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Genetic and Rare Diseases Information Center (GARD)

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Prune belly syndrome


Other Names for this Disease
  • Abdominal muscles, absence of, with urinary tract abnormality and cryptorchidism
  • Eagle-Barrett syndrome
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Your Question

My daughter was born with prune belly syndrome. She is missing her stomach muscles, but has no other issues. We have been told that she needs to protect her stomach from accidental injury at school.  Do you have information on treatment? Is there anything that can be done to let her live a normal life?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is prune belly syndrome?

Prune belly syndrome, also called Eagle-Barrett syndrome, is a condition characterized by three main features: (1) a lack of abdominal muscles, causing the skin on the belly area to wrinkle and appear "prune-like";  (2) undescended testicles in males; and (3) urinary tract problems. The incidence of prune belly syndrome (PBS) is 1 in 40,000 births; 95% of cases occur in boys. The severity of symptoms in infants with prune belly syndrome can vary greatly from child to child.  At one end of the spectrum, the condition may cause severe urogenital and pulmonary problems incompatible with life (resulting in stillbirth); at the other end of the spectrum, the condition may cause few, if any, urological abnormalities that require no treatment other than  undescended testicle repair in males. The cause of the condition is unknown.[1][2]
Last updated: 5/18/2009

What causes prune belly syndrome?

The underlying cause of prune belly syndrome is unknown. The condition may occur if there is a blockage preventing the flow of urine through the urinary tract. The blockage can cause the urine to flow back into the bladder, enlarging it.[3]
Last updated: 12/8/2008

What are the symptoms of prune belly syndrome?

The severity of symptoms in infants with prune belly syndrome can vary greatly from child to child. Common symptoms are poorly developed abdominal muscles, undescended testicles in males, and urinary tract problems such as swelling of the kidney, abnormally developed kidneys, and enlarged ureters, bladder, and urethra. Prune belly syndrome may also cause lung, heart, gastrointestinal, and other organ, bone, and muscle damage.[2]
Last updated: 12/8/2008

How might prune belly syndrome be treated?

The initial evaluation of the newborn with prune belly syndrome requires a team consisting of a neonatologist, nephrologist, urologist and in some cases other specialists (e.g., cardiologist) as well.[2] Treatment options depend on the child's age, health, medical history, extend of disease, tolerance for certain treatments or procedures, the expected course of the disease, and the parent's and/or guardian's opinions and preferences.[3]

In general, surgery may be done to repair abdominal muscle, genital, and bladder problems.[4][2]  Antibiotics may be given to infants to treat or prevent urinary tract infections.[4] Timing of therapy may vary from patient to patient. To learn more about your child’s specific treatment options we recommend that you speak to her healthcare provider.
Last updated: 12/8/2008

What is the prognosis (chance of recovery) for children with prune belly syndrome?

One of the most important factors in determining the prognosis of a child with prune belly syndrome is the severity of the urinary tract anomaly, in particular how well the kidneys are developed. The condition can become life threatening in severely affected children.[2]

You can find more information on the prognosis in adults with prune belly syndrome at the following eMedicine Website. You may need to register to view the article but registration is free.
http://www.emedicine.com/med/TOPIC3055.HTM#section~OutcomeandPrognosis
Last updated: 12/8/2008

References