Laparoscopic treatment of Caroli’s disease

Andrea Ruzzenente, Laura Alaimo, Simone Conci, Fabio Bagante, Tommaso Campagnaro, Andrea Ciangherotti, Alfredo Guglielmi

Abstract

Caroli’s disease is a rare hepatobiliary disorder characterized by malformations of the intrahepatic medium and large bile ducts, resulting in non-obstructive ductal dilatation, with focal or multifocal segmental involvement of the liver. It is often associated with hepatolithiasis and acute cholangitis. The curative treatment of the localized form is surgical liver resection involving any pathologic bile duct tissue, in order to avoid the development of recurrent disease and life-threatening complications. We have reviewed the literature during the period 2005–2018 concerning the surgical treatment of Caroli’s disease (including 243 liver resections), in order to evaluate the impact of laparoscopic surgery, its technical aspects, feasibility, safety, short- and long-term outcomes. Only 6 reports describing 16 cases available in the most recent literature (years 2009–2018) reported laparoscopic liver resections (7 left lateral hepatic sectionectomies, 7 left hepatectomies, 1 segmentectomy, 1 right anterior sectionectomy). Only 1 conversion to open surgery and 1 complication (a fluid collection) treated conservatively were described. Few data have been reported about the long-term post-operative follow-up. Caroli’s disease is a rare condition with variable clinical complexity, due to the extension of the disease, the localization of pathological bile ducts and the presence of complications. Radical surgery is the treatment of choice for localized disease and in these cases minimally invasive liver resection can have an important therapeutic role, particularly for left located Caroli’s disease. Minimally invasive approach to Caroli’s disease requires high expertise in liver surgery and in complex biliary procedures. Data of the literature are still lacking in long term results and more studies are necessary to confirm the short and long-term results.