Early fetal cystoscopy for first-trimester severe megacystis.

[megacystis-microcolon-intestinal hypoperistalsis syndrome]

To report the feasibility of early fetal cystoscopy for the prenatal diagnosis and therapy of severe first -trimester megacystis .Between January 2008 and February 2010 , early fetal cystoscopy at 16 weeks of gestation was offered to 15 patients whose fetuses presented with severe first -trimester megacystis . All infants were followed up for 6 -12 months after birth . Autopsy was always performed whenever fetal or neonatal deaths occurred .Seven patients decided to undergo fetal therapy , and eight elected to continue with expectant observation . One fetus died before early fetal cystoscopy was performed . Therefore , six fetuses underwent early fetal cystoscopy . Urethral atresia was diagnosed in three fetuses during fetal cystoscopy and confirmed at autopsy following termination of pregnancy at 19 -20 weeks in all cases . Posterior urethral valves were diagnosed and successfully fulgurated by laser during early cystoscopy in three fetuses , two of which survived with normal renal and bladder function after birth ; the remaining fetus had a postnatal diagnosis of megacystis -microcolon intestinal hypoperistalsis syndrome and died neonatally . In the expectantly managed group , no survivals were observed , even among cases with 'isolated ' posterior urethral valves .Percutaneous early fetal cystoscopy is feasible for prenatal diagnosis and therapy of severe megacystis .