Primary intrahepatic gallstones

Caroli's disease is characterized by congenital segmental dilation of the intrahepatic bile ducts producing a variety of symptoms and, usually, primary intrahepatic gallstones. We are reporting five new cases of Caroli's disease in addition to the 91 reported in the literature. The diagnosis is made by intrahepatic cholangiography; techniques are emphasized. The diagnoses were confirmed by the surgical specimen of the left lobe of the liver in cases 1 and 2, by autopsy in case 3, and by intrahepatic cholangiogram in cases 4 and 5. The treatment resulted in leftsided lobectomy of liver in cases 1 and 2. Cholecystectomy, removal of common duct stones, choledochoduodenostomy or, preferably Roux-en-Y choledochojejunostomy, were done in cases 3, 4, and 5. Two patients died more than five years after their original surgery, and three are alive at six months, six, and 13 years, respectively. This is a more encouraging outcome than is usually reported.