Focal Segmental Bile Duct Dilation

Caroli's disease: Causes and features of rare biliary tract malformation

By Klaus Mönkemüller, MD, PhD, FASGE, FJGES 

Professor of Medicine, Virginia Tech Carilion School of Medicine, Virginia, USA 

A close-up of a scan of a human body Description automatically generated

Few conditions can result in segmental (focal) non-obstructive bile duct dilation. These include primary or secondary sclerosing cholangitis, hepatolithiasis, parasitic infections (e.g. Clonorchis sinensis or oriental cholangiohepatitis, Opistorchis viverrini, Fasciola hepatitis or liver fluke), biliary papillomatosis, choledochal cysts or, as in this case, Caroli’s disease and syndrome. Caroli's disease is a rare congenital disease of the liver generally characterized by segmental cystic dilation of the intrahepatic bile ducts. Classic Caroli's disease involves malformations of the biliary tract alone, whereas Caroli's syndrome refers to the presence of associated congenital hepatic fibrosis.