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多支血管腔内治疗未确诊的血管型埃勒斯-当洛综合征。在紧急情况下成功地经皮导管栓塞肝动脉假性动脉瘤，并对右肾动脉和髂动脉进行支架置入术。

Multivessel endovascular therapy for undiagnosed vascular type Ehlers-Danlos syndrome. Successful percutaneous transcatheter coil embolization of hepatic artery pseudoaneurysm with stenting of right renal and iliac arteries in emergency setting.

作者信息

Moramarco Lorenzo Paolo, Capodaglio Carlo Alberto, Quaretti Pietro, Cionfoli Nicola, Fiorina Ilaria, Disabella Eliana, D'agostino Antonio Mauro, Urtis Mario, Arbustini Eloisa

机构信息

Department of Radiology, IRCCS Fondazione Policlinico San Matteo, Pavia, Italy.

Unit of Interventional Radiology and Department of Radiology, IRCCS Fondazione Policlinico San Matteo, Pavia, Italy.

出版信息

BJR Case Rep. 2020 Jul 6;6(4):20200025. doi: 10.1259/bjrcr.20200025. eCollection 2020 Dec 1.

DOI:10.1259/bjrcr.20200025
PMID:33299587
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7709055/
Abstract

Among Ehlers-Danlos syndromes, the vascular type is the most severe because of its vascular complications. Transcatheter embolization of medium-sized arteries has become the first-line therapy for life-threatening hemorrhage. Ongoing multiple lesions causing hemorrhagic or ischemic complications in the acute phase can challenge patient management. Multivessel endovascular treatment has never been reported. In this study, we report successful single-session treatment by coiling of a ruptured pseudoaneurysm of the hepatic artery with stenting of dissected right renal and iliac arteries in a 46-year-old female. Percutaneous transfemoral approach was gained and sealed with a plug-based closure device. Genetic disease was subsequently confirmed by molecular analysis.

摘要

在埃勒斯-当洛综合征中，血管型最为严重，因其会引发血管并发症。经导管栓塞中等大小动脉已成为危及生命出血的一线治疗方法。在急性期持续出现导致出血或缺血并发症的多处病变会给患者管理带来挑战。多血管腔内治疗此前从未有过报道。在本研究中，我们报告了对一名46岁女性成功进行单阶段治疗的病例，该患者肝动脉假性动脉瘤破裂，通过弹簧圈栓塞治疗，同时对右肾动脉及髂动脉夹层进行支架置入。采用经皮股动脉入路，并使用基于封堵器的闭合装置进行封堵。随后通过分子分析确诊为遗传性疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8747/7709055/10ae173b86e7/bjrcr.20200025.g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8747/7709055/a0c7d4dd7458/bjrcr.20200025.g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8747/7709055/10ae173b86e7/bjrcr.20200025.g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8747/7709055/a0c7d4dd7458/bjrcr.20200025.g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8747/7709055/10ae173b86e7/bjrcr.20200025.g002.jpg

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本文引用的文献

1
Renal Artery Dissection in a Young Woman: Diagnoses Beyond Fibromuscular Dysplasia.年轻女性肾动脉夹层：纤维肌性发育不良之外的诊断。
Nephron. 2019;143(2):128-132. doi: 10.1159/000501039. Epub 2019 Aug 8.
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Spontaneous pneumothorax and hemothorax frequently precede the arterial and intestinal complications of vascular Ehlers-Danlos syndrome.自发性气胸和血胸常先于血管型 Ehlers-Danlos 综合征的动脉和肠道并发症出现。
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Giant carotid pseudoaneurysm amenable to pipeline stenting in a patient with Ehlers-Danlos type IV.
一名患有IV型埃勒斯-当洛综合征的患者，其巨大颈总动脉假性动脉瘤适合采用密网支架置入术治疗。
Surg Neurol Int. 2019 Jan 25;10:11. doi: 10.4103/sni.sni_343_18. eCollection 2019.
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Bilateral Vertebral Artery Dissection and Unilateral Carotid Artery Dissection in Case of Ehlers-Danlos Syndrome Type IV.IV型埃勒斯-当洛综合征患者双侧椎动脉夹层与单侧颈动脉夹层
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The 2017 international classification of the Ehlers-Danlos syndromes.2017年埃勒斯-当洛综合征国际分类法。
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Embolization of life-threatening arterial rupture in patients with vascular Ehlers-Danlos syndrome.血管性埃勒斯-当洛综合征患者危及生命的动脉破裂的栓塞治疗
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Vascular Ehlers-Danlos syndrome presenting as rapidly progressive multiple arterial aneurysms and dissections.表现为快速进展性多发性动脉动脉瘤和夹层的血管型埃勒斯-当洛斯综合征。
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