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| Definition: | A disease characterized by three major findings (triad): deficient abdominal muscles (causing the skin of the abdomen to wrinkle like a prune), undescended testicles, and a dilated abnormal urinary tract.
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| Alternative Names: | Eagle-Barrett syndrome
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| Causes, incidence, and risk factors: | The underlying causes of prune belly syndrome are unknown. There is blockage to the flow of urine from the kidney to the outside of the body during fetal life. For example, dilatation of the urinary tract may develop as a result of urethral obstruction in the womb. The incidence is approximately 1 in 30,000 to 40,000 births. Boys are almost solely affected.
Expectant mothers carrying affected infants may develop varying degrees of oligohydramnios (insufficient amniotic fluid) that predisposes the infant to lung problems (see Potter syndrome). The newborn infant has a wrinkled abdomen (it looks like a prune). This appearance results from swelling with fluid in the womb then loss of that fluid after birth leading to wrinkles of excess skin. The appearance is accentuated by the lack of adequate abdominal musculature. Genital abnormalities may be prominent as undescended testes. Approximately one third of the infants are either stillborn or die within the first few weeks of life from severe lung, kidney, or combined problems.
Other abnormalities may be present including musculoskeletal defects, heart abnormalities, and a rotated bowel.
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Review Date: 12/3/2001
Reviewed By: David G. Brooks, M.D., Ph.D., Division of Medical Genetics, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
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