PDF(Pubmed)
Abstract：
Neurofibromatosis type 1 (NF1) can cause vascular complications even in undiagnosed NF1 patients. A ruptured aneurysm of the branches of the subclavian artery is a rare but life-threatening event, and the hemorrhage can cause upper airway obstruction. We present a case of NF1 patient with a ruptured transverse cervical artery aneurysm, which led to a nearly obstructed airway. A 52-year-old man who was not previously diagnosed with NF1 presented with sudden pain from the left shoulder to the neck. Since childhood, he has had multiple cutaneous neurofibromas and café-au-lait macules, and freckling in the bilateral axillae. His swollen left side of the neck and left shoulder suggested a hematoma, which compressed the upper airway. Contrast-enhanced computed tomography revealed a cervical hematoma caused by a ruptured aneurysm of the transverse cervical artery. We performed awake fiberoptic intubation because a difficult airway was predicted and surgical airway management may have been impossible due to the anterior cervical hematoma. His airway was secured, and his aneurysm was successfully treated by coil embolization. Based on his cutaneous findings, he was finally diagnosed with NF1. Those who have café-au-lait macules and cutaneous neurofibromas may present with acute cervical hematoma, and it is important to consider the possibility of ruptured aneurysms in the neck region. When patients develop an acute cervical hematoma that causes an acute upper airway obstruction, emergency physicians should consider awake fiberoptic intubation to secure the airway.
摘要：
即使在未确诊的NF1患者中，1型神经纤维瘤病（NF1）也会引起血管并发症。锁骨下动脉分支动脉瘤破裂是一种罕见但危及生命的事件，出血会导致上呼吸道阻塞.我们介绍了一例NF1患者的颈横动脉瘤破裂，导致气道几乎阻塞。一名52岁的男子以前没有被诊断为NF1，从左肩到颈部突然疼痛。从小,他有多个皮肤神经纤维瘤和咖啡色斑，双侧腋窝有雀斑.他的左侧颈部和左肩肿胀表明有血肿，压缩了上呼吸道。对比增强计算机断层扫描显示，由颈横动脉瘤破裂引起的宫颈血肿。我们进行了清醒的光纤插管，因为可以预测到困难的气道，并且由于宫颈前血肿，手术气道管理可能是不可能的。他的气道很安全,他的动脉瘤通过线圈栓塞成功治疗。根据他的皮肤发现,他最终被诊断出患有NF1。那些有café-au-lait黄斑和皮肤神经纤维瘤的人可能会出现急性宫颈血肿，重要的是要考虑颈部动脉瘤破裂的可能性。当患者出现急性宫颈血肿导致急性上呼吸道阻塞时，急诊医师应考虑清醒的光纤插管以确保气道的安全。
