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Objective: To assess clinical presentation and long-term results of surgical management of congenital intrahepatic bile duct dilatation (IHBDD) (Caroli disease and syndrome) in a multicenter setting., Background: Congenital IHBDD predisposes to biliary stasis, resulting in intrahepatic lithiasis, septic complications, and cholangiocarcinoma. Although liver resection (LR) is considered to be the treatment of choice for unilobar disease extent into the liver, the management of bilobar disease and/or associated congenital hepatic fibrosis remains challenging., Methods: From 1978 to 2011, a total of 155 patients (median age: 55.7 years) were enrolled from 26 centers. Bilobar disease, Caroli syndrome, liver atrophy, and intrahepatic stones were encountered in 31.0%, 19.4%, 27.7%, and 48.4% of patients, respectively. A complete resection of congenital intrahepatic bile ducts was achieved in 90.5% of the 148 patients who underwent surgery., Results: Postoperative mortality was nil after anatomical LR (n = 111) and 10.7% after liver transplantation (LT) (n = 28). Grade 3 or higher postoperative morbidity occurred in 15.3% of patients after LR and 39.3% after LT. After a median follow-up of 35 months, the 5-year overall survival rate was 88.5% (88.7% after LT), and the Mayo Clinic score was considered as excellent or good in 86.0% of patients. The 1-year survival rate was 33.3% for the 8 patients (5.2%) who presented with coexistent cholangiocarcinoma., Conclusions: LR for unilobar and LT for diffuse bilobar congenital IHBDD complicated with cholangitis and/or portal hypertension achieved excellent long-term patient outcomes and survival. Because of the bad prognosis of cholangiocarcinoma and the sizeable morbidity-mortality after LT, timely indication for surgical treatment is of major importance.

This study was undertaken to determine the results of resection of hepatocellular carcinoma in cirrhotic patients in Europe, using the same criteria as in the Orient for detection, surgical management, and pathology of the tumors. Seventy-two patients had a liver resection. One- and 3-yr survival rates were 68% and 51%, respectively. Survival rate was significantly higher in Child's/Pugh's class A than in class B-C patients. Patients with a thickly encapsulated tumor lived longer than those with an infiltrating tumor and had in addition a significantly lower incidence of cancer recurrence. Class A patients with a thickly encapsulated hepatocellular carcinoma had a 77% 3-year survival rate. There was no relation between the size of the tumor or the presence of symptoms and survival. These data suggest that good results can be achieved by resection of hepatocellular carcinomas in European cirrhotic patients. A thickly encapsulated tumor and an adequate liver function are the main determinants of low cancer recurrence and high survival. The clinical results in this series are similar to those reported from the Orient.