ABSTRACT

The primary life-threatening congenital urologic anomalies include posterior urethral valves, urethral atresia, and prune belly syndrome, which usually are characterized by bilateral hydroureteronephrosis and a distended bladder that does not empty. Approximately 40% of infants with urethral valves develop end-stage renal disease or chronic renal insufficiency.21,22 Although prune belly syndrome is considered non-obstructive, neonates with this condition frequently have renal insufficiency.23 Urethral atresia is nearly always fatal, because the kidneys are dysplastic. A severe adverse prognostic factor is oligohydramnios, which prevents normal pulmonary development. In fetuses with severe obstructive uropathy and renal dysplasia, neonatal demise usually results from pulmonary hypoplasia rather than chronic renal failure.