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Potter syndrome

Definition

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.

Alternative Names

Potter phenotype

Causes

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.

Symptoms

Symptoms include:

Exams and Tests

A pregnancy ultrasound may show lack of amniotic fluid, absence of fetal kidneys, or severely abnormal kidneys in the unborn baby.

The following tests may be used to help diagnose the condition in a newborn:

Treatment

Resuscitation at delivery may be attempted pending the diagnosis. Treatment will be provided for any urinary outlet obstruction.

Outlook (Prognosis)

This is a very serious condition. Most of the time it is deadly. The short-term outcome depends on the severity of lung involvement. Long-term outcome depends on the severity of kidney involvement.

Prevention

There is no known prevention.

Gallery

Amniocentesis
Amniocentesis is used to determine the health of an unborn baby. Amniotic fluid contains cells that are normally shed from the fetus. Samples of these cells are obtained by withdrawing some amniotic fluid. The chromosome analysis of these cells can be performed to determine abnormalities. In addition, the cells may be cultured and analyzed for enzymes, or for other materials that may indicate genetically transmitted diseases. Other studies can be done directly on the amniotic fluid including measurement of alpha-fetoprotein.
The face
The physical landmarks of the human face are very similar from one face to another.

References

Joyce E, Ellis D, Miyashita Y. Nephrology. In: Zitelli BJ, McIntire SC, Nowalk AJ, eds. Zitelli and Davis' Atlas of Pediatric Physical Diagnosis. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 14.

Marcdante KJ, Kliegman RM. Congenital and developmental abnormalities of the urinary tract. In: Marcdante KJ, Kliegman RM, eds. Nelson Essentials of Pediatrics. 8th ed. Philadelphia, PA: Elsevier; 2019:chap 168.

Mitchell AL. Congenital anomalies. In: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 30.

Last reviewed August 10, 2021 by Neil K. Kaneshiro, MD, MHA, Clinical Professor of Pediatrics, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team..

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