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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, 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.

Symptoms

• Widely separated eyes with epicanthal folds, broad nasal bridge, low set ears, and receding chin
• Absence of urine output
• Difficulty breathing

Signs 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:

• X-ray of the abdomen
• X-ray of the lungs

Treatment

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

Expectations (prognosis)

This is a very serious condition, usually 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.

References

Behrman RE. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, Pa: WB Saunders; 2004.

Review Date:7/26/2007
Reviewed By:Daniel Rauch, M.D., FAAP., Director, Pediatric Hospitalist Program, New York University School of Medicine, New York, NY. Review provided by VeriMed Healthcare Network.

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