Department of Neurosurgery, College of Medicine, Kyung Hee University, Seoul, Korea.
Department of Radiology, College of Medicine, Kyung Hee University, Seoul, Korea.
World Neurosurg. 2019 Oct;130:157-159. doi: 10.1016/j.wneu.2019.07.007. Epub 2019 Jul 9.
Although vascular abnormality is an uncommon comorbidity of neurofibromatosis type 1 (NF1), it is potentially fatal. We present spontaneous hemothorax caused by rupture of a vertebral artery (VA) aneurysm in a patient with NF1.
A 36-year-old man with a history of NF1 was transferred to the emergency department with dyspnea. Chest computed tomography scan revealed hemothorax in the left lung field with mediastinal shifting and aneurysmal dilatation of the left VA at the C6 vertebra level. Immediate drainage of the hematoma by chest tube insertion was performed. Diagnostic angiogram showed a 3- to 4-cm fusiform aneurysm of the VA. After the angiogram, cardiopulmonary arrest occurred after a rebleed of the VA aneurysm. The aneurysmal segment of the VA was urgently occluded with detachable coils. Postoperatively, the patient was in intensive care for 1 month because of fulminant pneumonia. After the patient regained consciousness, he was found to have right hemiparesis from a small infarction at the pons. The patient's function improved to near normal after 1 year of recovery.
Hemothorax caused by VA rupture in a patient with NF1 is an extremely rare condition that can be fatal. Careful examination with suspicion for early detection and treatment is required for this urgent condition. Endovascular coiling was safe even for an unstable patient with massive bleeding.
尽管血管异常是神经纤维瘤病 1 型（NF1）的一种罕见合并症，但它可能是致命的。我们报告了一例 NF1 患者因椎动脉（VA）动脉瘤破裂导致自发性血胸的病例。
一名 36 岁男性，有 NF1 病史，因呼吸困难转入急诊科。胸部 CT 扫描显示左侧肺野血胸，纵隔移位，C6 椎体水平左侧 VA 呈动脉瘤样扩张。立即行胸腔引流管插入以排出血肿。诊断性血管造影显示 VA 呈 3 至 4cm 梭形动脉瘤。血管造影后，VA 动脉瘤再次出血导致心跳骤停。紧急用可解脱弹簧圈闭塞 VA 的动脉瘤段。术后，患者因暴发性肺炎在重症监护病房接受了 1 个月的治疗。患者意识恢复后，发现由于脑桥小梗死导致右侧偏瘫。经过 1 年的恢复，患者的功能接近正常。
NF1 患者 VA 破裂导致血胸是一种极其罕见的情况，可能是致命的。对于这种紧急情况，需要仔细检查并怀疑早期发现和治疗。即使对于大量出血不稳定的患者，血管内弹簧圈栓塞也是安全的。
