Hepatolithiasis — Intrahepatic Bile Duct Stones

Hepatolithiasis is the presence of calculi proximal to the confluence of the right and left hepatic ducts, with or without concurrent extrahepatic stones. It is endemic in East and Southeast Asia and increasingly recognized in Western countries in the setting of biliary strictures and parasitic infestations such as Clonorchis sinensis.

Pathogenesis involves bile stasis from intrahepatic strictures, bacterial deconjugation of bilirubin, and parasitic factors leading to brown pigment stones. Patients present with recurrent pyogenic cholangitis (RPC), abdominal pain, jaundice, and over time develop atrophy of affected liver segments and a markedly increased risk of intrahepatic cholangiocarcinoma (5-13%).

Diagnosis is made with MRCP, CT cholangiography, and cholangioscopy. Treatment combines endoscopic and percutaneous transhepatic cholangioscopic lithotripsy with biliary drainage, while hepatic resection is the definitive option for unilateral disease with stricture, atrophy, or suspicion of malignancy. Liver transplantation is considered for diffuse bilateral disease with secondary biliary cirrhosis.