- autosomal recessive abnormality in ductal plate development
- communicating cavernous biliary ectasia
- US will show:
- dilated intrahepatic bile ducts that are saccular or fusiform
- outpouchings may contain sludge/calculi due to biliary stasis
- central dot sign - color Doppler shows flow in the portal triads surrounded by the dilated ducts
- ERCP/MRCP will show dilated ducts that communicate with the central biliary tree
Caroli's syndrome is Caroli's disease + congenital hepatic fibrosis.
Levy AD, Rohrmann CA, Murakata LA, et al. Caroli's disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol 2002;179:1053-7.