- Pathogenesis:
- A rare disorder characterized by saccular intrahepatic duct dilations with stones and strictures.
- Usually presents in adults as right upper quadrant pain, fever, and jaundice.
- Predisposes one to stones, cholangitis, liver abscess, hepatic fibrosis, portal hypertension.
- Treatment involves antibiotics, biliary drainage, and stone removal. However, overall prognosis is poor.
- Radiographic findings:
- Cholangiography shows saccular dilations with stones, strictures, and communicating hepatic abscesses.
- US: multiple cystic areas within the liver.
- CT: Also shows multiple cystic areas within the liver. In addition, a "central dot" sign is apparent, caused by visualization of a bright contrast-enhancing dot surrounded by a fluid density. The dot represents the intraluminal portal vein within a dilated duct.
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