POTTER’S SYNDROME: A STUDY OF 15 PATIENTS

Background – Potter’s syndrome is a rare congenital disorder diagnosed at birth and characterized by bilateral renal agenesis, lack of amniotic fluid, pulmonary hypoplasia, limb deformities, and typical facies. It is found in 0.2% to 0.4% of the autopsies performed on stillborn infants or those who die soon after birth. Currently, there is no way to prevent or treat it and patients have a poor prognosis with the respiratory insufficiency being the leading cause of death. To the best of our knowledge, the current report seems to be the first report of Potter’s syndrome in Iran. Methods − In this prospective report, we studied 15 patients with clinical and pathological characteristics of Potter’s syndrome. Results − Male to female ratio was 2:1. Of the studied cases, 53% had history of oligohydramnios and 60% had intrauterine growth problems. All patients had congenital renal anomalies and two-third of them had lung hypoplasia. All of the cases died, with 70% of fatalities having occurred in the first few hours of life. Conclusion − We recommend establishing the Iranian National Potter’s Syndrome Supportive Group (INPSSG) and prenatal multicenter studies in high-risk populations.