Caroli’s Disease

Caroli’s disease (CD) is a rare autosomic recessive congenital pathology that belongs to the spectrum of fibro-polycystic liver disease, a group of entities caused by an abnormal embryologic development of the ductal plate. CD is characterized by non-obstructive dilatations of the large intrahepatic bile ducts that communicate with the biliary system. It can be diffuse, segmental, or multifocal. “Caroli syndrome” defines the presence of CD and hepatic fibrosis. The diagnosis is based upon Magnetic Resonance (MR) findings. Typical symptoms include right upper quadrant abdominal pain and jaundice, related to recurrent bile stasis, with consequent stone formations and cholangitis episodes.