Potter's syndrome

This code represents Potter's syndrome, a rare and severe condition characterized by bilateral renal agenesis or severe renal hypoplasia, leading to oligohydramnios. The lack of amniotic fluid results in a cascade of secondary abnormalities, including pulmonary hypoplasia, characteristic facial features (Potter facies), and limb deformities. It is typically a fatal condition due to respiratory insufficiency.

This code is used when documentation confirms a diagnosis of Potter's syndrome, often identified prenatally via ultrasound showing absent kidneys and severe oligohydramnios, or postnatally upon examination. Supporting documentation should clearly describe the renal abnormalities and associated features consistent with the syndrome.

• Ensure documentation specifies "Potter's syndrome" rather than just bilateral renal agenesis, as the syndrome encompasses the full spectrum of associated findings.
• Consider sequencing this code as the primary diagnosis when it is the reason for admission or the focus of care.
• Do not confuse with isolated renal agenesis (Q60.0, Q60.1) if the full constellation of Potter's syndrome findings is not present.