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Potter syndrome

Show Alternative Names
Potter phenotype
Potter's 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

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) that surrounds the unborn baby.

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.

Review Date: 8/5/2023

Reviewed By

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

References

Miyashita Y, Formeck C, Moritz M. Nephrology. In: Zitelli BJ, McIntire SC, Nowalk AJ, Garrison J, eds. Zitelli and Davis' Atlas of Pediatric Physical Diagnosis. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 14.

Marcdante KJ, Kliegman RM, Schuh AM. Congenital and developmental abnormalities of the urinary tract. In: Marcdante KJ, Kliegman RM, Schuh AM, eds. Nelson Essentials of Pediatrics. 9th ed. Philadelphia, PA: Elsevier; 2023: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.

Disclaimer

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. No warranty of any kind, either expressed or implied, is made as to the accuracy, reliability, timeliness, or correctness of any translations made by a third-party service of the information provided herein into any other language. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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Amniotic fluid - Illustration Thumbnail

Amniotic fluid

Amniotic fluid not only protects the fetus from injury and temperature changes, it also is circulated by the fetus every 3 hours.

Illustration

Broad nasal bridge - Illustration Thumbnail

Broad nasal bridge

Broad nasal bridge, or widening of the base of the nose, is a relative term. It can be a normal facial feature, but it can also be associated with certain congenital disorders such as basal cell nevus syndrome, or trisomy 8, a chromosome defect.

Illustration

 
 
Amniotic fluid - Illustration Thumbnail

Amniotic fluid

Amniotic fluid not only protects the fetus from injury and temperature changes, it also is circulated by the fetus every 3 hours.

Illustration

Broad nasal bridge - Illustration Thumbnail

Broad nasal bridge

Broad nasal bridge, or widening of the base of the nose, is a relative term. It can be a normal facial feature, but it can also be associated with certain congenital disorders such as basal cell nevus syndrome, or trisomy 8, a chromosome defect.

Illustration

 
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