Wiewiora Maciej, Wiewiora Hanna, Chmielik Ewa, Jarzab Michal, Grynkiewicz Michael, Kubeczko Marcin
Department of Cardiac Vascular and Endovascular Surgery and Transplantology, Faculty of Medical Sciences in Zabrze, Medical University of Silesia in Katowice, ul. Sklodowskiej-Curie 9, Zabrze, 41-800, Poland.
Student Scientific Society, Department of Cardiac, Vascular and Endovascular Surgery and Transplantology, Faculty of Medical Sciences in Zabrze, Sklodowskiej-Curie 9, Zabrze, 41-800, Poland.
Cardiooncology. 2024 Nov 25;10(1):83. doi: 10.1186/s40959-024-00287-1.
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.
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.
In cases where a thrombus extends from the IVC to the right atrium, urgent surgical intervention with extracorporeal circulation should be considered.
布加综合征是一种罕见且严重的肝脏血管疾病。我们报告了一例继发于下腔静脉平滑肌肉瘤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）血栓形成继发暴发性肝衰竭就诊。对比增强计算机断层扫描随后显示IVC内有血栓，向上延伸至右心房，向下延伸至肾静脉。患者病情以早期昏迷症状为特征，需要手术干预。在体外循环下，进行了右心房切开术以清除血栓病变并对IVC进行降段血栓切除术。血栓清除后肝脏充血缓解。对切除的血栓进行病理检查发现存在高级别平滑肌肉瘤。
在血栓从IVC延伸至右心房的病例中，应考虑在体外循环下进行紧急手术干预。
