PMC2248586_01
Non-contrast head CT (Fig 1A) demonstrates frontal lobe hemorrhage (arrows). Source image from MRA of the brain (Fig 1B) demonstrates 8 mm aneurysm in the distal A1 segment of the right ACA (arrowheads). DSA (AP/oblique) from right ICA injections (Fig 1C) confirms aneurysm of the distal A1 ACA segment. 3-D TOF MRA abdomen (Fig 1D) with bilateral internal iliac artery aneurysms (arrows), narrowing of mid-abdominal aorta (arrowheads), diffusely narrowed bilateral common and external iliac arteries (asterisks). 2-D TOF MRA abdomen (Fig 1E) shows worsening involvement of left common iliac artery (arrows), with right external and internal iliac arterial aneurysms unchanged (arrowheads).
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F1
Head CT (Fig 1A) demonstrated hemorrhage and dilated ventricles. Brain MRI/A demonstrated aneurysms of the right anterior cerebral artery (ACA) (Fig 1B) and the left internal carotid artery (ICA), confirmed by digital subtraction angiogram (DSA; Fig 1C). Full body MRA revealed iliac artery aneurysms, aortic narrowing (Fig 1D), and gluteal aneurysms. Repeat imaging showed progression in the left common iliac artery (Fig 1E) and narrowing of the renal arteries.
H&E stain (200x, Fig 2A; 400x, Fig 2B) from right internal iliac artery shows transmural vasculitis with intimal thickening (asterisks) and giant cells (arrowheads). Elastic stain reveals disruption of elastic lamina (Fig 2C). AP DSA, fusiform aneurysms supraclinoid left ICA (arrowheads; Fig 2D), right ICA (arrowheads; Fig 2E). Note aneurysm clips (arrows). DSA, diminutive, irregular aorta (arrows; Fig 2F). Right common iliac artery not identified (asterisks), no flow distal left common iliac artery (arrowheads). Non-contrast head CT (Fig 2G) demonstrates acute hemorrhage (arrows).
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Pathology demonstrated a transmural vasculitis with intimal thickening, giant cells (Fig 2A, B) and elastic lamina disruption (Fig 2C). Head CT showed new intraventricular hemorrhage, and DSA revealed fusiform aneurysms in the supraclinoid ICA's (Fig 2D,E), right lumbar, and internal maxillary arteries. On imaging, her aorta was narrowed (Fig 2F). Despite aggressive management, she had a massive intracerebral hemorrhage (Fig 2G).
Gross photograph, aortic and bilateral iliac artery aneurysms (Fig 3A). Left subclavian artery, focal intimal thickening (asterisks; H&E stain 40x, Fig 3B), transmural lymphohistiocytic infiltrate (asterisks; H&E 200x, Fig 3C), elastic lamina disruption (arrowheads; elastic stain 40x, Fig 3D). Gross photograph brain, saccular aneurysms right ICA (arrowhead), ACoA (asterisks; Fig 3E).
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Autopsy showed fusiform aneurysms of the descending aorta with thrombosis of 95% of the luminal cavity (Fig 3A). The rest of the aorta was grossly unremarkable, although histologic examination showed transmural lymphohistiocytic vasculitis with rare giant cells in the subclavian artery (Figs 3B-D), abdominal aorta (above the aneurysm), and iliac arteries. Cerebral vessels had saccular aneurysms in the right ICA and anterior communicating artery (ACoA; Fig 3E).