Curbside Consultation of the Pancreas: 49 Clinical Questions

Scott Tenner, MD, MPH; Alphonso Brown, MD, MS Clin Epi; Frank Gress, MD

Question 43: Caroli’S Disease, What Do I Need To Know? (continued)

Mustafa A. Tiewala, MD; Frank G. Gress, MD

Cystic lesions of the hepatobiliary system are being increasingly diagnosed. These fibropolycystic diseases involve multiple organs. They are usually inherited and occur in various combinations, consisting of polycystic liver disease, microhamartoma, congenital hepatic fibrosis, Caroli’s disease, and choledochal cysts. Caroli’s disease, originally described by Jacques Caroli in 1958, is a congenital disorder characterized by multiple segmental or saccular dilatations of the large intrahepatic bile ducts. Caroli’s syndrome is a congenital disorder characterized by multiple…