Surgical Management of Congenital Intrahepatic Bile Duct Dilatation, Caroli's Disease and Syndrome: Long-term Results of the French Association of Surgery Multicenter Study.
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.
Mabrut, J.-Y., Kianmanesh, R., Nuzzo, G., Castaing, D., Boudjema, K., Létoublon, C., Adham, M., Ducerf, C., Pruvot, F.-R., Meurisse, N., Cherqui, D., Azoulay, D., Capussotti, L., Lerut, J., Reding, R., Mentha, G., Roux, A., & Gigot, J.-F. (2013). Surgical Management of Congenital Intrahepatic Bile Duct Dilatation, Caroli’s Disease and Syndrome: Long-term Results of the French Association of Surgery Multicenter Study. Annals of Surgery, 258(5), 713-721. https://doi.org/10.1097/SLA.0000000000000269 (Original work published 2013)