Caroli disease (CD) is a rare congenital hepatobiliary disorder characterized by multifocal segmental dilatation of the intrahepatic bile ducts, which may involve the entire liver or only a specific region. This rare pathology is seen in a very small proportion of the global population. Clinical manifestations vary between individuals and may overlap with other hepatobiliary disorders, making diagnosis and management challenging. Due to limited understanding and experience, early diagnosis and intervention are crucial for improving survival. In our case, Caroli disease is associated with autosomal recessive polycystic kidney disease (ARPKD), both of which are autosomal recessive disorders most commonly found in infants and children, with survival into adulthood being rare.
Aien et al. (Sat,) studied this question.