背景:肺动静脉畸形(PAVM),也被称为肺动静脉瘘,是一种罕见的血管发育异常。大多数PAVM病例与遗传性出血性毛细血管扩张症(HHT)有关。与PAVM相关的血胸甚至更罕见,有关这一并发症的管理仍然面临挑战。
方法:我院收治一名突然出现呼吸困难和胸痛的55岁男性患者。他有鼻出血病史,腹膜内生殖细胞肿瘤和PAVM。胸部未增强CT显示左侧胸腔积液伴部分被动肺不张,间隔6天逐渐增加。诊断性胸腔穿刺术进一步显示出血性积液。CT血管造影(CTA)显示左下肺动脉和PAVM的管腔弯曲扩张,并形成动脉瘤。由于他的家人拒绝手术,患者接受了经导管栓塞治疗.然而,左胸腔积液没有明显减少,即使在介入治疗后血红蛋白值也缓慢下降,表明持续活动性出血的可能性。最终,患者接受了左下叶肺叶切除术,结果令人满意。
结论:PAVM破裂进入胸膜腔引起的大量血胸可导致致命的结果。CTA可以准确诊断这种病理状况。经导管栓塞术常用于治疗PAVM,但在血胸患者中达到理想的效果可能是具有挑战性的。结合我们的案例和文献回顾,当PAVM并发血胸和大直径的引流静脉时,直接根治性手术可以导致成功的结果。
BACKGROUND: Pulmonary arteriovenous malformation (PAVM), also known as pulmonary arteriovenous fistula, is a rare vascular developmental anomaly. Most cases of PAVM are associated with hereditary hemorrhagic telangiectasia (HHT). Hemothorax associated with PAVM is even rarer, and management concerning this complication still challenges.
METHODS: A 55-year-old man with sudden onset of dyspnea and chest pain was admitted to our hospital. He had a medical history of epistaxis, intraperitoneal germ cell tumor and PAVM. Chest unenhanced CT revealed the left-sided pleural effusion together with partial passive atelectasis and gradual increase at the interval of six days. Diagnostic thoracocentesis further revealed hemorrhagic effusion. CT angiography (CTA) showed tortuously dilated lumen of the left lower pulmonary artery and PAVM with the formation of aneurysm. Due to his family\'s refusal of surgery, the patient underwent transcatheter embolization therapy. However, the left pleural effusion did not significantly reduce and there was a slow drop in hemoglobin value even after interventional treatment, indicating the possibility of ongoing active bleeding. Eventually, the patient received lobectomy of the left lower lobe with a satisfactory outcome.
CONCLUSIONS: Massive hemothorax resulting from PAVM rupture into the pleural space can lead to fatal outcomes. CTA can accurately diagnose this pathologic condition. Transcatheter embolization is frequently used in the treatment of PAVM, but it may be challenging to achieve the desirable effect in patients with hemothorax. Combined with our
case and literature review, direct radical surgery can lead to a successful outcome when PAVM complicated with hemothorax and a large diameter of the draining vein.