Potter Syndrome

Potter syndrome is a fatal congenital disorder characterized by the changes in physical appearances of neonate due to oligohydramnios caused by renal agenesis and impairment. It is incompatible with life as neonates with Potter syndrome have pulmonary hypoplasia that leads to respiratory distress within an hour of birth. Potter sequence and Potter syndrome are used interchangeably because the sequence of events leading to oligohydramnios is consistent. But the Potter sequence more specifically describes the decreased amniotic fluid irrespective of the cause. The frequency of Potter syndrome in multiple pregnancies is very rare. Discordance of dichorionic and diamniotic twins may be found with one twin showing extrarenal manifestation along with renal agenesis and another twin with normal pulmonary function. Potter syndrome is classified on the basis of the cause of renal anomalies. Bilateral renal agenesis is classic to Potter syndrome. Subtype I is associated with autosomal recessive polycystic kidney, subtype II is due to renal dysplasia, subtype III is due to autosomal dominant polycystic kidney, and subtype IV is related with obstruction of ureter or pelvis causing hydronephrosis.