Rare Cause of Cholestasis: Caroli’s Syndrome

Caroli disease is a rare congenital disease characterized by multiple segmental cystic, saccular dilatations of the intrahepatic biliary tract. We diagnosed Caroli’s syndrome in our patient who applied with cholangitis attack with the 'Central Dot Sign' finding in abdominal computed tomography, which is a characteristic finding for Caroli’s Syndrome; we wanted to emphasize the value of radiological data in diagnosis. As it is a rare syndrome, it should be considered during the differential diagnosis of cholestasis. An 18-month-old girl was admitted to our hospital because of her jaundice that had not improved for a month. The patient had hypertransaminasemia and direct hyperbilirubinemia, because of the high markers of infection, antibiotherapy was started by considering cholangitis. In abdominal ultrasonography, liver parenchyma was interpreted as having multiple cystic lesions and dilatation in intrahepatic bile ducts. The patient's abdominal computed tomography had saccular, fusiform dilatations of the intrahepatic bile ducts in both liver lobes. 'Central Dot Sign' finding was observed in intrahepatic bile ducts. Portal, periportal fibrosis and bile duct proliferation, bridging, nodule formation were present in the patient's liver biopsy. In our patient who was investigated for neonatal cholestasis, we diagnosed Caroli’s syndrome on the presence of the 'Central Dot Sign', a characteristic finding in computed tomography. The early diagnosis of this very rare disease affects the prognosis after liver transplantation. Caroli’s syndrome should be considered in the differential diagnosis in every patient presenting with cholestasis, especially in the presence of recurrent cholangitis attacks.