Primary Intrahepatic Lithiasis

• Primary intrahepatic lithiasis (stones originated inside the intrahepatic biliary tree) is endemic in East Asia and much less common in the West. It is usually associated with intrahepatic biliary strictures and is responsible for recurrent episodes of cholangitis, hepatic atrophy, secondary biliary cirrhosis, and intrahepatic cholangiocarcinoma.

• Diagnosis of hepatolithiasis is usually established by non-invasive radiological methods. Accurate location of stones, detection of biliary strictures, identification of hepatic segments involved and/or atrophic, suspicion of cholangiocarcinoma, and/or the presence of cirrhosis and portal hypertension will ultimately lead the therapeutic approach.

• Treatment is challenging and sometimes controversial, and aims to prevent recurrent cholangitis and consequences of progression of disease (ultimately cholangiocarcinoma).

• Liver resection allows remotion of the stones, of the biliary strictures, and of the atrophic parenchyma, and ultimately diminishes the risk of cholangiocarcinoma. It seems to be the best surgical option for treatment of primary intrahepatic lithiasis.

• Well-established indications for hepatic resection of primary intrahepatic lithiasis include: i) lithiasis limited to one lobe, sector, or segment; ii) parenchymal hypo-atrophy; iii) presence of liver abscess; iv) failure of previous treatments; and v)suspected cholangiocarcinoma.