Surgical treatment of congenital dilatation of the biliary system

Over a total of 1612 patients undergoing surgery for biliary diseases, in 6 cases a cystic dilatation of intrahepatic bile-ducts suggestive of Caroli's disease was diagnosed. One patient with multiple dilatation of intrahepatic bile ducts and septic shock was treated by external drainage without improvement and died. Two patients were treated by left hepatectomy. One case was treated by right extended hepatic lobectomy. One patient with liver cirrhosis and bleeding oesophageal varices was treated by a distal splenorenal shunt. The last patient showed the association of a choledochal cyst and cystic dilatation of the left intrahepatic bile ducts. She was treated by resection of the cyst and biliary-jejunal anastomosis. Five patients had satisfactory recovery from the operation. They are leading a normal life, and are symptom-free. The different forms of treatment are discussed confirming that the best surgical technique on patients with partial cystic dilatation of bile ducts is liver resection. When the extrahepatic bile ducts are affected, biliary-jejunal diversion is necessary. If a choledochal cyst is present, the removal of the cyst is the most radical approach. When cirrhosis and portal hypertension with bleeding varices are diagnosed, portosystemic shunt is necessary.