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马凡综合征患者的颈内动脉动脉瘤和颈部巨大冗长动脉

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

作者信息

Croisile B, Deruty R, Pialat J, Chazot G, Jourdan C

机构信息

Service de Neurologie, Hôpital Neurologique de Lyon.

出版信息

Neurochirurgie. 1988;34(5):342-7.

PMID:3231295
Abstract

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.

摘要

作者描述了一例马方综合征患者,该患者出现巨大颅内颈内动脉瘤,同时伴有双侧颈内动脉和椎动脉延长及迂曲。皮肤显微镜检查显示弹性纤维断裂和扭曲。未对血管进行显微镜研究。马方综合征中很少报告颅外和颅内血管异常:心血管变化主要见于主动脉和肺动脉。文献中报道了一些颈、基底和椎动脉的巨大动脉瘤、夹层和扩张。通常血管显微镜检查显示囊性中层坏死。其他结缔组织疾病(弹性假黄瘤、埃勒斯-当洛综合征、早老症)也有此类临床异常的描述。文中提到,在没有结缔组织疾病但出现动脉夹层或动脉瘤的患者中,可能存在未知的组织学和化学损伤削弱血管。因此,血管对衰老、动脉硬化或高血压可能更敏感。

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