Liver 1989 Feb;9(1):30-5

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

Jordon D, Harpaz N, Thung SN
Lillian and Henry M. Stratton-Hans Popper Department of Pathology, Mount Sinai
School of Medicine, City University of New York, New York.

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