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Prune belly syndrome
Alternative namesEagle-Barrett syndrome
Prune belly syndrome is a group of congenital anomalies characterized by three major findings:
Causes, incidence, and risk factors
The underlying causes of prune belly syndrome are unknown. 96-97% of cases are boys. Expectant mothers carrying affected infants may develop varying degrees of oligohydramnios (insufficient amniotic fluid ) that make it likely the infant will have lung problems. (See also Potter syndrome .)
The newborn infant has a wrinkled abdomen that looks like a prune. This is because the abdomen swelled with fluid in the womb, then lost that fluid after birth, leading to wrinkles of excess skin. The appearance is accentuated by the lack of adequate abdominal musculature. Many infants are either stillborn or die within the first few weeks of life from severe lung or kidney problems, or a combination of congenital anomalies.
Signs and tests
Prune Belly Syndrome is a serious and often life threatening problem. Many newborns survive with varying degree of chronic problems, but others are stillborn or die in the first two years of life.
Complications depend on the associated abnormalities. The most common are chronic renal failure and club foot.
Calling your health care provider
Prune Belly Syndrome is usually diagnosed before birth or at the time of birth.
If you have a child with diagnosed prune belly syndrome, call your health care provider at the first sign of a urinary tract infection or other urinary symptoms. If your child is diagnosed with distended bladder or enlarged kidneys on prenatal ultrasound, seek prompt consultation with a specialist in high-risk obstetrics or a perinatologist.
PreventionThere is no known guaranteed prevention. If a prenatal diagnosis of urinary tract obstruction is made, it may be possible to prevent it from progressing to prune belly syndrome with prenatal surgery.
Update Date: 11/3/2003Celeste Krauss, M.D., Board Certified Clinical Genetics, Clinical Cytogenetics and Pediatrics. Founding Fellow, American College of Medical Genetics. Review provided by VeriMed Healthcare Network.
Last updated: Tue, 06 Jan 2009 00:20:03 GMT