Health Encyclopedia

Prune belly syndrome

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 collecting structures.

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.

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.

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