TY - JOUR
T1 - Caroli's Disease
T2 - Report of Surgical Options and Long-Term Outcome of Patients Treated in Argentina. Multicenter Study
AU - Lendoire, Javier C.
AU - Raffin, Gabriel
AU - Grondona, Jorge
AU - Bracco, Ricardo
AU - Russi, Rodolfo
AU - Ardiles, Victoria
AU - Gondolesi, Gabriel
AU - Defelitto, Jorge
AU - de Santibañes, Eduardo
AU - Imventarza, Oscar
PY - 2011/10
Y1 - 2011/10
N2 - 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.
AB - 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.
KW - Biliary cyst
KW - Caroli's disease
KW - Hepaticojejunostomy
KW - Liver cyst
KW - Liver resection
UR - http://www.scopus.com/inward/record.url?scp=80053112322&partnerID=8YFLogxK
U2 - 10.1007/s11605-011-1620-9
DO - 10.1007/s11605-011-1620-9
M3 - Article
C2 - 21796462
AN - SCOPUS:80053112322
SN - 1091-255X
VL - 15
SP - 1814
EP - 1819
JO - Journal of Gastrointestinal Surgery
JF - Journal of Gastrointestinal Surgery
IS - 10
ER -