Giant intracranial aneurysm associated with Marfan's syndrome

Case report

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✓ The authors describe a giant intracranial internal carotid aneurysm in a patient with Marfan's syndrome. Treatment consisted of internal carotid ligation with subsequent thrombosis of the lesion. The patient's course was complicated by carotid arterial dissection and possible laminar thrombosis within the aneurysm increasing the size of the lesion. Three clinical syndromes, Marfan's, Ehlers-Danlos, and pseudoxanthoma elasticum, are discussed. The literature is reviewed with reference to the hazards of treatment of vascular pathology in patients with these connective tissue disorders.

Article Information

Address reprint requests to: H. Lee Finney, M.D., Division of Neurosurgery, University of Utah Medical School, Salt Lake City, Utah 84112.

© AANS, except where prohibited by US copyright law.

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Figures

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    Angiography by selective left common carotid injection, anteroposterior (left), and lateral (right) views, shows laminar flow through a giant aneurysm involving the cavernous carotid artery segment.

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    Initial angiographic arch study shows normal appearance of the arch and initial segments of the great vessels. The left vertebral artery is grossly tortuous and shows multiple areas of aneurysmal dilatation. Lumen diameters of the right vertebral artery and right internal mammary artery (arrow) are also irregular.

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    Second angiographic arch study following surgery (note surgical clamp on the left internal carotid artery). Left: A double lumen is seen along most of the length of the left common carotid artery, indicating subintimal dissection (arrows). The left subclavian artery does not opacify distally (arrowhead). The caliber of the opacified lumen of the innominate artery is reduced compared to the initial study (Fig. 2), and neither the right vertebral nor the right internal mammary artery opacify from this source. Center: Several seconds later, slow antegrade filling of the right common carotid artery has occurred revealing a fine radiolucent line (arrows) representing another intimal dissection in this vessel. The right vertebral artery can be seen opacifying in a retrograde fashion. Note also the occlusion of the left subclavian artery (arrowhead). Right: Later film from the same injection shows progress of contrast material down the right vertebral artery into the right subclavian artery (arrow), which also contains a true and a false lumen, since this vessel opacifies separately from innominate and vertebral channels.

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    Postoperative angiography by right common carotid injection reveals dense opacification of the left middle cerebral artery, indicating that the principal collateral pathway to the left middle cerebral artery following left carotid occlusion is the anterior communicating artery from the right anterior circulation.

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