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.
   

2. 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.