GI Radiology > Biliary > Bile Ducts > Congenital > Caroli's Disease

Congenital

Caroli's Disease (Type V Choledochal cyst)

  1. 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.
     
  1. 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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