Secondary Budd-Chiari syndrome occurred after adjuvant radiotherapy for perihilar cholangiocarcinoma: a case report

Abstract

Background: Budd-Chiari syndrome (BCS) is a rare vascular disorder of the liver, and acute and secondary BCS is even rarer.

Case presentation: A 62-year-old man with perihilar cholangiocarcinoma of Bismuth type IIIa underwent right hemi-hepatectomy with caudate lobectomy and pancreatoduodenectomy. Adjuvant chemoradiotherapy was performed due to a positive hepatic ductal margin. Subsequently, the disease passed without recurrence. The patient visited for acute onset abdominal pain at the 32nd postoperative month. Multidetector-row computed tomography (MDCT) showed stenosis of the left hepatic vein (LHV) root, which was the irradiated field, and thrombotic occlusion of the LHV. The patient was diagnosed with acute BCS caused by adjuvant radiotherapy. Although anticoagulation therapy was performed, the patient complained of sudden upper abdominal pain again. MDCT showed an enlarged LHV thrombus and hepatomegaly. The patient was diagnosed with exacerbated acute BCS, and stenting for the stenotic LHV root was performed with a bare stent. Although stenting for the LHV root was very effective, restenosis occurred twice due to thrombus in the existing stent, so re-stenting was performed twice. The subsequent clinical course was acceptable without recurrence or restenosis of the LHV root as of 6 months after the last stenting using a stent graft.

Conclusion: Although no case of BCS caused by radiotherapy has yet been reported, the present case showed that late side effect of radiotherapy can cause hepatic vein stenosis and secondary BCS.

Keywords: Adjuvant radiotherapy; Budd–Chiari syndrome; Radiation-induced stenosis of the hepatic vein; Stenosis of the hepatic vein; Stenting for the hepatic vein; The side effect of radiation.

Fig. 1

Radiotherapy planning summary. The root of the left hepatic vein was included in the irradiated field

Fig. 2

Multidetector-row computed tomography findings at the 32nd postoperative month. (before treatment for Budd-Chiari syndrome). a, b, c Yellow arrowheads show thrombotic occlusion of the LHV. d Massive ascites was confirmed

Fig. 3

Multidetector-row computed tomography findings of the gradual narrowing process of the left hepatic vein. Yellow arrowheads show the root of the left hepatic vein. a The 3rd postoperative month. b The 18th postoperative month. c The 24th postoperative month. d The 30th postoperative month

Fig. 4

a An overview of the postoperative clinical course. b A time series showing multidetector-row computed tomography findings, the course of treatment, and the changes in serum AST and ALT levels. b-1 Before the anticoagulation therapy. Yellow arrowheads show thrombotic occlusion of the LHV. b-2 After the anticoagulation therapy. Yellow arrowheads show improvement in blood flow of the LHV. b-3 Before the first endovascular intervention. Yellow arrowheads show thrombotic occlusion of the LHV. Hepatic congestion in the drainage area of the occluded LHV can be identified. b-4 After the first endovascular intervention. Yellow arrowheads show improvement of blood flow of the LHV. b-5 Before the second endovascular intervention. Contrast effects of the LHV were not observed. b-6 Before the third endovascular intervention. Contrast effects of the LHV were not observed. b-7 After the third endovascular intervention. Yellow arrowheads show improvement of blood flow of the LHV. POM, postoperative month; LHV, left hepatic vein; AST, aspartate aminotransferase; ALT, alanine aminotransferase

Fig. 5

Findings of endovascular intervention. a The first time of intervention. Angiography of the V2 showing that the hepatic vein is completely occluded, with only collateral veins were contrasted (a-1). Improvement of blood flow of the LHV to IVC was confirmed after deployment of the LHV stent (a-2). b The second intervention. Angiography of the V2 showed no contrast effect from the V2 to IVC. (b-1) Improvement of the blood flow of the LHV to IVC was confirmed after deployment of the LHV stent (stent-in-stent) (b-2). b The third intervention. Angiography of the V2 showed marked stenosis in the existing stent (c-1). Improvement of the blood flow of the LHV to IVC was confirmed after deployment of the LHV covered stent (c-2). V2, hepatic vein of the segment 2; LHV, left hepatic vein; IVC, inferior vena cava