Monolobar Caroli's disease

Two cases of Caroli's disease confined to the left lobe of the liver are described. One patient is a 13-yr-old who presented with acute pancreatitis. The second patient presented with obstructive jaundice at age 60. In the latter case, the diagnosis was made by percutaneous transhepatic cholangiography, in the former by postoperative T-tube cholangiography. The diseased bile ducts were not resected in either patient. Both remain well at 4 and 5.5 yr postoperatively. Monolobar involvement in Caroli's disease has been described previously in only 34 published cases, and these are reviewed in detail.