Sign Up for a Free Account
  • Updated 06.01.2020
  • Released 04.01.1994
  • Expires For CME 06.01.2023

Potter sequence


This article includes discussion of Potter sequence, oligohydramnios sequence, Potter syndrome, Potter’s sequence, Potters sequence, congenital anomalies of the kidney and urinary tract, and CAKUT. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.


Potter syndrome, also known as oligohydramnios sequence, covers a phenotypically and genetically heterogeneous group of familial or sporadic conditions that may stem from different origins. Affected newborns have characteristic facial and limb deformities and pulmonary hypoplasia resulting from oligohydramnios, primarily consequent to severe renal pathologies or occasional nonurinary mechanisms. The author highlights new developments in the treatment of oligohydramnios and reviews CNS anomalies associated with some cases of Potter syndrome.

Key points

• In classic Potter syndrome, bilateral renal agenesis and consequent anhydramnios lead to a constellation of findings characterized by congenital deformations (flattened “Potter” facies and limb deformities).

• Potter “sequence” enlarges the pathogenetic spectrum by including other renal or urinary anomalies that also lead to oligo/anhydramnios either by failure of urine production or obstruction to urine outflow.

• Oligo/anhydramnios can also stem from nonrenal causes (eg, chronic leakage of amniotic fluid or placental pathology), but a major complication arising from all causes is pulmonary hypoplasia, with subsequent death in the newborn period from respiratory insufficiency.

• Some cases are associated with gene mutations, most recently integrin alpha 8; inheritance in familial cases has been described as autosomal dominant, recessive, or multifactorial.

Historical note and terminology

Edith Potter first described a syndrome in infants characterized by bilateral renal agenesis and associated deformities, such as flattening of facies (68). Potter later extended the syndrome to include other severe cystic kidney conditions. Subsequently, the term “sequence” replaced “syndrome” because the characteristic phenotype of these infants is independent of the kidney pathology and is related to the ensuing oligohydramnios. Consequently, “oligohydramnios sequence” and “Potter sequence” are used interchangeably (16; 53).

Involvement of the CNS in oligohydramnios sequence has sometimes been mentioned in the literature; however, only exceptionally has a detailed neuropathologic study of these defects been conducted (43).

This is an article preview.
Start a Free Account
to access the full version.

  • Nearly 3,000 illustrations, including video clips of neurologic disorders.

  • Every article is reviewed by our esteemed Editorial Board for accuracy and currency.

  • Full spectrum of neurology in 1,200 comprehensive articles.

  • Listen to MedLink on the go with Audio versions of each article.

Questions or Comment?

MedLink®, LLC

3525 Del Mar Heights Rd, Ste 304
San Diego, CA 92130-2122

Toll Free (U.S. + Canada): 800-452-2400

US Number: +1-619-640-4660



ISSN: 2831-9125