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Neurochirurgie 1988

[Aneurysm of the internal carotid artery and cervical mega-dolicho-arteries in Marfan syndrome].

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B Croisile
R Deruty
J Pialat
G Chazot
C Jourdan

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The authors describe the case of a Marfan syndrome who presented a giant intracranial internal carotid aneurysm associated with elongation and tortuosity of internal carotid and vertebral arteries on both sides. The skin microscopic examination showed fragmentation and distorsion of elastic fibers. There was no microscopic study of the vessels. Extra and intracranial vascular abnormalities are rarely reported in marfan syndrome: cardiovascular changes are seen mostly in aorta and pulmonary artery. In the literature are reported some giant aneurysms, dissections and dilatations of carotid, basilar and vertebral arteries. Usually the microscopic examination of the vessels show cystic medial necrosis. Other connective tissue diseases (pseudo-xanthoma elasticum, Ehlers-Danlos syndrome, progeria) are described with such clinical abnormalities. Reference is made to the possibility of unknown histological and chemical lesions weakening the vessels in patients without connective tissue disorders and presenting with arterial dissection or arterial aneurysm. So vessels would be more sensible to aging, arteriosclerosis or hypertension.

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