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November 1963

Neonatal Polycystic Kidney With Brain Defect

Author Affiliations

Mayo Clinic, Rochester, Minn.; Fellow in Pediatrics (Dr. Goldston), Section of Pediatrics (Dr. Burke), Fellow in Pathology (Dr. D'Agostino), and Research Associate, Section of Experimental and Pathologic Anatomy (Dr. McCaughey), Mayo Clinic and Mayo Foundation.

Am J Dis Child. 1963;106(5):484-488. doi:10.1001/archpedi.1963.02080050486016

This report concerns the occurrence of polycystic kidney and abnormalities of the brain in three siblings. Two had the Dandy-Walker syndrome (cystic dilatation of the fourth ventricle with incomplete development of the vermis). One of the infants was stillborn, and the other two survived only a few hours. The infants did not show signs of cystic disease in the other organs, as did the group of patients reported by Lundin and Olow1 and the Dalgaard2 series. The detailed neuropathology found in these infants is included in a report on the Dandy-Walker syndrome by D'Agostino, Kernohan, and Brown.3

Bilateral polycystic renal disease without cerebral malformations occurs in adults as well as infants and children. This disorder has two peaks in age distribution: the neonatal, between birth and three months4-6; and the adult, between 30 and 60 years. The adult and congenital infantile forms of the disease are