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

  • Javier C. LendoireEmail author
  • Gabriel Raffin
  • Jorge Grondona
  • Ricardo Bracco
  • Rodolfo Russi
  • Victoria Ardiles
  • Gabriel Gondolesi
  • Jorge Defelitto
  • Eduardo de Santibañes
  • Oscar Imventarza
Original Article

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.

Keywords

Liver cyst Biliary cyst Caroli’s disease Liver resection Hepaticojejunostomy 

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Copyright information

© The Society for Surgery of the Alimentary Tract 2011

Authors and Affiliations

  • Javier C. Lendoire
    • 1
    Email author
  • Gabriel Raffin
    • 1
  • Jorge Grondona
    • 2
  • Ricardo Bracco
    • 2
  • Rodolfo Russi
    • 3
  • Victoria Ardiles
    • 4
  • Gabriel Gondolesi
    • 5
  • Jorge Defelitto
    • 6
  • Eduardo de Santibañes
    • 4
  • Oscar Imventarza
    • 1
  1. 1.Liver Transplant UnitHospital Dr. Cosme ArgerichBuenos AiresArgentina
  2. 2.UNACIR HPBPcia. Buenos AiresArgentina
  3. 3.Hepato-Pancreato-Biliary Surgical UnitHospital Naval Pedro MalloBuenos AiresArgentina
  4. 4.Liver Transplant UnitHospital ItalianoBuenos AiresArgentina
  5. 5.Hepato-Biliary Surgery and Liver Transplant Unit, Fundación FavaloroBuenos AiresArgentina
  6. 6.Hepato-Pancreato-Biliary UnitInstituto del Diagnóstico de La PlataBuenos AiresArgentina

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