recurrent pyogenic cholangiohepatitis

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Recurrent pyogenic cholangiohepatitis, previously known as oriental cholangiohepatitis, is a condition most commonly found in patients residing in or immigrated from Southeast Asia and is characterized by intra and extrahepatic bile duct strictures and dilatation with an intraductal pigmented stone formation.

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

Clinical presentation

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

Pathology

The exact etiology is not well understood but 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, either directly, or by causing strictures which aid stone 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 heterogeneous 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.