title: Long-term outcome of liver resection and transplantation for Caroli disease and syndrome.
abstract: OBJECTIVE
  To assess the preoperative disease characteristics as well as the rate of postoperative complications, patient survival, and course of symptoms after liver resection or orthotopic liver transplantation (OLT) for Caroli disease (CD) or syndrome (CS).
  
  
  BACKGROUND
  The clinical course of monolobar or diffuse CD or CS is often characterized by multiple conservative treatment attempts and interventions with recurrent episodes of cholangitis and a serious reduction in quality of life. The role and effectiveness of surgical treatment is still not well defined.
  
  
  METHODS
  Between June 1989 and December 2002, we treated 44 consecutive patients with CD or CS who had failure of conservative treatment before and were referred for surgical intervention. Demographic and clinical data, operative procedures and related morbidity, course of symptoms, and long-term follow-up were reviewed. Four patients with palliative resection for cholangiocarcinoma and incidental diagnosis of CD were excluded from the analysis.
  
  
  RESULTS
  Twenty-two women and 18 men had a median period of 26.5 months from onset of symptoms to surgical therapy. Their median age at therapy was 49 years and 80% of the patients had monolobar disease with a left-right ratio of 2.6 to 1. Thirty-three (82.5%) patients underwent liver resection, while 4 (10%) patients received OLT for diffuse disease. Biliodigestive anastomosis alone was performed in 3 (7.5%) patients with contraindications to OLT. Patients (37.5%) had minor postoperative complications, which were treated conservatively, while 2 (5%) transplanted patients had a reoperation due to intraperitoneal bleeding. After a median follow-up of 86.5 months, we observed a favorable patient and graft survival. Three deaths during follow-up were not related to treatment or disease complications. Follow-up of disease-related symptoms, biliary complications, and antibiotic treatment revealed a significant improvement.
  
  
  CONCLUSIONS
  Our data show that liver resection for monolobar CD or CS and OLT for diffuse manifestations can achieve excellent long-term patient survival with marked symptom relief. Because of life-threatening long-term complications such as biliary sepsis and development of cholangiocarcinoma, timely indication for surgical treatment is crucial.
authors.affiliation: Department of General, Visceral and Transplantation Surgery, Charité, Campus Virchow Clinical Centre, Berlin, Germany. frank.ulrich@charite.de
authors.forename: Frank
authors.initials: F
authors.lastname: Ulrich
authors.forename: Johann
authors.initials: J
authors.lastname: Pratschke
authors.forename: Andreas
authors.initials: A
authors.lastname: Pascher
authors.forename: Ulf P
authors.initials: UP
authors.lastname: Neumann
authors.forename: Enrique
authors.initials: E
authors.lastname: Lopez-Hänninen
authors.forename: Sven
authors.initials: S
authors.lastname: Jonas
authors.forename: Peter
authors.initials: P
authors.lastname: Neuhaus
mesh_terms: D000328:Adult; D000368:Aged; D016767:Caroli Disease / Q000401:mortality / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006085:Graft Survival; D006498:Hepatectomy / Q000379:methods*; D006801:Humans; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D015996:Survival Rate; D013577:Syndrome; D013997:Time Factors; D016896:Treatment Outcome