In 1958 Jacques Caroli described communicating cavernous ectasia of th
e biliary tree as an uncommon cause of chronic, often life-threatening
hepatobiliary disease. The disease now most often referred to as Caro
li's disease is a rare condition characterized by nonobstructive saccu
lar or fusiform dilatation of the intrahepatic bile ducts, In the so-c
alled pure form, dilatation is classically segmental and saccular and
is associated with stone formation and recurrent bacterial cholangitis
. In the form associated with congenital hepatic fibrosis, bile duct d
ilatation usually is less prominent; portal hypertension and eventual
liver failure typically develop as a result of the hepatic fibrosis. C
aroli's disease usually is manifested in childhood and is thought to b
e congenital and probably inherited. Associated conditions include ren
al cystic disease, choledochal cysts, and cholangiocarcinoma. This pic
torial essay illustrates the broad spectrum of imaging findings in Car
oli's disease.