Caroli's Disease: Report of Surgical Options and Long-Term Outcome of Patients Treated in Argentina. Multicenter Study

Javier C. Lendoire, Gabriel Raffin, Jorge Grondona, Ricardo Bracco, Rodolfo Russi, Victoria Ardiles, Gabriel Gondolesi, Jorge Defelitto, Eduardo de Santibañes, Oscar Imventarza

Research output: Contribution to journalArticlepeer-review

18 Scopus citations

Abstract

Background: Caroli's disease (CD) management is still controversial. Aim: The purpose of this study is to report the most frequent clinical features, treatment options, and outcome obtained after surgical management of CD. Methods: A voluntary survey was conducted. Demographic, clinical, surgical, and pathological variables were analyzed. Results: Six centers included 24 patients having received surgical treatment from 1991 to 2009. Seventeen (70. 8%) patients were female, with average age of 48. 7 years old (20-71), and 95. 5% were symptomatic. There was left hemiliver involvement in 75% of the patients. Surgical procedures included nine left lateral sectionectomies, eight left hepatectomies, and four right hepatectomies for those with hemiliver disease, while for patients with bilateral disease, one right hepatectomy and two Roux-en-Y hepaticojejunostomies were performed. The average length of hospitalization was 7 days. For perioperative complications (25%), three patients presented minor complications (types 1-2), while major complications occurred in three patients (type 3a). No mortality was reported. After a median follow-up of 166 months, all patients are alive and free of symptoms. CD diagnosis was confirmed by histology. Congenital hepatic fibrosis was present in two patients (8. 3%) and cholangiocarcinoma in one (4. 2%). Conclusions: CD in Argentina is more common in females with left hemiliver involvement. Surgical resection is the best curative option in unilateral disease, providing long-term survival free of symptoms and complications. In selected cases of bilateral disease without parenchymal involvement, hepaticojejunostomy should be proposed. However, a close follow-up is mandatory because patients might progress and a transplant should be indicated.

Original languageEnglish
Pages (from-to)1814-1819
Number of pages6
JournalJournal of Gastrointestinal Surgery
Volume15
Issue number10
DOIs
StatePublished - Oct 2011
Externally publishedYes

Keywords

  • Biliary cyst
  • Caroli's disease
  • Hepaticojejunostomy
  • Liver cyst
  • Liver resection

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