Diffuse Caroli's disease with hepatolithiasis: a case report

Caroli disease is characterized by segmental, non-obstructive dilatation of the intrahepatic bile ducts, leading to bile stasis, recurrent cholangitis, hepatolithiasis, and an increased risk of cholangiocarcinoma. Caroli's disease, together with hepatic fibrosis, is termed Caroli's syndrome, which may lead to portal hypertension. Caroli's disease often requires treatment in symptomatic patients. Symptoms include recurrent cholangitis due to intrahepatic stones in dilated ducts or cirrhosis due to fibrosis. Liver transplantation remains the definitive treatment for diffuse disease. However, owing to organ shortages and long waiting lists, liver resection is an alternative for localized disease. Here, we present a rare case of an 18-year-old male with symptomatic, right-lobe-predominant Caroli's disease and hepatolithiasis who underwent surgical resection (right hepatectomy, stone extraction from exposed ducts at the resection margin, and intrahepatic-jejunostomy) with symptom resolution. This case highlights the role of liver resection in localized disease and reinforces the importance of individualized interventions in resource-limited settings.