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Emergent embolization of a ruptured splenic artery aneurysm complicating Menkes disease.

ABSTRACT: We report a 7-year-old boy with Menkes disease complicated by rupture of a large splenic artery aneurysm. The aneurysm was successfully embolized with microcoils and n-butyl cyanoacrylate. Further angiographic evaluation revealed marked tortuosity of mesenteric and lower extremity vasculature, including the femoral arteries bilaterally, without aneurysm formation. The patient has since been evaluated annually with computed tomography angiography and there have been no additional vascular complications of his disease during 3-year follow up.

SUBMITTER: Olivieri JF

PROVIDER: S-EPMC6158999 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:BackgroundEndovascular aneurysm repair (EVAR) has been a revolutionary development in the treatment of abdominal aortic aneurysms (AAAs). Meanwhile, unfavorable anatomy of the aneurysm has always been a challenge to vascular surgeons, and the application of EVAR in emergent and elderly patients are still in dispute.Case presentationA 79-year-old woman presented as an emergency of abdominal pain with acute hypotension, heart rate elevation and a rapid decrease of hemoglobin. Emergent computed tomographic angiography (CTA) showed a ruptured AAA (rAAA) extending from below the opening of bilateral renal arteries down to the celiac artery and elongated to both common iliac arteries. The hostile neck and severely tortuous iliac artery made the following procedure a great challenge. An emergent endovascular approach was performed in which an excluder aortic main body was deployed below the origin of the bilateral renal arteries covering the ruptured aortic segment. Two iliac legs were placed superior to the opening of the right hypogastric respectively. In order to avoid the type Ib endoleak, we tried to deploy another cuff above the bifurcation of the iliac artery. However, the severely tortuous right iliac artery made this procedure extremely difficult, and a balloon-assisted technique was used in order to keep the stiff wire stable. Another iliac leg was placed above the bifurcation of the left iliac artery. The following angiography showed a severe Ia endoleak in the proximal neck and therefore, a cuff was deployed distal to opening of the left renal artery with off-the-shelf solution. The patient had an uneventful recovery with a resolution of the rAAA. She is well and symptom-free 6 months later.ConclusionEndovascular aneurysm repair (EVAR) in emergent elderly rAAA with hostile neck and severe tortuous iliac artery is extremely challenging, and endovascular management with integrated technique is feasible and may achieve a satisfactory early result.

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Emergent embolization of a ruptured splenic artery aneurysm complicating Menkes disease.

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