Choroba czy zespół Caroliego? Od zaburzeń embriogenezy do przeszczepienia wÄ troby

Caroli's disease is a rare congenital disease of the liver characterized by multiple segmental cystic dilatation of intrahepatic bile ducts. The disease is referred as Caroli's syndrome when it is associated with congenital hepatic fibrosis. The clinical features include jaundice, right upper abdominal pain, and fever due to the associated complications of bacterial cholangitis. Owing to the rarity of this disorder, the diagnosis and treatment is often delayed.