Case Report: Pseudo-prune belly syndrome after early fetal bladder decompression: implications for the pathogenesis of prune belly spectrum disorders.

Background: Prune Belly Syndrome (PBS) is a rare congenital disorder characterized by abdominal wall deficiency, urinary tract dilatation, and bilateral cryptorchidism. Its pathogenesis remains controversial, with both developmental and mechanical theories proposed. Whether early fetal decompression influences the phenotypic expression of PBS has not been established. Case presentation: We report a male fetus diagnosed at 12 weeks' gestation with severe megacystis and bilateral hydroureteronephrosis. A Somatex Conclusion: This case illustrates a favorable early outcome following prenatal vesicoamniotic shunting in a fetus with severe lower urinary tract obstruction who subsequently developed an attenuated phenotype consistent with PPBS. Although causality cannot be inferred from a single observation, these findings are consistent with the fetal outlet obstruction hypothesis and raise the possibility that early fetal decompression may influence phenotypic expression in selected patients. Further studies are required to clarify the role of prenatal intervention in the pathogenesis and clinical spectrum of PBS.