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Surgical Management of Congenital Intrahepatic Bile Duct Dilatation, Caroli's Disease and Syndrome: Long-term Results of the French Association of Surgery Multicenter Study

Mabrut, Jean-Yves MD, PhD*; Kianmanesh, Reza MD, PhD; Nuzzo, Gennaro MD; Castaing, Denis MD§; Boudjema, Karim MD, PhD; Létoublon, Christian MD; Adham, Mustapha MD, PhD**; Ducerf, Christian MD*; Pruvot, François-René MD††; Meurisse, Nicolas MD, PhD‡‡; Cherqui, Daniel MD§; Azoulay, Daniel MD, PhD§§; Capussotti, Lorenzo MD¶¶; Lerut, Jan MD, PhD‖‖; Reding, Raymond MD‖‖; Mentha, Gilles MD, PhD***; Roux, Adeline MSc†††; Gigot, Jean-François MD, PhD‖‖

doi: 10.1097/SLA.0000000000000269
Original Articles From the ESA Proceedings

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.

A total of 155 patients with congenital intrahepatic bile duct dilatation (Caroli disease and syndrome) were enrolled in a retrospective multicenter study. After a median follow-up of 35 months, the 5-year overall survival rate was 88.5% after liver resection or liver transplantation. Excellent or good results were achieved in 86.0% of patients.

*Croix-Rousse University Hospital, Lyon, France

Reims University Hospital, France

Gemelli Hospital Rome Italy

§Paul Brousse University Hospital, Villejuif, France

Rennes University Hospital France

University Hospital, Grenoble, France

**Edouard Herriot University Hospital, Lyon, France

††University Hospital, Lille, France

‡‡Gasthuisberg University Hospital (KUL) Leuven Belgium

§§University Hospital, Créteil, France

¶¶Mauriziano University Hospital Torino Italy

‖‖Cliniques Universitaires Saint-Luc (UCL), Brussels, Belgium

***University Hospital, Geneva, Switzerland; and

†††HCL, Pôle Information Médicale Evaluation Recherche, Unité de Recherche Clinique, Lyon France.

Reprints: Jean-Yves Mabrut, MD, PhD, Department of Digestive Surgery and Liver Transplantation, Croix-Rousse University Hospital, 103 Grande rue de la Croix-Rousse, 69 317 Lyon Cedex 04, France. E-mail: jean-yves.mabrut@chu-lyon.fr.

Disclosure: The authors declare no conflicts of interest.

© 2013 by Lippincott Williams & Wilkins.