Caroli's disease: liver resection and liver transplantation. Experience in 33 patients.

Background: The aim of this study was to review and discuss our observations on 33 patients who underwent surgical treatment for Caroli's disease (CD), focusing on diagnosis, current surgical management, and long-term outcome.
Methods: Between May 1993 and June 2004, 642 liver resections and 286 liver transplantations in 252 patients were performed in our department of surgery. Thirty-three patients were referred to our center for diagnostic and therapeutic management of CD. Prior surgical interventions for hepatobiliary disorders, current diagnostic and surgical procedures, procedure-specific complications, duration of hospital stay, duration of follow-up, outpatient information, and long-term outcome were reviewed.
Results: Fifteen male and 18 female patients were treated in this study. Initial symptoms and signs of the disease noted in our patients included right upper quadrant pain, fever, and jaundice. In 2 of the 33 patients, we noted clinical evidence of cirrhosis followed by histologic confirmation. One patient suffered from variceal bleeding. In 26 patients, diagnoses were established by a combined endoscopic retrograde cholangiopancreatography, ultrasonography, and computed tomographic studies. The disease was localized in 25 and diffuse in 8 patients. Liver resection was carried out in 29 patients. Partial hepatectomies were performed in 27 of these 29 at our institution. Two female patients with the diffuse disease underwent orthotopic liver transplantation. Thirteen of the 31 patients who underwent surgery at our institution had an uneventful postoperative course. Fourteen patients had minor postoperative complications and responded well to medical management. Four patients had major complications that required further surgical treatment. Two patients died of complications related to postoperative hemorrhage and sepsis. Two patients with intrahepatic cholangiocarcinoma died because of primary tumor progress. One patient with cholangiocarcinoma died 1 year after a successful left hepatectomy because of metastatic disease recurrence. The long-term results of the 26 surviving patients were assessed during a mean follow-up of 3.7 years (range, 1-11 years). All 26 patients remained free of biliary symptoms or complications. In 25 patients, surgery including liver transplantation was curative.
Conclusions: Partial hepatectomy for localized CD is potentially curative. In patients with diffuse CD, liver transplantation provides gratifying long-term results.