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Potter syndrome - Overview

Alternative Names

Potter phenotype

Definition of Potter syndrome:

Potter syndrome and Potter phenotype refers to a group of findings associated with a lack of amniotic fluid and kidney failure in an unborn infant.

Causes, incidence, and risk factors:

In Potter syndrome, the primary problem is kidney failure. The kidneys fail to develop properly as the baby is growing in the womb. The kidneys normally produce the amniotic fluid (as urine).

Potter phenotype refers to a typical facial appearance that occurs in a newborn when there is no amniotic fluid. The lack of amniotic fluid is called oligohydramnios. Without amniotic fluid, the infant is not cushioned from the walls of the uterus. The pressure of the uterine wall leads to an unusual facial appearance, including widely separated eyes .

Potter phenotype may also lead to abnormal limbs, or limbs that are held in abnormal positions or contractures.

Oligohydramnios also stops development of the lungs, so the lungs do not work properly at birth.

Amniotic fluid

Broad nasal bridge

Reviewed last on: 8/2/2009
Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

Elder JS. Congenital anomalies and dysgenesis of the kidneys. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 537.

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