Oriental cholangiohepatitis: pathologic, clinical, and radiologic features.

Oriental cholangiohepatitis, an endemic disease in Southeast Asia, is characterized by recurrent attacks of abdominal pain, fever, and jaundice. Pathologically, the intra- and extrahepatic ducts are dilated and contain soft, pigmented stone and pus. There is proliferation of bile ducts and infiltration of inflammatory cells along the periportal spaces and hepatic parenchyma. Localized intrahepatic segmental ductal stenosis may be present, especially in the lateral segment of the left lobe or posterior segment of the right hepatic lobe. The cause of the disease is not known, but associations with clonorchiasis, ascariasis, and nutritional deficiency have been suggested. Sonographic and CT findings include intra- or extrahepatic duct stones, dilatation of the extrahepatic duct with relatively mild or no dilatation of the intrahepatic ducts, localized dilatation of the lobar or segmental bile ducts, increased periportal echogenicity, segmental hepatic atrophy, and gallstones. Cholangiographic findings include bile duct stones; disproportionately severe dilatation of the extrahepatic ducts with mild or no dilatation of the intrahepatic ducts; and focal strictures, acute peripheral tapering, straightening, rigidity, decreased arborization, and an increased branching angle of the intrahepatic bile ducts.