Children's Hospital Colorado
Urology

Eagle-Barrett Syndrome (Prune Belly Syndrome)

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What is Eagle-Barrett syndrome (prune belly syndrome)?

Eagle-Barrett syndrome, also called prune belly syndrome, is a rare condition that affects the abdomen, testes and bladder. The condition is characterized by a clinical triad, meaning that it typically has three characteristics:

  1. Poorly developed abdominal muscles
  2. Undescended testicles (testicles in the abdomen)
  3. Enlarged bladder and an enlarged ureter (tubes that drain urine from the kidneys to the bladder), also known as a megaureter

Boys with Eagle-Barrett syndrome may also have urethral differences, including congenital megalourethra (enlarged urethra) or urethral atresia (blockage of the urethra).

Children with Eagle-Barrett syndrome often have lung issues. These can stem from a lack of amniotic fluid (which surrounds a baby in the womb) in children who have urethral blockage. Lung issues can also arise from an abnormal or underdeveloped diaphragm (a muscle in the chest that helps you breathe) or abnormal abdominal muscles that are needed to help infants breathe.

Boys with Eagle-Barrett syndrome are at risk of future infertility as a result of undescended testicles and abnormal testicular and prostate development.

Who gets Eagle-Barrett syndrome?

Eagle-Barrett syndrome primarily affects boys, but girls may be affected as well. The syndrome is relatively uncommon, affecting about 1 in 40,000 infants.

What causes Eagle-Barrett syndrome?

The exact cause of the syndrome is not known but there have been cases of it in siblings, suggesting that there may be a genetic cause. Eagle-Barrett syndrome is usually diagnosed before birth using prenatal ultrasound and then confirmed with fetal MRI. The condition may be associated with an enlarged bladder, small lungs, and decreased amniotic fluid levels, if associated with urethral blockage.

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