title: Surgical Management of Caroli's Disease: Single Center Experience and Review of the Literature.
abstract: BACKGROUND
  Caroli's disease is a rare congenital condition characterized by non-obstructive dilatation of intrahepatic ducts. In Caroli's syndrome, there is additionally an associated congenital hepatic fibrosis.
  
  
  METHODS
  With institutional review board approval, we identified all patients with Caroli's disease and syndrome.
  
  
  RESULTS
  Nine patients were identified, seven males and two females, with a median age of 40 years. Final pathological diagnoses included Caroli's disease (n = 6) and Caroli's syndrome (n = 3). Patients presented with deranged liver function, cholangitis, cholangiocarcinoma, abdominal pain, cirrhosis, or were diagnosed incidentally. Four patients underwent resection and two underwent liver transplantation. Of the resection group, two patients subsequently underwent transplantation for recurrent cholangitis due to anastomotic stricture in one patient and for end-stage liver disease in the other. All patients with Caroli's syndrome underwent liver transplantation. Three patients died during follow-up at 26.2, 7.8, and 3 months post-diagnosis with recurrence of cholangiocarcinoma, liver failure, and metastatic cholangiocarcinoma, respectively. Six patients are alive with a median follow-up of 60 months since presentation (range = 10-134 months).
  
  
  CONCLUSIONS
  Caroli's disease and syndrome have a varied presentation. Most individuals with Caroli's disease may be adequately treated by resection, but transplantation is required for Caroli's syndrome patients due to the associated hepatic fibrosis.
authors: Department of HPB Surgery, Digestive Disease Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA. moslimm@ccf.org.
authors: Maitham A
authors: MA
authors: Moslim
authors: Recanati Miller Transplant Institute, Mount Sinai Hospital, 1425 Madison Avenue, New York, NY, 10029, USA.
authors: Ganesh
authors: G
authors: Gunasekaran
authors: Department of HPB Surgery, Digestive Disease Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA.
authors: David
authors: D
authors: Vogt
authors: Department of Anatomic Pathology, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA.
authors: Michael
authors: M
authors: Cruise
authors: Department of HPB Surgery, Digestive Disease Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA.
authors: Gareth
authors: G
authors: Morris-Stiff
mesh_terms: D000293:Adolescent; D000328:Adult; D016767:Caroli Disease / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D030342:Genetic Diseases, Inborn / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D007223:Infant; D008103:Liver Cirrhosis / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D055815:Young Adult
keywords: Caroli’s disease; Caroli’s syndrome; Liver resection; Liver transplantation