Review autosomal recessive polycystic kidney disease (ARPKD) and its main presentation in association with liver disease. Show the difference between Caroli's disease and Caroli's syndrome. Familiarize radiologists with the spectrum of these uncommon diagnoses using different imaging methods.
Caroli’s disease (CD),
also known as communicating cavernous ectasia or congenital saccular dilatation of the intrahepatic bile ducts,
is a rare congenital disorder first specifically described in 1958.
Although they are diagnosed in patients of all ages,
biliary tree cysts are primarily seen in children,
particularly in Asian populations.
It is characterized by multifocal segmental dilatation of the intrahepatic bile ducts,
involving the entire liver,
a lobe or a single segment,
Findings and procedure details
Reviewing our database we foundtwo interesting cases demonstrating multimodal images (ultrasound and MRI) showing typical findings in Caroli's syndrome.
Based on prevalence and morbid/mortality of this condition,
we believe that radiologists should have knowledge about its imaging findings that may turn the diagnosis a bit uncomplicated.
[A new disease,
of the bile ducts: unilobar cystic dilation of the
Sem Hop 1958;34:496-502/SP. Giovanardi RO.
Monolobar Caroli’s disease in an adult.
Hepatogastroenterology 2003;50:2185-2187. Pezzilli R,
Education and imaging.
and pancreatic: Caroli’s disease.
J Gastroenterol Hepatol 2008;23:1621.