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Long-term outcome of liver resection and transplantation for Caroli disease and syndrome.
Ann Surg. 2008 Feb; 247(2):357-64.AnnS

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).

SUMMARY BACKGROUND DATA

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.

PATIENTS AND 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.

CONCLUSION

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+Show Affiliations

Department of General, Visceral and Transplantation Surgery, Charité, Campus Virchow Clinical Centre, Berlin, Germany. frank.ulrich@charite.deNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article

Language

eng

PubMed ID

18216545

Citation

Ulrich, Frank, et al. "Long-term Outcome of Liver Resection and Transplantation for Caroli Disease and Syndrome." Annals of Surgery, vol. 247, no. 2, 2008, pp. 357-64.
Ulrich F, Pratschke J, Pascher A, et al. Long-term outcome of liver resection and transplantation for Caroli disease and syndrome. Ann Surg. 2008;247(2):357-64.
Ulrich, F., Pratschke, J., Pascher, A., Neumann, U. P., Lopez-Hänninen, E., Jonas, S., & Neuhaus, P. (2008). Long-term outcome of liver resection and transplantation for Caroli disease and syndrome. Annals of Surgery, 247(2), 357-64. https://doi.org/10.1097/SLA.0b013e31815cca88
Ulrich F, et al. Long-term Outcome of Liver Resection and Transplantation for Caroli Disease and Syndrome. Ann Surg. 2008;247(2):357-64. PubMed PMID: 18216545.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Long-term outcome of liver resection and transplantation for Caroli disease and syndrome. AU - Ulrich,Frank, AU - Pratschke,Johann, AU - Pascher,Andreas, AU - Neumann,Ulf P, AU - Lopez-Hänninen,Enrique, AU - Jonas,Sven, AU - Neuhaus,Peter, PY - 2008/1/25/pubmed PY - 2008/3/14/medline PY - 2008/1/25/entrez SP - 357 EP - 64 JF - Annals of surgery JO - Ann Surg VL - 247 IS - 2 N2 - 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). SUMMARY BACKGROUND DATA: 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. PATIENTS AND 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. CONCLUSION: 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. SN - 0003-4932 UR - https://www.unboundmedicine.com/medline/citation/18216545/Long_term_outcome_of_liver_resection_and_transplantation_for_Caroli_disease_and_syndrome_ L2 - https://Insights.ovid.com/pubmed?pmid=18216545 DB - PRIME DP - Unbound Medicine ER -