BACKGROUND Marfan Syndrome (MFS) is a heritable connective tissue disorder with a high degree of clinical variability including respiratory diseases; a rare case of MFS with massive intrathoracic bleeding has been reported recently. CASE PRESENTATION A 32-year-old man who had been diagnosed with MFS underwent a Bentall operation with artificial valve replacement for aortic dissection and regurgitation of an aortic valve in 2012. Warfarin was started postoperatively, and the dosage was gradually increased until 2017, when the patient was transported to our hospital due to sudden massive haemoptysis. Computed tomography (CT) with a maximum intensity projection (MIP) revealed several giant pulmonary cysts with fluid levels in the apex of the right lung with an abnormal vessel from the right subclavian artery. Transcatheter arterial embolization was performed with angiography and haemostasis was achieved, which suggested that the bleeding vessel was the lateral thoracic artery (LTA) branch. CT taken before the incident indicated thickening of the cystic wall adjacent to the thorax; therefore, it was postulated that the bleeding originated from fragile anastomoses between the LTA and pulmonary or bronchial arteries. It appears that the vessels exhibited inflammation that began postoperatively, which extended to the cysts. CONCLUSION We experienced a case of MFS with massive haemoptysis from the right LTA. We have to be aware of the possibility that massive haemoptysis could be induced in MFS with inflamed pulmonary cysts.

中文翻译：

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一例马凡氏综合症伴有胸腔外侧动脉大量咯血。

背景技术马凡氏综合症（MFS）是一种遗传性结缔组织疾病，具有高度的临床变异性，包括呼吸系统疾病；最近报道了罕见的MFS伴大量胸腔内出血。病例介绍2012年，一名32岁的被诊断患有MFS的男子接受了Bentall手术，其中包括人工瓣膜置换术以进行主动脉夹层和主动脉瓣反流。华法林术后开始使用，剂量逐渐增加，直到2017年，该患者因突然发生大量咯血而被送往我们医院。具有最大强度投影（MIP）的计算机断层扫描（CT）显示，右侧肺尖中有数个巨大的肺囊肿，右肺尖有积液，右锁骨下动脉血管异常。经血管造影术经导管动脉栓塞并止血，这表明出血血管是胸外侧动脉（LTA）分支。事发前进行的CT检查表明，邻近胸腔的囊性壁增厚。因此，推测出血是由于LTA与肺或支气管动脉之间的脆弱吻合引起的。看来血管表现出从术后开始的炎症，延伸到囊肿。结论我们从正确的LTA经历了一例MFS伴有大量咯血的病例。我们必须意识到在发炎性肺囊肿的MFS中可能诱发大量的咯血的可能性。这表明出血血管是胸外侧动脉（LTA）分支。事发前进行的CT检查表明，邻近胸腔的囊性壁增厚。因此，推测出血是由于LTA与肺或支气管动脉之间的脆弱吻合引起的。看来血管表现出从术后开始的炎症，延伸到囊肿。结论我们从正确的LTA经历了一例MFS伴有大量咯血的病例。我们必须意识到在发炎性肺囊肿的MFS中可能诱发大量的咯血的可能性。这表明出血血管是胸外侧动脉（LTA）分支。事发前进行的CT检查表明，邻近胸腔的囊性壁增厚。因此，推测出血是由于LTA与肺或支气管动脉之间的脆弱吻合引起的。看来血管表现出从术后开始的炎症，并延伸至囊肿。结论我们从正确的LTA经历了一例MFS伴有大量咯血的病例。我们必须意识到在发炎性肺囊肿的MFS中可能诱发大量的咯血的可能性。据推测，出血是由于LTA与肺或支气管动脉之间的脆弱吻合引起的。看来血管表现出从术后开始的炎症，延伸到囊肿。结论我们从正确的LTA经历了一例MFS伴有大量咯血的病例。我们必须意识到在发炎性肺囊肿的MFS中可能诱发大量的咯血的可能性。据推测，出血是由于LTA与肺或支气管动脉之间的脆弱吻合引起的。看来血管表现出从术后开始的炎症，延伸到囊肿。结论我们从正确的LTA经历了一例MFS伴有大量咯血的病例。我们必须意识到在发炎性肺囊肿的MFS中可能诱发大量的咯血的可能性。