title: Surgical management of congenital intrahepatic bile duct dilatation, Caroli's disease and syndrome: long-term results of the French Association of Surgery Multicenter Study.
abstract: 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.
authors.affiliation: *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.
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Mabrut
authors.forename: Reza
authors.initials: R
authors.lastname: Kianmanesh
authors.forename: Gennaro
authors.initials: G
authors.lastname: Nuzzo
authors.forename: Denis
authors.initials: D
authors.lastname: Castaing
authors.forename: Karim
authors.initials: K
authors.lastname: Boudjema
authors.forename: Christian
authors.initials: C
authors.lastname: Létoublon
authors.forename: Mustapha
authors.initials: M
authors.lastname: Adham
authors.forename: Christian
authors.initials: C
authors.lastname: Ducerf
authors.forename: François-René
authors.initials: FR
authors.lastname: Pruvot
authors.forename: Nicolas
authors.initials: N
authors.lastname: Meurisse
authors.forename: Daniel
authors.initials: D
authors.lastname: Cherqui
authors.forename: Daniel
authors.initials: D
authors.lastname: Azoulay
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Capussotti
authors.forename: Jan
authors.initials: J
authors.lastname: Lerut
authors.forename: Raymond
authors.initials: R
authors.lastname: Reding
authors.forename: Gilles
authors.initials: G
authors.lastname: Mentha
authors.forename: Adeline
authors.initials: A
authors.lastname: Roux
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Gigot
mesh_terms: D001650:Bile Duct Neoplasms / Q000150:complications / Q000601:surgery; D001653:Bile Ducts, Intrahepatic / Q000002:abnormalities*; D016767:Caroli Disease / Q000601:surgery*; D005260:Female; D005602:France; D006498:Hepatectomy / Q000379:methods; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications / Q000601:surgery; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications; D015996:Survival Rate; D016896:Treatment Outcome