Hu Yue, Deng Zaichun, Chen Yongbin, Wu Tingting
Department of Respiratory, Beilun People's Hospital, No.1288, Lushan East Road, Ningbo, 315800, Zhejiang, China.
Department of Respiratory and Critical Care Medicine, Key Laboratory of Respiratory Disease of Ningbo, The First Affiliated Hospital of Ningbo University, 247 Renmin Road, Ningbo, 315020, Zhejiang, China.
BMC Pulm Med. 2025 Apr 23;25(1):193. doi: 10.1186/s12890-025-03666-1.
Budd-Chiari syndrome (BCS) is a hepatic venous system disease caused by obstruction of the hepatic blood flow outflow tract. The definition of hemothorax is that blood accumulates in the chest cavity, and the hematocrit value of the effusion exceeds 50%. Hemothorax caused by intrathoracic variceal rupture associated with BCS is rare.
A 43-year-old female patient with just 69 g/L hemoglobin, complaining of shortness of breath for 2 days, was admitted to gastroenterology department. The chest computed tomography (CT) revealed right pleural effusion and contrast-enhanced CT in portal venous phase revealed portal hypertension and multiple tortuous veins. The ratio of red blood cells to white blood cells in bloody pleural effusion was about 500:1, and the neuron-specific enolase (NSE) and cytokeratin 19 fragment antigen 21 - 1 (CYFRA21-1) in the pleural effusion were significantly increased. Therefore, the patient was transferred to the respiratory medicine department to exclude malignant pleural effusion. The enhanced chest CT reexamination showed a continuous enhanced soft tissue-like lump in the thoracic cavity, which was a varicose vein. The vascular interventional physician reviewed the contrast-enhanced CT in portal venous phase to see a stenosis between the hepatic vein and the inferior vena cava, so BCS was suspected. Vascular interventional surgery was performed, and identified obstructed blood flow at the upper end of the inferior vena cava, which significantly improved after thrombolysis. Therefore, the intrathoracic variceal rupture linked to BCS was the source of the patient's pleural effusion.
when there is unexplained bloody pleural effusion and the tumor index of pleural effusion increases, thoracoscopic pleural biopsy should not be blindly performed, and pleural effusion caused by vascular rupture should be further excluded.
布加综合征(BCS)是一种由肝血流流出道梗阻引起的肝静脉系统疾病。血胸的定义是血液积聚在胸腔内,且胸腔积液的血细胞比容值超过50%。由与BCS相关的胸内静脉曲张破裂引起的血胸较为罕见。
一名血红蛋白仅为69g/L的43岁女性患者,因气短2天入住消化内科。胸部计算机断层扫描(CT)显示右侧胸腔积液,门静脉期增强CT显示门静脉高压和多条迂曲静脉。血性胸腔积液中红细胞与白细胞的比例约为500:1,胸腔积液中的神经元特异性烯醇化酶(NSE)和细胞角蛋白19片段抗原21-1(CYFRA21-1)显著升高。因此,患者被转至呼吸内科以排除恶性胸腔积液。胸部增强CT复查显示胸腔内有一个持续强化的软组织样肿块,为曲张静脉。血管介入医生查看门静脉期增强CT,发现肝静脉与下腔静脉之间有狭窄,故怀疑为BCS。进行了血管介入手术,发现下腔静脉上端血流受阻,溶栓后明显改善。因此,与BCS相关的胸内静脉曲张破裂是患者胸腔积液的来源。
当出现不明原因的血性胸腔积液且胸腔积液肿瘤指标升高时,不应盲目进行胸腔镜胸膜活检,应进一步排除血管破裂引起的胸腔积液。