KEY FACTS
Terminology
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Recurrent episodes of acute pyogenic cholangitis with intra- and extrahepatic biliary pigment stones
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Synonyms: Hepatolithiasis, oriental cholangiohepatitis
Imaging
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Intra- and extrahepatic biliary ductal dilatations with stones
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Lateral segment of left lobe and posterior segment of right lobe more commonly involved
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Biliary ductal thickening due to repeated inflammation
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Severe atrophy of affected lobe/segment, biliary cirrhosis
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Grayscale ultrasound
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Presence of echogenic sludge/stones ± posterior acoustic shadowing in intrahepatic and extrahepatic duct
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Periportal hypo-/hyperechogenicity due to periductal inflammation
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Ductal rigidity and straightening, rapid tapering of peripheral intrahepatic duct
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Cholangiography and MRCP
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Intra- and extrahepatic duct dilatation with stones as filling defects
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Top Differential Diagnoses
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Ascending cholangitis
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Sclerosing cholangitis
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Cholangiocarcinoma
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Intrahepatic stones secondary to biliary stricture
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Caroli disease
Clinical Issues
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Common symptoms/signs: Recurrent episodes of RUQ pain, fever, and jaundice
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Risk of developing cholangiocarcinoma (5-6%)
Diagnostic Checklist
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Consider recurrent pyogenic cholangitis in southeast Asian patients with recurrent episodes of acute bacterial cholangitis
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Imaging interpretation pearl: Intra- and extrahepatic bile duct dilatation with pigmented stones and ductal inflammation
Scanning Tips
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To better demonstrate posterior acoustic shadowing of intrahepatic stones, consider turning off compounding
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Echogenic sludge may be mistaken for artifact; zooming in or changing windows may help to distinguish real echoes from artifact
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