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Resume Introduction La maladie de Caroli (MC) est une dilatation congenitale intrahepatique des voies biliaires. Elle est appelee syndrome de Caroli (SC) quand s’y associe une fibrose hepatique ou une cirrhose. Les infections et le cholangiocarcinome en sont les principales complications secondaires. Le but de cette etude etait d’analyser les aspects cliniques et les resultats du traitement chirurgical des patients ayant une MC ou un SC. Methodes De janvier 2004 a decembre 2016, 21 patients ayant une MC/SC ont ete traites par resection hepatique (RH) ou transplantation hepatique (TH). Les donnees pre- et postoperatoires etaient recueillies a partir d’une base de donnees. Resultats Deux patients ont eu une TH et 19 patients une RH pour MC ou SC. Au cours du suivi, un patient a developpe un cancer du poumon 9 ans apres TH. Les patients ayant eu une RH etaient surtout des femmes (74 %) avec une comorbidite faible. Le score median de Clavien-Dindo etait de 1 (extremes 0–3). Aucun deces n’est survenu au cours des 5 ans de suivi. Un cholangiocarcinome etait retrouve chez 4 patients. Une recidive tumorale est survenue chez 3 de ces patients, elle a ete traitee par chimiotherapie ou chirurgie iterative. Conclusions Les TH ou RH pour MC/SC sont relativement rares et associees a une faible morbi-mortalite postoperatoires. La chirurgie devrait etre proposee le plus tot possible afin d’eviter les angiocholites recidivantes et l’evolution vers un cholangiocarcinome.

Summary Introduction Caroli disease (CD) is a congenital dilatation of the intrahepatic bile ducts. In combination with liver fibrosis or cirrhosis, it is called Caroli syndrome (CS). Infectious complications and intrahepatic cholangiocarcinoma are secondary problems. The aim of this study was to analyse the clinical pattern and outcome in patients with CD/CS who underwent liver surgery. Methods Between January 2004 and December 2016, 21 patients with CD/CS were treated with liver resection or transplantation (LTX) and post-operative data of patients with CD/CS were retrospectively analysed in a database. Results Two patients underwent LTX, and 19 patients underwent liver resection due to CD/CS. During follow-up, one patient developed lung cancer nine years after LTX. Patients resected due to CD/CS were predominantly females (74%) with an overall low incidence of co-morbidities. The median post-operative Clavien-Dindo score was 1 (range: 0–3). There was no death during a median follow-up period of over five years. In four patients, cholangiocarcinoma was confirmed. Tumor recurrence was seen in three patients, and was treated with chemotherapy or repeated liver resection. Conclusions LTX and liver resections due to CD/CS are rare and associated with an acceptable post-operative morbidity and low mortality. Surgical treatment should be performed as early as possible to avoid recurrent episodes of cholangitis or carcinogenesis.