title: Caroli's disease: report of surgical options and long-term outcome of patients treated in Argentina. Multicenter study.
abstract: BACKGROUND
  Caroli's disease (CD) management is still controversial.
  
  
  OBJECTIVE
  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.
authors.affiliation: Hospital Dr. Cosme Argerich, Buenos Aires, Argentina. jlendoire@yahoo.com.ar
authors.forename: Javier C
authors.initials: JC
authors.lastname: Lendoire
authors.forename: Gabriel
authors.initials: G
authors.lastname: Raffin
authors.forename: Jorge
authors.initials: J
authors.lastname: Grondona
authors.forename: Ricardo
authors.initials: R
authors.lastname: Bracco
authors.forename: Rodolfo
authors.initials: R
authors.lastname: Russi
authors.forename: Victoria
authors.initials: V
authors.lastname: Ardiles
authors.forename: Gabriel
authors.initials: G
authors.lastname: Gondolesi
authors.forename: Jorge
authors.initials: J
authors.lastname: Defelitto
authors.forename: Eduardo
authors.initials: E
authors.lastname: de Santibañes
authors.forename: Oscar
authors.initials: O
authors.lastname: Imventarza
mesh_terms: D000328:Adult; D000368:Aged; D001118:Argentina; D016767:Caroli Disease / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D005260:Female; D006498:Hepatectomy; D006801:Humans; D007582:Jejunostomy; D007902:Length of Stay; D008297:Male; D008875:Middle Aged; D018579:Patient Selection; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult