Sternotomy and extracorporal circulation for fulminant Budd-Chiari syndrome due to leiomyosarcoma of the inferior vena cava.

IF 3.2 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Cardio-oncology Pub Date : 2024-11-25 DOI:10.1186/s40959-024-00287-1

Maciej Wiewiora, Hanna Wiewiora, Ewa Chmielik, Michal Jarzab, Michael Grynkiewicz, Marcin Kubeczko

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Abstract

Background: Budd-Chiari syndrome is a rare and severe vascular liver disease. We presented patient with fulminant liver failure secondary to leiomyosarcoma of the IVC and thrombosis.

Case presentation: A 44-year-old female presented with fulminant liver failure secondary to inferior vena cava (IVC) thrombosis. Contrast-enhanced computed tomography subsequently revealed a thrombus within the IVC, extending cranially to the right atrium and caudally to the renal veins. The patient's condition, characterized by early comatose symptoms, necessitated surgical intervention. Under extracorporeal circulation, a right atriotomy with thrombus lesion removal and descending thrombectomy of the IVC was performed. Hepatic congestion resolved after the thrombus was removed. A pathological examination of the excised thrombus revealed the presence of high-grade leiomyosarcoma.

Conclusions: In cases where a thrombus extends from the IVC to the right atrium, urgent surgical intervention with extracorporeal circulation should be considered.

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下腔静脉利肌肉瘤导致的暴发性巴德-卡里综合征的截流术和椎体外循环。

背景：巴德-恰里综合征是一种罕见的严重血管性肝病：布德-恰里综合征是一种罕见的严重血管性肝病。我们接诊了一名继发于下腔静脉癌和血栓形成的暴发性肝衰竭患者：一名 44 岁女性因下腔静脉（IVC）血栓形成而出现暴发性肝衰竭。随后，对比增强计算机断层扫描显示，下腔静脉内存在血栓，血栓头端延伸至右心房，尾端延伸至肾静脉。患者的病情以早期昏迷症状为特征，因此有必要进行手术治疗。在体外循环下，进行了右心房切开术，切除了血栓病灶，并对 IVC 进行了降支血栓切除术。血栓取出后，肝充血症状缓解。对切除的血栓进行病理检查后发现，存在高分化腺癌：结论：当血栓从内静脉延伸至右心房时，应考虑使用体外循环进行紧急手术治疗。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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