HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Grunnet, M. L. Articles by Bale, J. F. Search for Related Content PubMed PubMed Citation Articles by Grunnet, M. L. Articles by Bale, J. F., Jr.

Brain abnormalities in infants with Potter syndrome (oligohydramnios tetrad)

Departments of Neurology (Drs. Grunnet and Bale), Pathology (Dr. Grunneti, and Pediatrics (Dr. Bale), University of Utah College of Medicine, Salt Lake City, UT.

Address correspondence and reprint requests to Dr. Grunnet, Department of Pathology, University of Connecticut Health Center, Farmington, CT 06032.

We examined the brains of seven unrelated infants with Potter syndrome (oligohydramnios tetrad), a lethal neonatal disorder characterized by abnormal facies, lung hypoplasia, limb deformities, and classically, renal agenesis. All infants had defects of neuronal migration. The brains were small for gestational age in five of seven infants, and in four infants, the middle and inferior temporal gyri were incompletely demarcated. Cerebellar heterotopia occurred in five infants. All had abnormal hippocampi and abnormal lamination of the cerebral cortex. These neuropathologic abnormalities suggest that Potter syndrome may not be the result of a single toxic or infectious insult but may represent a polygenic inherited disorder.