Toggle: English / Spanish
Potter syndrome and Potter phenotype refers to a group of findings associated with a lack of
and in an unborn infant.
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.
- Widely separated eyes with , , , and
- Absence of urine output
- Difficulty breathing
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:
Resuscitation at delivery may be attempted pending the diagnosis. Treatment will be provided for any urinary outlet obstruction.
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.
There is no known prevention.
Copelovitch L, Kaplan BS. Developmental abnormalities of the kidneys. In: Gleason CA, Devaskar SU eds. Avery's diseases of the newborn. 9th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 83.
Elder JS. Congenital anomalies and dysgenesis of the kidneys. In: Kliegman RM, Stanton BF, St Geme JW III, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 537.
- Last reviewed on 7/10/2015
- Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
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. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- 2013 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.