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使用髂支装置对一名因无效变异而患有血管性埃勒斯-当洛综合征患者的髂总动脉瘤进行血管内修复。

Endovascular repair of a common iliac artery aneurysm with an iliac branch device in a patient with vascular Ehlers-Danlos syndrome due to a null variant.

作者信息

Shalhub Sherene, Byers Peter H

机构信息

Division of Vascular and Endovascular Surgery, Department of Surgery, Oregon Health & Science University, Portland, OR.

Department of Pathology, University of Washington, Seattle, WA.

出版信息

J Vasc Surg Cases Innov Tech. 2023 Apr 20;9(2):101192. doi: 10.1016/j.jvscit.2023.101192. eCollection 2023 Jun.

Abstract

Endovascular repair is avoided in patients with connective tissues disorders due to concerns for stent graft migration and endoleaks. We describe a successful endovascular repair of a common iliac artery aneurysm with a bifurcated aortoiliac stent graft and iliac branch endoprosthesis in a patient with Vascular Ehlers-Danlos syndrome (VEDS) due to a null variant. This case demonstrates that the VEDS genotype is associated with tissue integrity, specifically, individuals with VEDS due to null/haploinsufficiency variants, and adds to our understanding of endovascular repair in this population.

摘要

由于担心支架移植物移位和内漏,患有结缔组织疾病的患者避免进行血管内修复。我们描述了一例血管性埃勒斯-当洛综合征(VEDS)患者因无效变异,使用分叉型主动脉髂动脉支架移植物和髂支内假体成功进行髂总动脉瘤血管内修复的病例。该病例表明VEDS基因型与组织完整性相关,特别是因无效/单倍体不足变异导致VEDS的个体,并增进了我们对该人群血管内修复的理解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e169/10238461/dc1af78906e9/gr1.jpg

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