Hepatolithiasis (oriental cholangiohepatitis) has reportedly been endemic only in East Asia. The disease is now occasionally recognized in Western societies, especially in people who have lived in the Orient. Hepatolithiasis is characterized by its intractable nature and frequent recurrence, requiring multiple operative interventions, which is in distinct contrast to gallbladder stones. In addition to frequent cholangitis and chronic sepsis, it is widely known that longstanding intrahepatic stones lead to intrahepatic cholangiocarcinoma. Symptoms of hepatolithiasis include abdominal pain, jaundice and cholangitis. Pyogenic cholangitis due to strictures and hepatolithiasis tends to recur, and sometimes patients may present with liver abscesses. Radiological studies and percutaneous procedures are keys in the diagnosis and treatment of hepatolithiasis. Noninvasive imaging modalities such as ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) accurately depict the normal anatomy and presence of intrahepatic stones. It should be stressed that each modality has its pros and cons, and imaging studies should be performed on the basis of understanding the pathophysiology. As the diagnostic role of magnetic resonance cholangiopancreatography (MRCP) evolves, the roles of both endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC), and their most significant advantage, is primarily therapeutic with their ability to extract stones, biopsy intraductal lesions, and place stents easily. The primary goals of treatment are to eliminate attacks of cholangitis and to stop the progression of the disease (which leads to biliary cirrhosis). Surgery has a primary role in hepatolithiasis because hepatolithiasis tends to recur, so that multiple sessions of the endoscopic approach (i.e. two or three times a year) are often required. PTC is an alternative when surgical resection of the affected lobe is difficult. Techniques for lithotripsy, including shockwave and laser, can be applied in endoscopic sessions, offering a better chance of clearing the stones.
