Potter syndrome

Potter syndrome

Definition:

Potter syndrome and Potter phenotype is a complex of findings associated with renal failure that develops before an infant is born, and with a lack of amniotic fluid (oligohydramnios).

Causes, incidence, and risk factors:

Potter phenotype describes a typical appearance that, in the newborn, is determined by the absence of amniotic fluid (oligohydramnios). In the absence of amniotic fluid the infant is not cushioned from the walls of the uterus. The pressure of the uterine wall causes a typical facial appearance (Potter’s facies): widely separated eyes with epicanthal folds, broad nasal bridge, low set ears and receding chin. In addition, because of limited intrauterine space, the limbs may be abnormal or held in abnormal positions or contractures. Oligohydramnios also stops development of the lungs (hypoplastic lungs) so that at birth the lungs do not function properly.

In Potter syndrome the primary defect is renal failure that occurs before the baby is born, either from failure of the kidneys to develop (bilateral renal agenesis) or from other diseases of the kidney which cause them to fail. The kidneys normally produce the amniotic fluid (as urine) and it is the absence of amniotic fluid that causes the typical appearance of Potter syndrome.

Symptoms:

Potter facies (described above)
absence of urine output
respiratory distress

Signs and tests:

stiff lungs during resuscitation effort (require high pressure for ventilation)
Potter phenotype
oligohydramnios
urogenital abnormalities

Tests:

fetal ultrasound (mother) demonstrating:
- oligohydramnios
- absence of fetal kidneys or severely abnormal kidneys
X-ray of abdomen (newborn)
X-ray of the lung(s) (newborn)
blood gases

Treatment:

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

Expectations (prognosis):

Bilateral renal agenesis and hypoplastic lungs are incompatible with life.

Complications:

This is a fatal condition.

Calling your health care provider:

This condition is discovered at the time of birth.

Prevention:

There is no known prevention.

Review Date: 12/10/2001
Reviewed By: Jonathan Fanaroff, M.D., Department of Pediatrics, Rainbow Babies and Children's Hospital, Case Western Reserve University, Cleveland, OH. Review provided by VeriMed Healthcare Network.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information and services. A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician 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. Copyright 2003 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.