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Congenital hepatic fibrosis with saccular dilatation of intrahepatic bile ducts and infantile polycystic kidneys
Authors:C. H. Davies  D. A. Stringer  H. Whyte  A. Daneman  K. Mancer
Affiliation:(1) Department of Radiology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada;(2) Department of Paediatrics (Division of Neonatology), The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada;(3) Department of Pathology, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada
Abstract:A case report is presented of a child who died in the neonatal period and who had the sonographic findings of multiple, tubular, fluid-filled spaces in the liver characteristic of Caroli's disease. There was an echodense renal cortex and pyramids. Necropsy findings were consistent with Caroli's disease but the liver also showed evidence of congenital hepatic fibrosis and there was infantile type polycystic disease of the kidneys.
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