Hepatolithiasis and the syndrome of recurrent pyogenic cholangitis: clinical, radiologic, and pathologic features.

Primary hepatothiasis (HL) and recurrent pyogenic cholangitis (RPC) are two terms describing the different aspects of the same disease, with HL emphasizing the pathologic changes and RPC emphasizing the clinical presentation and suppurative inflammation. It is predominantly a disease of the Far East. In the 1960s, it was the third most common cause of emergency abdominal surgery at a university hospital in Hong Kong. Thereafter, its incidence has decreased considerably, possibly due to improved standards of living and Westernized diet. Clinically, patients may present acutely with recurrent bacterial cholangitis and its possible complications, such as liver abscess and septicemic shock, or with chronic complications, such as cholangiocarcinoma. Pathologically, it is characterized by pigmented calcium bilirubinate stones within dilated intrahepatic bile ducts featuring chronic inflammation, mural fibrosis, and proliferation of peribiliary glands, without extrahepatic biliary obstruction. Episodes of suppurative inflammation cumulate in sclerosing cholangitis of peripheral ducts and parenchymal fibrosis resulting from collapse and scarring. Mass-forming inflammatory pseudotumor and neoplasms like intraductal papillary neoplasms and cholangiocarcinoma are increasingly recognized complications. Modern imaging techniques allow definitive diagnosis, accurate assessment for treatment planning, and detection of complications. A multidisciplinary team approach (interventional endoscopist, interventional radiologist, hepatobiliary surgeon, and intensivists) is important for optimal patient outcome.