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Definition Return to top
Potter syndrome and Potter phenotype is a complex of findings associated with kidney failure that develops before an infant is born and with a lack of amniotic fluid.Causes, incidence, and risk factors Return to top
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) that includes widely separated eyes with epicanthal folds, broad nasal bridge, low set ears and receding chin.
In addition, because of limited space in the uterus, 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 kidney failure that occurs before the baby is born, either from failure of the kidneys to develop (bilateral renal agenesis) or from other diseases which cause the kidneys 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 Return to top
Signs and tests Return to top
Signs include:Treatment Return to top
Resuscitation at delivery may be attempted pending the diagnosis. Treatment will be provided for any urinary outlet obstruction.Expectations (prognosis) Return to top
This is a fatal condition. Bilateral renal agenesis and hypoplastic lungs are incompatible with life.Prevention Return to top
There is no known prevention. Update Date: 10/30/2003 Updated by: Philip L. Graham III, M.D., M.S., F.A.A.P., Department of Pediatrics, Children's Hospital of New York, Columbia University, New York, NY. Review provided by VeriMed Healthcare Network.
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Page last updated: 28 October 2004 |