A 62-year-old man with perihilar cholangiocarcinoma of Bismuth type IIIa underwent right hemi-hepatectomy with caudate lobectomy and pancreatoduodenectomy. After management of postoperative complications of pancreatic fistula and refractory ascites, the patient was discharged on the 45th postoperative day. Although the resected specimen was grossly completely resected, microscopic findings revealed invasive carcinoma at the hepatic ductal margin. Therefore, adjuvant chemoradiotherapy with S-1 (S-1 80 mg/m2/day with administered total 28 days with 50.4 Gy/28 Fr) has performed from the third month after the operation (Fig. 1). Additional adjuvant chemotherapy with S-1 (S-1 80 mg/m2/day administered on days 1–28 every 6 weeks) was performed for 3 cycles, and the patient had no recurrence after surgery .
The patient visited for acute onset abdominal distension and epigastric pain at the 32nd postoperative month. Elevated levels of serum aspartate aminotransferase and alanine aminotransferase were confirmed (Table. 1), and multidetector-row computed tomography (MDCT) showed massive ascites and a contrast defect of the left hepatic vein (LHV) (Fig. 2). Cytology of ascites was negative for cancer and the serum-ascites albumin gradient was 1.3 g/dL (Table. 1), suggesting the presence of portal hypertension due to hepatic congestion. Focusing on the LHV root, the part was gradually narrowed compared with the findings of MDCT before adjuvant chemoradiotherapy (Fig. 3). As the LHV root was included in the scope of radiotherapy (irradiated with about 46 Gy) (Fig. 1) and there were no surgical procedures that could have resulted in LHV root stenosis, it was determined that late side effects of radiotherapy had caused the LHV thrombotic occlusion. The diagnosis was therefore acute and secondary BCS caused by adjuvant radiotherapy.
Figure 4 shows the treatment course for BCS. Anticoagulation therapy with heparin and administration of antithrombin III, which was insufficient, was started for reperfusion of the LHV. A slight reduction in the thrombus in the hepatic vein of the segment 2 (V2) was observed (Fig. 4b), and blood tests showed improvement of liver damage, with the ascites controlled with diuretics. However, after the 18th day of anticoagulation, the patient complained of sudden upper abdominal pain again, and the levels of serum AST and ALT were re-elevated (Fig. 4). Furthermore, the prothrombin % activity fell to 8% due to progressive liver failure. MDCT showed an enlarged LHV thrombus and a congested and swollen lateral segment of the liver (Fig. 4c).
The patient was diagnosed with exacerbated acute BCS that might lead to fatal liver failure, so stenting was planned for the stenotic LHV root by endovascular intervention after correction of the severe hypocoagulable state by administration of 40 units of fresh-frozen plasma. Right internal jugular vein catheterization was performed under local anesthesia using the Seldinger technique. A 5-Fr diagnostic catheter (cobra type and hook type) was inserted through a 6-Fr sheath. Because the approach failed to cannulate the LHV, we added a transhepatic approach using a 21-gauge needle and established a pull-through route. Angiography of the V2 revealed the hepatic vein to be completely occluded (Fig. 5a-1). An 8 × 40-mm bare stent (E-LUMINEXX®; Bard Peripheral Vascular, New Providence, NJ, USA) was successfully deployed (Fig. 5a-2), and the tract in the hepatic parenchyma was embolized with a mixture of N-butyl-2-cyanoacrylate (NBCA) (Histoacryl; B. Braun, Melsungen, Germany) and iodized oil (lipiodol; Guerbet, Tokyo, Japan) (NBCA:lipiodol, 1:1).
The blood flow of the LHV showed improvement (Fig. 4d), and the abdominal pain symptoms rapidly improved after deployment of the stent, implying that hepatic congestion had improved. The subsequent clinical course was good with anticoagulant therapy (30 mg/day of Edoxaban Tosilate Hydrate), and the patient was discharged 16 days after placement of the LHV stent.
One month after the placement of the LHV stent, the patient complained of sudden abdominal pain again. MDCT showed a loss of contrast effect in the LHV (Fig. 4e). It was considered that LHV thrombus occlusion had reoccurred, and a second angioplasty procedure was performed. An angiography of the V2 via the transjugular approach revealed no contrast effect from the V2 to the IVC (Fig. 5b-1). Although thrombolysis and balloon angioplasty were performed, the pressure gradient between the IVC and the LHV was still high. Another stent (E-LUMINEXX®; 10 × 40mm) was deployed in the existing stent, and the blood flow of the LHV showed improvement (Fig. 5b-2), while the pressure gradient was markedly decreased.
However, after the patient's condition had recovered, a rapid increase in ascites was confirmed two weeks later. MDCT revealed a loss of contrast effect in the LHV (Fig. 4f), and we determined that LHV thrombus occlusion had occurred a third time. Angiography of the V2 via the transjugular route revealed marked stenosis in the existing stent (Fig. 5c-1). A 10 × 50-mm stent graft (Viabahn®; W. L. Gore, Flagstaff, AZ, USA) was successfully deployed in the second stent (Fig. 5c-2), and the blood flow of the LHV showed improvement (Fig. 4g). The LHV pressure was dropped to the 20 mmHg from 60 mmHg. After stabilization of his condition, the patient was started on 100 mg/day of Aspirin and 60 mg/day of Edoxaban Tosilate Hydrate.
As of six months since the last stenting, the patient is alive without restenosis of the LHV or recurrence of cholangiocarcinoma.