Prognosis of posterior urethral valves presenting at birth

Urethral obstruction due to posterior urethral valves (PUV) is a more lethal condition in the newborn than is commonly recognized. To study its prognosis when the diagnosis of PUV is made shortly after birth, cases of PUV presenting in the first week of life were reviewed. Eleven cases were seen at the University of California, San Francisco from 1974 to 1982. Five babies died, three within two hours of birth of respiratory insufficiency secondary to severe pulmonary hypoplasia, and two within three weeks with rapidly progressive renal failure. Of the six survivors, four exhibited respiratory difficulty (prolonged ventilatory support required in two, and bilateral pneumothoraces in two), three developed renal failure despite early urinary diversion, and one had renal tubular dysfunction. Only two infants had normal renal function after undergoing a successful series of reconstructions. Prune-belly syndrome and limb deformities were present in two surviving and two fatal cases. Although most cases of congenital hydronephrosis can be successfully treated after birth, those presenting in the newborn period represent a subset with high morbidity and mortality. With advances in prenatal diagnosis of congenital hydronephrosis, salvage of severe cases detected early in gestation may require more aggressive management in the prenatal period.