Perihilar Cholangiocarcinoma

Perihilar cholangiocarcinomas (h-CCA) are defined as tumors located in the extrahepatic biliary tree proximal to the origin of the cystic duct. Their most common presentation is jaundice associated with weight loss. Imaging workup should be done before biliary drainage, preferably in specialized centers. Nodal (outside the hepatoduodenal ligament) or distant metastasis should preclude resection as well as significant baseline comorbidities. Biliary drainage is a controversial topic. It is mandatory in patients with cholangitis, and should be considered in patients with patients with less than 50% of future liver remnant, or in those who are going to be submitted to portal vein embolization. The Bismuth-Corlette classification indicates the proximal extent of biliary involvement and is used along with the detailed study of vascular compromise to plan the resection. The surgical techniques for resecting h-CCA includes hepatic hilum lymphadenectomy, extended parenchyma resections, resection of the caudate lobe and Intrahepatic biliary reconstruction. Due to the high risk of fistula, an abdominal drain is placed close to the hepaticojejunal anastomosis. Surgery for h-CCA has high rates of morbidity and mortality, and both the preoperative and the postoperative management are highly specialized and require intensive multidisciplinary support.