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