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马凡综合征合并大型颈总动脉假性动脉瘤伴进行性呼吸窘迫的血管内栓塞治疗

Endovascular Trapping of Large Cervical Carotid Pseudoaneurysm in Marfan Syndrome Presenting with Progressive Respiratory Distress.

作者信息

Salem Mohamed M, Ascanio Luis C, Kicielinski Kimberly P, Cambria Richard P, Ogilvy Christopher S, Alturki Abdulrahman Y

机构信息

Neurosurgical Service, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, USA.

Neurosurgical Service, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, USA.

出版信息

World Neurosurg. 2019 Mar;123:323-327. doi: 10.1016/j.wneu.2018.12.050. Epub 2018 Dec 24.

DOI:10.1016/j.wneu.2018.12.050
PMID:30590218
Abstract

BACKGROUND

Isolated cervical pseudoaneurysms of the internal carotid artery (ICA) in patients with Marfan syndrome are extremely rare.

CASE DESCRIPTION

A 73-year-old female with Marfan syndrome and history of multiple aortic aneurysms presented to an outside hospital with dysphagia, a mass in her neck, dizziness, nausea, and vomiting. A computed tomography angiogram showed bilateral cervical ICA pseudoaneurysms, more significant on the right side. A laryngoscopy demonstrated edematous vocal cords and a significant mass effect on the right side. She suffered from significant respiratory distress and aspiration, for which she had to be intubated and transferred to our institution for definitive care. The patient was taken for angiography for carotid artery test occlusion and endovascular trapping with coils and onyx embolization of the carotid pseudoaneurysm. The hospital course was complicated with femoral closure device failure, leading to groin hematoma, pneumonia, and respiratory failure requiring tracheostomy and percutaneous endoscopic gastrostomy. The patient was discharged to rehabilitation 34 days after admission. Stable exclusion of the large right pseudoaneurysm was observed at 3 months' follow-up with computed tomography angiography, as well as significant improvement in the last clinical follow-up obtained 6 months after the procedure.

CONCLUSION

Pseudoaneurysms of the cervical ICA associated with Marfan syndrome are rare. Carotid test occlusion and endovascular permanent embolization are possible in a patient with life-threatening symptoms. Cautious use of vascular closure devices, especially collagen plug-based ones, are required in such patients, given their abnormal extracellular matrix structure.

摘要

背景

马凡综合征患者孤立性颈内动脉假性动脉瘤极为罕见。

病例描述

一名73岁患有马凡综合征且有多个主动脉瘤病史的女性因吞咽困难、颈部肿块、头晕、恶心和呕吐就诊于外院。计算机断层血管造影显示双侧颈内动脉假性动脉瘤,右侧更为明显。喉镜检查显示声带水肿且右侧有明显的肿块效应。她出现严重的呼吸窘迫和误吸,为此不得不插管并转至我院接受确定性治疗。患者接受了颈动脉造影以进行颈动脉试验性闭塞以及采用弹簧圈和Onyx栓塞剂对颈动脉假性动脉瘤进行血管内圈套术。住院过程中出现股动脉闭合装置故障,导致腹股沟血肿、肺炎和呼吸衰竭,需要进行气管切开术和经皮内镜下胃造口术。患者入院34天后出院接受康复治疗。术后3个月的计算机断层血管造影显示右侧大的假性动脉瘤稳定排除,术后6个月的最后一次临床随访显示病情有显著改善。

结论

与马凡综合征相关的颈内动脉假性动脉瘤罕见。对于有危及生命症状的患者,颈动脉试验性闭塞和血管内永久性栓塞是可行的。鉴于此类患者细胞外基质结构异常,需要谨慎使用血管闭合装置,尤其是基于胶原塞的装置。

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