancy may show that the baby has a swollen bladder or enlarged kidney.

In some cases, a pregnancy ultrasound may also help determine if the baby has:

  • Heart problems
  • Musculoskeletal abnormalities
  • Stomach and intestinal problems
  • Underdeveloped lungs

The following tests may be performed on the baby after birth to diagnose the condition:

  • Blood tests
  • Intravenous pyelogram (IVP)
  • Ultrasound
  • Voiding cystourethrogram (VCUG)
  • X-ray


Treatment:

Surgery is commonly done to repair genital and bladder problems.

The baby may be given antibiotics to treat or help prevent urinary tract infections.



Support Groups:



Expectations (prognosis):

Prune belly syndrome is a serious and often life-threatening problem.

Many infants with prune belly syndrome are either stillborn or die within the first few weeks of life from severe lung or kidney problems, or a combination of birth problems.

Some newborns survive with varying degree of recurring problems.



Complications:

Complications depend on the related problems. The most common are chronic kidney failure and club foot.



Calling your health care provider:

Prune belly syndrome is usually diagnosed before birth or at the time of birth.

If you have a child with diagnosed prune belly syndrome, call your health care provider at the first sign of a urinary tract infection or other urinary symptoms. If, before birth, your baby is seen to have a distended bladder or enlarged kidneys on prenatal ultrasound, seek the advice of a specialist in high-risk pregnancy or perinatology.



Prevention:

There is no known guaranteed prevention. If a prenatal diagnosis of urinary tract obstruction is made, in rare cases surgery performed during the pregnancy may help prevent the problem from progressing to prune belly syndrome.




Review Date: 10/11/2007
Reviewed By: Deirdre O’Reilly, MD, MPH, Neonatologist, Division of Newborn Medicine, Children’s Hospital Boston and Instructor in Pediatrics, Harvard Medical School, Boston, Massachusetts. Review Provided by VeriMed Healthcare Network.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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