Recurrent pyogenic cholangiohepatitis
Updates to Article Attributes
Oriental cholangiohepatitis (or recurrent pyogenic cholangiohepatitis) is a condition essentially found in Southeast Asia and is causedcharacterised by hepatobilliary infestationintra- and extrahepatic bile duct strictures and dilatation withChlonorchis sinensis (liver fluke) (see: clonorchiasis) Although other organisms have also been implicated:
It Diagnosis is a diagnosis made after exclusion of themore common differential diagnosis for this condition, that include Intrahepatic stones secondary toconditions such as biliary stricture of known cause like previous surgery, trauma etc., primary or secondary sclerosing cholangitis and cholangiocarcinoma.
The condition is usually associated with poor nutritional status.
Clinical presentation
The common clinical presentation is that of recurrent RUQ pain, recurrent fevers fever and jaundice. Leukocytosis, with elevated alkaline phosphatase and bilirubin are seen.
Pathology
The exact etiology is not well understood but strongly association with hepatobilliary infestation with Chlonorchis sinensis (liver fluke) (see: clonorchiasis) or ascaris lumbricoideshave 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 aids which to their formation.
Periductal inflammatory changes with infiltration of periportal spaces with inflammatory cells leading to periductal fibrosis and stricture which could ultimately leads into focal liver fibrosis or diffuse biliary cirrhosis are the histopathological findings.
Radiographic features
MRCP is superior to ERCP in depicting intra- and extrahepatic changes.
The best diagnostic clues are intra- and extra-hepatic biliary dilatation due toand multilevel strictures andwith intraductal pigmented calculi within them withoutusually in the absence of gall bladder calculi, 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 scan
- 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 extra hepatic biliary strictures
Management
Interventional radiology plays a role in 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 about 5% of cases)
-<p><strong>Oriental cholangiohepatitis</strong> (or <strong>recurrent pyogenic cholangiohepatitis</strong>) is a condition essentially found in Southeast Asia and is caused by hepatobilliary infestation with <em><a href="/articles/chlonorchis-sinensis">Chlonorchis sinensis</a></em> (liver fluke) (see: <a href="/articles/clonorchiasis">clonorchiasis</a>). Although other organisms have also been implicated:</p><ul>-<li><em><a href="/articles/ascaris-lumbricoides">Ascaris lumbricoides</a></em></li>-<li><em><a href="/articles/fasciola-hepatica">Fasciola hepatica</a></em></li>-<li><em><a href="/articles/opisthorchis-sinesis">Opisthorchis sinesis</a></em></li>-<li><em><a href="/articles/entamoeba">Entamoeba </a></em></li>-</ul><p>It is a diagnosis made after exclusion of the common differential diagnosis for this condition, that include Intrahepatic stones secondary to biliary stricture of known cause like previous surgery, trauma etc., <a href="/articles/sclerosing-cholangitis">sclerosing cholangitis</a> and <a href="/articles/cholangiocarcinoma">cholangiocarcinoma</a>.</p><p>The condition is usually associated with poor nutritional status.</p><h4>Clinical presentation</h4><p>The common clinical presentation is that of RUQ pain, recurrent fevers and <a href="/articles/jaundice">jaundice</a>. Leukocytosis, elevated alkaline phosphatase and bilirubin are seen.</p><h4>Pathology</h4><p>The fluke acts like a nidus for stone formation and either directly or by causing strictures aids which to their formation.</p><p>Periductal inflammatory changes with infiltration of periportal spaces with inflammatory cells leading to periductal fibrosis and ultimately biliary cirrhosis are the histopathological findings.</p><h4>Radiographic features</h4><p>The best diagnostic clues are intra- and extra-hepatic biliary dilatation due to multilevel strictures and calculi within them without gall bladder calculi, combination of variable density calculi/sludge and regions of segmental liver atrophy secondary to chronic biliary obstruction.</p><h4>Management</h4><p>Interventional radiology plays a role in 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. </p><h5>Complications</h5><ul>- +<p><strong>Oriental cholangiohepatitis</strong> (or <strong>recurrent pyogenic cholangiohepatitis</strong>) is a condition essentially found in Southeast Asia and is characterised by intra- and extrahepatic <span style="line-height:13.8667px">bile duct strictures and dilatation with intra ductal </span><span style="line-height:1.6">pigmented stone formation. Diagnosis is made after exclusion of more common conditions such as biliary stricture of known cause like previous surgery, trauma etc, primary or secondary </span><a style="line-height: 1.6;" href="/articles/sclerosing-cholangitis">sclerosing cholangitis</a><span style="line-height:1.6"> and </span><a style="line-height: 1.6;" href="/articles/cholangiocarcinoma">cholangiocarcinoma</a><span style="line-height:1.6">. </span></p><h4>Clinical presentation</h4><p>The common clinical presentation is that of recurrent RUQ pain, fever and <a href="/articles/jaundice">jaundice</a>. Leukocytosis with elevated alkaline phosphatase and bilirubin are seen.</p><h4>Pathology</h4><p>The exact etiology is not well understood but strongly association with hepatobilliary infestation with <em><a href="/articles/chlonorchis-sinensis">Chlonorchis sinensis</a></em> (liver fluke) (see: <a href="/articles/clonorchiasis">clonorchiasis</a>) or <em><a href="/articles/ascaris-lumbricoides">ascaris lumbricoides</a> </em>have been implicated. Other associations include poor nutritional or socioeconomic status and ascending cholangitis from gut Escherichia coli flora. </p><p>The fluke acts like a nidus for stone formation and either directly or by causing strictures aids which to their formation.</p><p>Periductal inflammatory changes with infiltration of periportal spaces with inflammatory cells leading to periductal fibrosis and stricture which could ultimately leads into focal liver fibrosis or diffuse biliary cirrhosis.</p><h4>Radiographic features</h4><p>MRCP is superior to ERCP in depicting intra- and extrahepatic changes. </p><p>The best diagnostic clues are intra- and extra-hepatic biliary dilatation and multilevel strictures with intraductal pigmented calculi usually in the absence of gall bladder calculi, 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. </p><h5>CT scan </h5><ul>
- +<li>stones are usually hyperdense to the liver parenchyma</li>
- +<li>focal areas of fibrosis with heterogenous enhancement and focal steatosis </li>
- +</ul><h5>MRCP</h5><ul>
- +<li>reduced arborization of peripheral ducts "arrowhead sign"</li>
- +<li>multiple intra- and extra hepatic biliary strictures</li>
- +</ul><h4>Management</h4><p>Interventional radiology plays a role in 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. </p><h5>Complications</h5><ul>
-<li><a href="/articles/cholangiocarcinoma">cholangiocarcinoma</a></li>- +<li>
- +<a href="/articles/cholangiocarcinoma">cholangiocarcinoma</a> (in about 5% of cases)</li>
References changed:
- 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. <a href="http://dx.doi.org/10.1148/rg.343125211">doi:10.1148/rg.343125211</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/24819781">Pubmed citation</a><span class="auto"></span>
- 10. Gore RM, Levine MS. Textbook of Gastrointestinal Radiology. Elsevier Health Sciences. (2015) ISBN:0323278116. <a href="http://books.google.com/books?vid=ISBN0323278116">Read it at Google Books</a> - <a href="http://www.amazon.com/gp/product/0323278116">Find it at Amazon</a><span class="auto"></span>
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