title: [The Caroli's disease. Report of 16 cases].
abstract: BACKGROUND
  Caroli's disease is a congenital dilatation of the intrahepatic biliary duct.
  
  
  OBJECTIVE
  To analyse and discuss diagnostic and therapeutics difficulties through 16 patients with Caroli's disease.
  
  
  METHODS
  Between January 1990 and September 2010, 16 patients underwent surgical procedure for Caroli's disease. Data recorded for each patient included clinical symptoms, biologic findings, previous biliary procedures, and the presenting symptoms. The distribution of the biliary lesions, the surgical procedure and the postoperative outcomes and follow up were detailed.
  
  
  RESULTS
  The mean age was 55 years. The mean interval between the first symptoms and diagnosis was 27 months. Five of 16 patients had undergone 12 surgical or endoscopic procedures prior to liver resection. Before the definitive diagnosis, 9 patients presented 15 episodes of acute cholangitis. The diagnosis was established preoperatively in 13 cases, 5 among them underwent previous biliary surgical procedures. The diagnosis was documented peroperatively in 2 cases and postoperatively in 1 case. The distribution of the biliary lesions was monolobar in 13 and bilobar in 3 patients. 13 patients underwent liver resection, in two cases we perfomed biliojejunostomy and the last one had endoscopic sphincterotomy. There was no mortality and the overall postoperative morbidity is about 43%. The follow-up shows that 12 patients still alive with a mean follow up for 53 months, from whom only one patient have intrahépepatic lithiasis.
  
  
  CONCLUSIONS
  The clinical course of Caroli's disease is often complicated by recurrent episodes of angiocholitis and requires iterative surgery. The hepatectomy witch prevented septic complications and degenerescence is possible only in a restricted number of patients.
authors.affiliation: Service de chirurgie A, Hopital La Rabta Tunis, Tunisie.
authors.forename: Fadhel
authors.initials: F
authors.lastname: Fteriche
authors.forename: Amine
authors.initials: A
authors.lastname: Makni
authors.forename: Rachid
authors.initials: R
authors.lastname: Ksantini
authors.forename: Wael
authors.initials: W
authors.lastname: Rebai
authors.forename: Haikal
authors.initials: H
authors.lastname: Bedioui
authors.forename: Kaies
authors.initials: K
authors.lastname: Nouira
authors.forename: Amine
authors.initials: A
authors.lastname: Daghfous
authors.forename: Sofiane
authors.initials: S
authors.lastname: Ayadi
authors.forename: Faouzi
authors.initials: F
authors.lastname: Chebbi
authors.forename: Mohamed
authors.initials: M
authors.lastname: Jouini
authors.forename: A
authors.initials: A
authors.lastname: Ammous
authors.forename: Jameleddine
authors.initials: J
authors.lastname: Montassar Kacem
authors.forename: Zouheir
authors.initials: Z
authors.lastname: Ben Safta
mesh_terms: D000328:Adult; D000368:Aged; D016767:Caroli Disease / Q000175:diagnosis / Q000601:surgery; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged