Curriculum · Pediatric / Nutrition & Growth / EPI / Routine Child Examination
Potter syndrome
What it is
Potter syndrome is a chain that starts in the kidney and ends in the lung. Bilateral renal agenesis or bilateral multicystic dysplastic kidneys give reduced fetal urine excretion; reduced urine gives oligohydramnios; and oligohydramnios causes fetal compression. Everything seen at birth follows from that compression, which is why the same picture is called the Potter sequence.
Causes and risk
Anything that cuts fetal urine can end here. Besides bilateral renal agenesis and bilateral multicystic dysplastic kidneys, autosomal recessive polycystic kidney disease is a cause: significant oliguric renal failure in utero can lead to the Potter sequence. In ARPKD there are bilateral enlarged echogenic kidneys detected on prenatal or postnatal ultrasound, ± oligohydramnios, pulmonary hypoplasia, or respiratory distress after birth, so a neonate with respiratory distress and enlarged kidneys on imaging should raise it.