It is a congenital abnormality of unknown aetiology with 3 characteristic features: Deficient development of abdominal muscles that causes the skin of the abdomen to wrinkle like a prune Cryptorchism Abnormalities of the urinary tract, usually hydronephrosis and vesico-ureteric reflux There may also be pulmonary hypoplasia.
Prune Belly Syndrome is a serious and often life threatening problem. Miscarriage, stillbirth and neonatal death are common. Multiple concomitant anomalies and bilateral renal lesions are the cause of fetal or infant death in 75% of cases. 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. Many die in the first two years of life. Bilateral abnormal kidneys on ultrasound or renal scan, a nadir serum creatinine above 62μmol/l and pyelonephritis are prognostic for renal failure.