Recurrent pyogenic cholangiohepatitis

Oriental cholangiohepatitis, or recurrent pyogenic cholangiohepatitis, is a condition essentially found in Southeast Asia and is characterised by intra and extrahepatic bile duct strictures and dilatation with intraductal pigmented stone formation. 

Diagnosis is made after exclusion of more common conditions such as biliary stricture of a known cause like previous surgery, trauma, primary or secondary sclerosing cholangitis, and cholangiocarcinoma. 

Clinical presentation

The common clinical presentation is that of recurrent RUQ pain, fever and jaundice. Leukocytosis with elevated alkaline phosphatase and bilirubin are seen.

Pathology

The exact aetiology is not well understood but strongly association with hepatobiliary infestation with Clonorchis sinensis (liver fluke) (see: clonorchiasis) or ascaris lumbricoides have been implicated. Other associations include poor nutritional or socioeconomic status and ascending cholangitis from gut Escherichia coli flora. 

The fluke acts like a nidus for stone formation and, either directly, or by causing strictures which aids which to theirstone formation.

Periductal inflammatory changes with infiltration of periportal spaces with inflammatory cells leading to periductal fibrosis and stricture which could ultimately result in focal liver fibrosis or diffuse biliary cirrhosis.

Radiographic features

MRCP is superior to ERCP in depicting intra- and extrahepatic changes. 

The best diagnostic clues are intra and extrahepatic biliary dilatation and multilevel strictures with intraductal pigmented calculi usually in the absence of gallbladder calculi, a combination of variable density calculi/sludge and regions of segmental liver atrophy (particularly lateral aspect of the left hepatic lobe) secondary to chronic biliary obstruction. 

CT 

• stones are usually hyperdense to the liver parenchyma
• focal areas of fibrosis with heterogenous enhancement and focal steatosis 

MRCP

• reduced arborization of peripheral ducts "arrowhead sign"
• multiple intra and extrahepatic biliary strictures

Treatment and prognosis

Interventional radiology plays a role in the percutaneous biliary drainage of affected segments, removal of pigment stones, balloon dilation of biliary strictures and repeated percutaneous procedures to clear pigment stones and mud-like biliary debris. 

Complications

• biliary cirrhosis
• cholangiocarcinoma (in ~5% of cases)