Diagnosis of Oligohydramnios-Related Pulmonary Hypoplasia (Potter Syndrome): Value of Portable Voiding Cystourethrography in Newborns with Respiratory Distress

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Abstract
Potter renal nonfunction syndrome is an association of facial and limb anomalies, pulmonary hypoplasia, and fetal renal anomalies which lead to marked oligohydramnios, including renal agenesis (true Potter syndrome), renal cystic dysplasia and obstructive uropathies. Some infants survive long enough to develop severe respiratory distress secondary to pulmonary hypoplasia. The underlying renal disease is often noted only at autopsy. Four infants, only 1 of whom had clinical signs of the renal nonfunction syndrome, were studied. Portable voiding cystourethrography revealed a tiny bladder in 3 infants with cystic dysplastic kidneys (2 of these infants had reflux into unused ureters). Bladder hypertrophy and vesicoureteral reflux secondary to posterior urethral valves were noted in the 4th infant.

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