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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Potter syndrome


Other Names for this Disease
  • Oligohydramnios sequence
  • Potter sequence
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Cause


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What causes Potter syndrome?

A sequence of events causes the features of Potter syndrome. After approximately 16 weeks gestation, the amount of amniotic fluid present mainly depends on fetal urine production. In normal fetal development, the fetus continuously swallows amniotic fluid, which then gets reabsorbed by the GI tract and then reintroduced into the amniotic sac by the kidneys. Oligohydramnios (too little of amniotic fluid) occurs if the volume of amniotic fluid is less than normal for that period of gestation. This may be due to decreased urine production caused by bilateral renal agenesis, obstruction of the urinary tract, or, occasionally, prolonged rupture of membranes. The resulting oligohydramnios is the cause of the abnormalities seen in Potter syndrome. The mechanism of lung hypoplasia (underdevelopment) in this condition is not clear, but it is believed that adequate space for fetal movement and the movement of amniotic fluid into the fetal lungs is required for the normal development of lungs.[1]

In one study that looked at 80 cases of Potter syndrome, the authors stated that the abnormal renal findings leading to oligohydramnios were bilateral renal agenesis in 21.25%; cystic dysplasia in 47.5%; obstructive uropathy in 25%; and others in 5.25%.[2]
Last updated: 1/14/2011

References
  1. Sushil Gupta and Carlos E Araya. Potter syndrome. eMedicine. June 30, 2010; http://emedicine.medscape.com/article/983477-overview. Accessed 5/31/2011.
  2. Curry CJ, Jensen K, Holland J, Miller L, Hall BD. The Potter sequence: a clinical analysis of 80 cases. American Journal of Medical Genetics. December 1984; 19(4):679-702. http://www.ncbi.nlm.nih.gov/pubmed/6393764. Accessed 1/14/2011.