Preferred Label : Caroli disease;
ICD-11 definition : Caroli's disease is a rare congenital disorder characterized by multifocal, segmental
dilatation of the large intrahepatic bile ducts. It presents with recurrent bacterial
cholangitis, biliary stones causing biliary pain or episodes of pancreatitis. The
more common variant of this disease, named Caroli's syndrome, is characterized by
dilatations of the large bile duct associated with congenital hepatic fibrosis, with
signs and symptoms of portal hypertension or bacterial cholangitis.;
ICD-11 synonym : Cystic dilatation of intrahepatic bile ducts;
Origin ID : 1757434239;
Currated CISMeF NLP mapping
Caroli's disease is a rare congenital disorder characterized by multifocal, segmental
dilatation of the large intrahepatic bile ducts. It presents with recurrent bacterial
cholangitis, biliary stones causing biliary pain or episodes of pancreatitis. The
more common variant of this disease, named Caroli's syndrome, is characterized by
dilatations of the large bile duct associated with congenital hepatic fibrosis, with
signs and symptoms of portal hypertension or bacterial cholangitis.
