Meta Description: Caroli’s disease was specialized by progressive cystic dilatation of intrahepatic bile ducts and belongs to an entity of fibropolycystic liver disease. A proportion of the patients maintain renal function into adulthood, where they typically have less severe kidney disease, and complications of the liver disease become predominant.
Caroli’s disease was specialized by progressive cystic dilatation of intrahepatic bile ducts and belongs to an entity of fibropolycystic liver disease. It is generally accompanied by congenital hepatic fibrosis and is also known as hepatic manifestation of autosomal recessive polycystic kidney disease. A significant proportion of cases die in the neonatal period, primarily by respiratory insufficiency.
A proportion of the patients maintain renal function into adulthood, where they typically have less severe kidney disease, and complications of the liver disease become predominant. These complications include those associated with portal hypertension related to congenital hepatic fibrosis, and cholangitis related to Caroli’s disease. Treatment for Caroli’s disease with congenital hepatic fibrosis is largely supportive, and is directed toward treating these complications. Caroli’s disease is characterized by progressive cystic dilatation of the intrahepatic bile ducts, and belongs to an entity of fibropolycystic liver disease.