Caroli's disease and choledochal cyst.

A 33-year old female was admitted to the hospital presented with right hypochondrium abdominal pain and jaundice. Abdominal ultrasonography showed multiple saccular dilatations of the intrahepatic biliary tract. Endoscopic Retrograde Cholangiopancreatography was performed (Figure 1A) showing nonobstructive saccular or fusiform dilatation of the intrahepatic bile ducts and choledochal cyst. Also it was performed a MR cholangiography (Figure 1B) which showed the same abnormalities described above. 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