Dysplasia of the kidneys, liver, and pancreas: report of a variant of Ivemark's syndrome.

A newborn girl with respiratory distress due to bilateral pneumothorax was found to be anuric, and died at 18 hours of age. Autopsy revealed a large pancreatic cyst, multiple large hepatic cysts, congenital hepatic fibrosis, bilateral dysplastic kidneys, and dysplasia of the pancreas. These findings constitute a variant of Ivemark's syndrome of dysplasia of the pancreas, liver, and kidneys.