Prune belly syndrome

Overview

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

1) Lack of development of abdominal muscles, causing the skin of the belly area to wrinkle like a prune

2) Undescended testicles

3) Urinary tract problems

Symptoms

Weak abdominal muscles can cause:

  • "Little Buddha" appearance
  • Constipation
  • Delay in sitting and walking
  • Difficulties coughing

Urinary tract problems can cause difficulty urinating.

Causes

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.

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.

Diagnosis

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

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.

Resources

Alternative Names : Eagle-Barrett syndrome