The complex nature of this syndrome has led to considerable speculation about its etioloqy and pathogenesis, including primary obstructive urinary anomalies, defective mesodermal development, intrauterine viral illness, and genetic factors. Although a genetic basis has not been established, cases have been documented in siblings and twin gestations. There is a wide spectrum of severity with this syndrome.
Survival is related to renal and pulmonary function.
If complete urinary obstruction exists leading to severe bilateral renal dysplasia, oligohydramnios, and pulmonary hypoplasia, then prognosis is dismal. Based on fetal sonographic findings consistent with distal urinary tract obstruction. In selected cases, in utero decompression and urinary diversion may allow sufficient renal and pulmonary development to permit the fetus to survive after birth. The classical triad of urinary tract anomalies, deficient abdominal musculature, and bilateral cryptorchidism describes the Prune-Belly syndrome. In addition to this classic triad, a broad spectrum of associated defects including musculoskeletal, cardiovascular, pulmonary, and genital malformations have been documented 1-2 .