[The Caroli's disease. Report of 16 cases].

Background: Caroli's disease is a congenital dilatation of the intrahepatic biliary duct.

Aim: 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.

Conclusion: 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.