In 1958
Jacques Caroli described communicating cavernous ectasia of the biliary
tree as an uncommon cause of chronic, often life-threatening hepatobiliary
disease. The disease now most often referred to as Caroli's disease is
a rare condition characterized by nonobstructive saccular or fusiform dilatation
of the intrahepatic bile ducts. In the so-called 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 dilatation usually is less prominent; portal
hypertension and eventual liver failure typically develop as a result of
the hepatic fibrosis. Caroli's disease usually is manifested in childhood
and is thought to be congenital and probably inherited. Associated conditions
include renal cystic disease, choledochal cysts, and cholangiocarcinoma.
This pictorial essay illustrates the broad spectrum of imaging findings
in Caroli's disease.
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