Prune-belly syndrome diagnosed at 14 weeks' gestation with severe urethral obstruction but normal kidneys

A fetus was diagnosed with prune-belly syndrome (PBS) found by ultrasound demonstration of cystomegaly at 12 weeks' gestation. Fetal urinary electrolyte and beta2-microglobulin concentrations at 14 weeks' gestation indicated a poor renal prognosis. The pregnancy was therefore terminated. An autopsy showed defects of the abdominal wall muscles and megalocystis caused by severe urethral obstruction. However, the kidneys were histologically normal. Together with previous reports, the present case suggests that renal dysplasia in PBS with severe uretheral obstruction may develop after 14 weeks' gestation. We discuss the possibility of preventing the development of renal dysplasia in PBS by early prenatal decompression of the obstructed fetal urinary tract.