Prune belly syndrome is a group of rare birth defects that involves these three main problems:

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

Eagle-Barrett syndrome; Triad syndrome

The exact causes of prune belly syndrome are unknown. The condition affects mostly boys.

While in the womb (uterus), the developing baby's abdomen swells with fluid. Often, the cause is a problem in the urinary tract. The fluid disappears after birth, leading to a wrinkled abdomen that looks like a prune. This appearance is more noticeable due to the lack of abdominal muscles.

Weak abdominal muscles can cause:

• Constipation
• Delay in sitting and walking
• Difficulties coughing

Urinary tract problems can cause difficulty urinating.

Early surgery is recommended to fix weak abdominal muscles, urinary tract problems, and undescended testicles.

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

More information and support for people with prune belly syndrome and their families can be found at:

• Prune Belly Syndrome Network -- prunebelly.org

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

Many infants with this condition are either stillborn or die within the first few weeks of life. The cause of death is from severe lung or kidney problems, or from a combination of birth problems.

Some newborns survive and can develop normally. Others continue to have many medical and developmental problems.

Complications depend on the related problems. The most common are:

• Constipation
• Bone deformities (clubfoot, dislocated hip, missing limb, finger, or toe, funnel chest)
• Disease of the urinary tract (may need dialysis and a kidney transplant)

Undescended testicles can lead to infertility or cancer.

Prune belly syndrome is usually diagnosed before birth or when the baby is born.

If you have a child with diagnosed prune belly syndrome, contact your health care provider at the first sign of a urinary tract infection or other urinary symptoms.

If a pregnancy ultrasound shows that your baby has a swollen bladder or enlarged kidneys, talk to a specialist in high-risk pregnancy or perinatology.

There is no known way to prevent this condition. If the baby is diagnosed with a urinary tract obstruction before birth, in rare cases, surgery during the pregnancy may help prevent the problem from progressing to prune belly syndrome.

Published Date: December 31, 2023
Published By: Anna C. Edens Hurst, MD, MS, Associate Professor in Medical Genetics, The University of Alabama at Birmingham, Birmingham, AL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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