GI Endoscopy · 1 min read

Focal Segmental Bile Duct Dilation

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

ByKlaus Mönkemüller, MD, PhD, FASGE, FJGES, FESGE

Experienced teaching points

Clinical Pearls

Caroli's disease is a rare congenital malformation characterized by focal, segmental cystic dilation of the intrahepatic bile ducts without mechanical obstruction.
When focal segmental bile duct dilation is accompanied by congenital hepatic fibrosis, the condition is formally classified as Caroli's syndrome.
The differential diagnosis for segmental non-obstructive bile duct dilation includes sclerosing cholangitis, hepatolithiasis, biliary papillomatosis, choledochal cysts, and parasitic infections like liver flukes.

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.

About the author

Klaus Mönkemüller, MD, PhD, FASGE, FJGES, FESGE

Editor-in-Chief, The Practicing Endoscopist

Professor of Medicine, Carilion Memorial Hospital / Virginia Tech Carilion School of Medicine, Roanoke, Virginia, USA

Klaus Mönkemüller, MD, PhD, FASGE, FJGES, FESGE, is the editor-in-chief of The Practicing Endoscopist and the founder of EndoCollab. He is Professor of Medicine at Virginia Tech Carilion School of Medicine and a practicing endoscopist at Carilion Memorial Hospital in Roanoke, Virginia.

Dr. Mönkemüller has published extensively on endoscopic techniques and devices, with a particular focus on therapeutic endoscopy, foreign body removal, GI bleeding, and the use of caps and accessories in everyday practice. He lectures internationally and has contributed to multiple GI endoscopy textbooks and atlases.

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