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Cerebrofacial venous metameric syndrome-spectrum of imaging findings.
Neuroradiology ( IF 2.4 ) Pub Date : 2020-01-14 , DOI: 10.1007/s00234-020-02362-7
Waleed Brinjikji 1, 2 , Patrick Nicholson 1 , Christopher A Hilditch 1 , Timo Krings 1 , Vitor Pereira 1 , Ronit Agid 1
Affiliation  

Cerebrofacial venous metameric syndrome (CVMS) is a complex craniofacial vascular malformation disorder in which patients have a constellation of venous vascular malformations affecting soft tissues, bone, dura, and neural structures including the eye and brain. It is hypothesized that a somatic mutation responsible for the venous abnormalities occurred prior to migration of the neural crest cells, and because of this, facial, osseous, and cerebral involvement typically follows a segmental or "metameric" distribution. The most commonly recognized form of CVMS is Sturge-Weber syndrome. However, a wide spectrum of CVMS phenotypical presentations exist with various metameric distributions of slow-flow vascular lesions including facial venous vascular malformations, developmental venous anomalies, venous angiomas, cavernous malformations (cavernomas), dural sinus malformations, and maybe even vascular tumors such as cavernous hemangiomas. Awareness of the various manifestations as described herewith is important for treatment and screening purposes.

中文翻译:

脑面静脉多态性综合征的影像学表现。

脑面静脉异基因综合症(CVMS)是一种复杂的颅面血管畸形疾病,患者体内有一系列影响软组织,骨骼,硬脑膜以及包括眼睛和大脑在内的神经结构的静脉血管畸形。据推测,引起静脉异常的体细胞突变发生在神经c细胞迁移之前,因此,面部,骨和脑受累通常遵循节段性或“介体”分布。CVMS最常见的形式是Sturge-Weber综合征。但是,存在多种CVMS表型表现形式,包括慢流性血管病变的各种同分异构分布,包括面部静脉血管畸形,发育性静脉异常,静脉血管瘤,海绵状畸形(海绵瘤),硬脑膜窦畸形,甚至可能是血管肿瘤,例如海绵状血管瘤。如此描述的各种表现形式的认识对于治疗和筛选目的是重要的。
更新日期:2020-01-14
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