EARLY DETECTION OF PRUNE-BELLY-SYNDROME IN-UTERO BY ULTRASONOGRAPHY

A report is presented of a male infant with prune belly syndrome (PBS) in whom bladder enlargement was detected by ultrasound (US) as early as 13 weeks of gestation. Subsequent fetal US identified progressive u rinary tract dilatation, ascites and oligohydramnios. At 2 weeks, the fetal bladder was drained under US guidance. A gradual resolution of o ligohydramnios was detected on US performed after 26 weeks of gestatio n. Delivery by cesarean section was performed at 34 weeks of gestation . The newborn had typical features of PBS with a vesico-cutaneous fist ula but did not show respiratory distress. Imaging studies showed hypo plastic left kidney, slightly dysplastic but functioning right kidney, megaureter, megacystis, vesicoureteral reflux and dilated prostatic u rethra. The early detection of genitourinary system abnormalities and serial US suggest that a distal urethral obstruction may be the underl ying mechanism of PBS. Spontaneous (or therapeutic) intra-uterine deco mpression of the bladder might ameliorate obstructive nephropathy and oligohydramnios, allowing adequate lung development.