Potter syndrome

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The Potter sequence is a constellation of findings demonstrated postnatally as a consequence of severe, prolonged oligohydramnios in utero.

Clinical presentation

It consists of:

pulmonary hypoplasia: often severe and incompatible with life
growth restriction (IUGR)
abnormal facies (Potter facies)
- low set ears
- flattened nose
- wrinkled skin
- micrognathia
limb abnormalities: including club feet and contractures

These common clinical features can be easily recalled using the mnemonic POTTER.

Pathology

Although originally associated with renal causes of olighydramnios, the Potter sequence may be seen with severe oligohydramnios of essentially any cause.

Common etiologies include:

fetal renal insufficiency
- bilateral renal agenesis: traditionally known as the classic Potter syndrome
- autosomal recessive polycystic kidney disease
- posterior urethral valves, and other causes of antenatal renal tract obstruction
premature rupture of membranes (PROM)
twin-twin transfusion

Treatment and prognosis

The prognosis is poor with a very high mortality rate, primarily secondary to pulmonary hypoplasia.

History and etymology

It is named after Edith Louise Potter (1901-1993), an American pathologist who described the syndrome specifically in association with bilateral renal agenesis .