Caroli disease presenting with recurrent cholangitis and secondary small bowel obstruction: a case report

Abstract

Caroli disease is a rare congenital hepatobiliary disorder characterized by non-obstructive dilatation of the intrahepatic bile ducts and classified as Todani type V choledochal cyst. Although congenital in origin, the disease is often diagnosed in adulthood due to its indolent course and nonspecific symptoms, frequently after complications such as recurrent cholangitis have occurred. We report a case of a 46-year-old male presenting with recurrent epigastric pain, abdominal distension, fever, jaundice, nausea, vomiting, and bowel movement disturbance. Laboratory findings revealed leukocytosis, direct hyperbilirubinemia, and cholestatic liver enzyme elevation consistent with acute cholangitis. Abdominal radiography demonstrated features of partial small bowel obstruction. Magnetic resonance cholangiopancreatography (MRCP) with contrast revealed multifocal cystic dilatation of the intrahepatic bile ducts without extrahepatic obstruction, consistent with Caroli disease. The patient was managed conservatively with intravenous antibiotics, ursodeoxycholic acid, bowel decompression, and supportive care, resulting in gradual clinical improvement. This case highlights the diagnostic value of MRCP and demonstrates that conservative management can be effective in adult Caroli disease complicated by recurrent cholangitis and secondary small bowel obstruction.