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

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

Lorenzo Paolo Moramarco  1 , Carlo Alberto Capodaglio  1 , Pietro Quaretti  2 , Nicola Cionfoli  2 , Ilaria Fiorina  2 , Eliana Disabella  3 , Antonio Mauro D'agostino  1 , Mario Urtis  3 , Eloisa Arbustini  3
Affiliations
Case Reports

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

Lorenzo Paolo Moramarco et al. BJR Case Rep. .

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.

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Figures

Figure 1.
Figure 1.
Coronal maximum intensity projection (a) and volume rendered 3D, (b) contrast-enhanced MDCT images showing hepatic artery pseudoaneurysm, the non-navigable celiac trunk, the dissected right renal and iliac axis. (c) Selective retrograde arteriogram via the inferior pancreaticoduodenal arcade vessels: the ruptured pseudoaneurysm involves hepatic artery posterior branch. (d) Completion angiography: complete packing is achieved. Exclusion of the pseudoaneurysm including (arrow) the distal common hepatic artery and the origin of the gastroduodenal artery is shown. Embolization of the anterior branch of the hepatic artery avoids late reperfusion through the backdoor of the pseudoaneurysm (arrowhead).
Figure 2.
Figure 2.
(a) Right renal angiography confirming the dissection. (b) Angiography post-stenting. Carotid Wallstent (Boston Sc., MA) had previously been proven to be compatible with the 6 Fr guiding catheter (c) MDCT at 5 months: patency of the two imbricated self-expandable stents in the right iliac axis. (d) Patent renal stent and consistency of the hepatic artery cast without reperfusion.
See this image and copyright information in PMC

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