Successful stent placement for cervical artery dissection associated with the Ehlers—Danlos syndrome

Case report and review of the literature

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✓ This 44-year-old man with Ehlers—Danlos syndrome (EDS) Type IV presented with hemiparesis and the Gerstmann syndrome. Left carotid artery (CA) angiography revealed a dissecting aneurysm with severe stenosis located in the common CA; the lesion was successfully treated with a stent graft. The patient's clinical course after endovascular surgery was uneventful, without occurrence of megacolon. The literature for spontaneous CA dissection in EDS Type IV cases is reviewed and points for investigation and treatment are discussed.

Article Information

Address reprint requests to: Akita Kurata, M.D., Department of Neurosurgery, Kitasato University School of Medicine, 1–15–1 Kitasato, Sagamihara, Kanagawa, Japan. email: akirak@med.kitasato-u.ac.jp.

© AANS, except where prohibited by US copyright law.

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Figures

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    Axial CT scan revealing a low-density area in the left frontoparietal lobe.

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    Left: Aortogram revealing stenosis associated with aneurysm formation in the left CCA. Right: Follow-up aortogram demonstrating aggravation of the dissecting aneurysm, with severe stenosis (> 90%) of the left CCA.

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    Photomicrographs revealing fragmentation of elastic fibers and disarrangement of collagen fibers. The small arteries show disruption of the internal elastic membranes. H & E (A), van Gieson elastic (B). Original magnifications × 400.

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    Left: A 123I-IMP SPECT scan revealing an extensive decrease in CBF in the left temporoparietal lobe. Right: A 123I-IMP SPECT scan obtained after treatment, demonstrating an increase in CBF in the temporal lobe.

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    Aortogram obtained after stent placement, demonstrating good blood flow through the left CCA and a decrease in blood flow to the dissecting aneurysm.

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    A: Follow-up MR angiogram obtained 6 months posttreatment, demonstrating artifacts (arrows) at the proximal and distal ends of the stent in the left CCA. B: Follow-up intravenous digital subtraction angiogram obtained 7 months after the procedure, demonstrating patency of the left CCA and disappearance of the dissecting aneurysm.

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