A 65-year-old woman with no previous medical conditions presented with jaundice. Biological tests showed severe cholestasis. Abdominal ultrasound revealed intrahepatic bile duct dilatation and a flat gallbladder. Abdominal computed tomography (CT) scan and magnetic resonance cholangiopancreatography (MRCP) showed a 30 mm tumor originating from the biliary convergence suggestive of perihilar cholangiocarcinoma (Fig. 1). The tumor was mainly left sided involving the confluence of the left lateral sectional bile ducts (B2, 3) and left medial sectional branches (B4). On the right side, the tumor reached the confluence of the right anterior and posterior sectoral branches. The confluence of segment 6 (B6) and segment 7 (B7) bile ducts was free from tumor invasion. Concerning vascular involvement, there was an anatomical variation with a common hepatic artery emerging from the superior mesenteric artery. The left hepatic artery was totally included in the tumor. The right hepatic artery had an intratumoral course over a length of 10 mm with slight stenosis. The bifurcation between the right anterior and right posterior sectoral arteries was tumor-free. The portal vein presented a modal distribution; its left branch was invaded by the tumor, resulting in total left hepatic atrophy. The tumor extended to the portal vein bifurcation, but the right axis remained patent. The right anterior and right posterior portal bifurcation remained tumor free. Chest CT showed no pulmonary extension.
This PHC was classified as Bismuth IV and type X of Rennes [2]. It was decided to perform left trisectionectomy, caudate lobectomy and resection of the main bile duct with simultaneous resection of the portal vein and right hepatic artery. A percutaneous transhepatic biliary drain was inserted into the right posterior sectoral bile duct for external drainage. Two weeks later, jaundice disappeared, and the bilirubin level was 25 µmol//l. There was no sign of infection, and the nutritional status of the patient was adequate. The future remnant liver volume (right posterior sector) was 40% of the total liver volume.
Intraoperative exploration using visualization, palpation and intraoperative color-Doppler ultrasound (IOUS) confirmed the preoperative findings. Liver parenchyma transection was performed under intermittent pedicle and vena cava clamping of 26 min in total. The hepatic artery was reconstructed using an autologous saphenous vein graft interposed between the right posterior sectoral artery and the common hepatic artery. The reconstruction lasted 32 min. Portal vein reconstruction was performed with end-to-end anastomosis between the main portal vein and the right posterior sectoral vein, while the liver remained arterialized. IOUS confirmed the patency of the reconstructed vessels and the adequate vascularization of the right posterior segments. Intraoperative frozen section analysis of B6, 7 confluence revealed that margins were positive, imposing a more proximal section. B6 and B7 were then separated. Biliary reconstruction using the Roux-en-Y jejunum limb was performed over the two separate segmental bile ducts. Transanastomotic drainage of both ducts was performed according to Volker's technique.
The operative time and intraoperative blood loss were 365 min and 800 ml, respectively. Postoperative outcomes were mainly marked by the occurrence of bilioma treated with cutaneous drainage and antibiotics. The patient was discharged on postoperative day 32.
Macroscopic analysis of the specimen showed a sclerosing tumor that adhered to both the portal vein and right hepatic artery. Histological examination showed moderately differentiated biliary adenocarcinoma invading surrounding adipose tissue. However, no microscopic invasion of the portal vein or of the right hepatic artery was revealed. The distance between the tumor and the wall of the portal vein was 0.41 mm (Fig. 2). Perineural invasion was present. Three lymph nodes were found in the lymphadenectomy specimen and were tumor free. All margins were negative. Eventually, the tumor was classified as pT2aN0M0, R0, or stage II according to the TNM staging of perihilar bile duct tumors (AJCC 8th edition).
The patient was included in the French phase III randomized trial PRODIGE 12 and received 12 cycles of adjuvant gemcitabine-oxaliplatin (GEMOX). She is now healthy with no sign of recurrence 8 years after surgery.