Caroli disease is a rare, congenital condition characterized by segmental dilation of large, intrahepatic bile ducts, which appear as cysts on imaging studies and histopathological examination. This condition is included in a group of congenital disorders known as fibropolycystic liver diseases, which originate from ductal plate malformations during embryological development. Patients with Caroli disease may frequently experience complications such as hepatolithiasis and recurrent cholangitis. Additionally, the patients are at increased risk for cholangiocarcinoma due to bile stasis and chronic inflammation. When Caroli disease is associated with congenital hepatic fibrosis, it is referred to as Caroli syndrome. Unlike Caroli disease alone, Caroli syndrome is characterized by sequela of hepatic fibrosis and portal hypertension. Recognizing Caroli disease and related conditions is crucial for effectively managing and preventing long-term complications. In Caroli disease, laboratory findings are often nonspecific, making imaging modalities such as ultrasonography, computed tomography, and magnetic resonance cholangiopancreatography essential for evaluation. Treatment is tailored to the clinical presentation and primarily involves supportive care. This activity reviews the pathophysiology, diagnosis, and treatment strategies for Caroli disease, emphasizing the condition’s diverse phenotypic spectrum. This activity also highlights the importance of collaboration within an interprofessional healthcare team to actively engage patients, improve clinical outcomes, and prevent long-term complications.
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FDA REMS
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