Recurrent pyogenic cholangiohepatitis
Citation, DOI, disclosures and article data
At the time the article was created Charudutt Jayant Sambhaji had no recorded disclosures.View Charudutt Jayant Sambhaji's current disclosures
At the time the article was last revised Jeremy Jones had no recorded disclosures.View Jeremy Jones's current disclosures
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.
The common clinical presentation is that of recurrent right upper quadrant pain, fever, and jaundice. Leukocytosis with elevated alkaline phosphatase and bilirubin are seen.
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 that 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.
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.
- stones are usually hyperdense to the liver parenchyma
- focal areas of fibrosis with heterogeneous enhancement and focal steatosis
- 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.
- 1. Park MS, Yu JS, Kim KW et-al. Recurrent pyogenic cholangitis: comparison between MR cholangiography and direct cholangiography. Radiology. 2001;220 (3): 677-82. doi:10.1148/radiol.2202001252 - Pubmed citation
- 2. Kim MJ, Cha SW, Mitchell DG et-al. MR imaging findings in recurrent pyogenic cholangitis. AJR Am J Roentgenol. 1999;173 (6): 1545-9. AJR Am J Roentgenol (citation) - Pubmed citation
- 3. Cosenza CA, Durazo F, Stain SC et-al. Current management of recurrent pyogenic cholangitis. Am Surg. 1999;65 (10): 939-43. - Pubmed citation
- 4. Vansonnenberg E, Casola G, Cubberley DA et-al. Oriental cholangiohepatitis: diagnostic imaging and interventional management. AJR Am J Roentgenol. 1986;146 (2): 327-31. AJR Am J Roentgenol (abstract) - Pubmed citation
- 5. Lim JH. Oriental cholangiohepatitis: pathologic, clinical, and radiologic features. AJR Am J Roentgenol. 1991;157 (1): 1-8. AJR Am J Roentgenol (abstract) - Pubmed citation
- 6. Goldberg HI, Gordon R. Diagnostic and interventional procedures for the biliary tract. Curr Opin Radiol. 1991;3 (3): 453-62. - Pubmed citation
- 7. Kusano S, Okada Y, Endo T et-al. Oriental cholangiohepatitis: correlation between portal vein occlusion and hepatic atrophy. AJR Am J Roentgenol. 1992;158 (5): 1011-4. AJR Am J Roentgenol (abstract) - Pubmed citation
- 8. Federle MP, Cello JP, Laing FC et-al. Recurrent pyogenic cholangitis in Asian immigrants. Use of ultrasonography, computed tomography, and cholangiography. Radiology. 1982;143 (1): 151-6. Radiology (abstract) - Pubmed citation
- 9. Katabathina VS, Dasyam AK, Dasyam N et-al. Adult bile duct strictures: role of MR imaging and MR cholangiopancreatography in characterization. Radiographics. 2014;34 (3): 565-86. doi:10.1148/rg.343125211 - Pubmed citation
- 10. Gore RM, Levine MS. Textbook of Gastrointestinal Radiology. Elsevier Health Sciences. (2015) ISBN:0323278116. Read it at Google Books - Find it at Amazon
- 11. Wani NA, Robbani I, Kosar T. MRI of oriental cholangiohepatitis. Clin Radiol. 2011;66 (2): 158-63. doi:10.1016/j.crad.2010.06.017 - Pubmed citation