Potter Syndrome: Bilateral Renal Agenesis

Definition of Potter's Syndrome

Bilateral renal agenesis (Potter's syndrome) is a rare and always fatal malformation with bilateral absent kidneys (Potter, 1965).

Epidemiology of Potter's syndrome:

Incidence: 3–15/100.000 births for bilateral renal agenesis, 3–20/100.000 births for bilateral renal dysgenesia. In 75%, boys are affected.

Etiology of Potter's Syndrome:

Recessive inheritance is likely, siblings of children with bilateral renal agenesis are also affected in 3.5%. The absence of a ureteric bud or the absence of metanephric blastema results in the complete absence of renal tissue.

The oligohydramnios and/or the lack proline production of the kidneys leads to pulmonary hypoplasia, respiratory insufficiency and Potter facies of the children.