Caroli's disease and adult polycystic kidney disease: a rarely recognized association

Authors

  • Deanna Jordon,

    1. The Lillian and Henry M. Stratton-Hans Popper Department of Pathology, Mount Sinai School of Medicine of the City University of New York, New York, USA
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  • Noam Harpaz,

    1. The Lillian and Henry M. Stratton-Hans Popper Department of Pathology, Mount Sinai School of Medicine of the City University of New York, New York, USA
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  • Swan N. Thung MD

    Corresponding author
    1. The Lillian and Henry M. Stratton-Hans Popper Department of Pathology, Mount Sinai School of Medicine of the City University of New York, New York, USA
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Department of Pathology Mount Sinai School of Medicine One Gustave L. Levy Place New York, NY 10029 USA

Abstract

ABSTRACT— Caroli's disease is a rare form of fibropolycystic disease of the hepatobiliary system characterized by segmental cystic dilatation of intrahepatic ducts. It is associated with intrahepatic cholelithiasis, cholangitis and hepatic abscesses. Like other forms of fibropolycystic disease of the liver, Caroli's disease is often accompanied by cystic renal disease, specifically renal tubular ectasia or medullary sponge kidney. Adult-type polycystic kidney disease associated with Caroli's disease is rare, only one such case having appeared in the literature to our knowledge. We present a second instance of this association.

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