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海绵状静脉畸形(存档)

Cavernous Venous Malformation(Archived)

作者信息

Zyck Stephanie, Gould Grahame C.

机构信息

SUNY Upstate Medical University

PMID:30252265
Abstract

Cerebral cavernous malformations, also known as cavernomas or cavernous hemangiomas, are clusters of abnormal and hyalinized capillaries without intervening brain tissue (see  Pons Cavernoma, Head CT, Cavernous Malformation). Due to recurrent microhemorrhages and thrombosis, they are typically surrounded by hemosiderin deposits and gliosis. These lesions have slow flow and low pressure, causing the average rupture risk to be much lower than that of some other vascular malformations, such as arteriovenous malformations. Cavernomas are often found incidentally, but can also present during evaluation of headaches, seizures, focal neurologic deficits, or symptomatic hemorrhage.  aSupratentorial location is the most common, though lesions in the basal ganglia, brainstem, cerebellum, and spine also can occur. They can be associated with developmental venous anomalies (DVAs). The average annual rate of hemorrhage is reported at 0.7%-1.1% per lesion in patients without a history of prior hemorrhage. This risk rises to approximately 4.5% in patients who have sustained previous intracerebral hemorrhage. Approximately 2-3 years after a hemorrhagic event, the risk of hemorrhage is thought to decrease. Rupture risk is also dependent on the cavernoma's location, presence of associated developmental venous anomaly, and gender. Infratentorial location, deep location, young age, and female gender are associated with increased risk., Asymptomatic familial cases are also thought to have a higher annual hemorrhage rate than asymptomatic sporadic cases. Cavernous malformations may also arise de novo. They may grow, shrink, or remain stable over time.

摘要

脑海绵状血管畸形,也称为海绵状瘤或海绵状血管瘤,是由异常且玻璃样变的毛细血管聚集而成,其间没有脑组织(见图“脑桥海绵状血管瘤、头部CT、海绵状血管畸形”)。由于反复发生微出血和血栓形成,它们通常被含铁血黄素沉积和胶质增生所环绕。这些病变血流缓慢、压力低,导致平均破裂风险远低于其他一些血管畸形,如动静脉畸形。海绵状瘤常为偶然发现,但也可在评估头痛、癫痫发作、局灶性神经功能缺损或症状性出血时出现。幕上部位最为常见,不过基底节、脑干、小脑和脊柱也可发生病变。它们可能与发育性静脉异常(DVA)相关。据报道,既往无出血史患者中每个病变每年的出血率平均为0.7%-1.1%。既往有脑出血的患者,这一风险升至约4.5%。出血事件发生后约2-3年,出血风险被认为会降低。破裂风险还取决于海绵状瘤的位置、是否存在相关的发育性静脉异常以及性别。幕下位置、深部位置、年轻和女性性别与风险增加相关。无症状的家族性病例每年的出血率也被认为高于无症状的散发性病例。海绵状血管畸形也可能是新发的。随着时间推移,它们可能会生长、缩小或保持稳定。