MediAI

  Abstract Caroli disease is a rare congenital hepatobiliary disorder characterized by non-obstructive segmental dilatation of the intrahepatic bile ducts. Despite its rarity, accurate diagnosis is crucial due to complications including recurrent cholangitis, hepatolithiasis, liver fibrosis, and an increased risk of cholangiocarcinoma. Advanced imaging modalities such as ultrasound and magnetic resonance imaging (MRI) play a pivotal role in identifying the disease, particularly through the characteristic central dot sign . This article presents an in-depth academic review of Caroli disease pathophysiology, epidemiology, clinical presentation, imaging features, differential diagnosis, treatment, and prognosis , based on the latest global literature. A real clinical case of a 25-year-old female presenting with abdominal pain and elevated liver function tests is analyzed with ultrasound and MRI findings. Diagnostic imaging interpretation, differential diagnosis, and management strate...