Recurrent cholangitis + intrahepatic ductal dilation, suspected Caroli disease
Recommended: MRCP is the preferred imaging study for recurrent cholangitis + intrahepatic ductal dilation, suspected caroli disease. Congenital saccular intrahepatic biliary dilation.
Recommended study
MRCP
Congenital saccular intrahepatic biliary dilation. Caroli syndrome adds congenital hepatic fibrosis. Increased cholangiocarcinoma risk.
If the default doesn't apply
Surveillance for cholangiocarcinoma
MRI
Annual MRCP + CA 19-9
No contrast
Watch-outs
Cholangiocarcinoma risk
~7% lifetime; annual MRCP + tumor markers; transplant for diffuse disease.
Associated with ARPKD
Look for renal findings.
Pearls
- Central dot sign: enhancing portal radicles within dilated ducts on CT/MRI.
- Distinguish from primary sclerosing cholangitis (segmental strictures, no saccular dilation).
- Treatment: lobar resection (focal) or transplant (diffuse).