Camilo Jaimes, Suely Fazio Ferraciolli, Darren B Orbach
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引用次数: 0
摘要
在胎儿成像中可以看到影响大脑的四种不同的血管畸形:盖伦静脉畸形、非盖伦动静脉瘘、硬脑膜窦畸形和颅内静脉畸形。这些先天性疾病影响发育中的大脑动脉和静脉,在新生儿期后很少见。这四种胎儿脑血管畸形的自然病史和预后各不相同。核磁共振成像能明确诊断、发现细微的实质损伤、详细描述异常血管的走向和在一定程度上显示血管流动的性质,以及识别缺血、血栓和出血性并发症,因此在评估这些病症的胎儿中起着举足轻重的作用。最近,一项针对治疗新生儿失代偿高风险的盖伦静脉畸形胎儿的研究性经输尿管栓塞术出现了,它是期待治疗和产后栓塞的一种很有前途的替代方法,影像学可用于确定适合进行干预的患者和术前计划。本手稿回顾了这四种胎儿神经血管畸形的基本影像学和临床特征,并强调了与咨询、预后和这些实体的多学科管理有关的实际问题:ACVRL1=类活化素 A 受体 1 型;b-SSFP=平衡稳态自由前旋;DSM=硬脑膜窦畸形;Ephrin B4= Ephrin B 型受体 4;icVM=颅内静脉畸形;ITGB1=整合素亚基 Beta 1;NOTCH1= Neurogenic locus notch homolog protein 1; PTPN11= Protein Tyrosine Phosphatase Non-Receptor Type 11; RASA1= RAS P21 Protein Activator 1; SSFSE= Single-shot fast spin echo; VOGM=Vein of Galen Malformation.
Four distinct vascular anomalies can be seen to affect the brain on fetal imaging: vein of Galen malformations, nongalenic arteriovenous pial fistulas, dural sinus malformations, and intracranial venous malformations. These congenital disorders affect the arteries and veins of the developing brain and are rarely seen beyond the neonatal stage. The 4 fetal cerebrovascular anomalies are associated with quite disparate natural histories and prognoses. MRI plays a pivotal role in the evaluation of fetuses with these conditions because of its ability to definitively establish the diagnosis, to detect subtle parenchymal injuries, to delineate the course of abnormal vessels in detail and to some extent the nature of vascular flow, and to identify ischemic, thrombotic, and hemorrhagic complications. Recently, an investigational transuterine embolization procedure targeted at treating fetuses with vein of Galen malformations who are at high risk for neonatal decompensation has emerged as a promising alternative to expectant management and postnatal embolization, with imaging being used to identify suitable patients for the intervention and in preprocedural planning. This manuscript reviews the essential imaging and clinical features of these 4 fetal neurovascular anomalies and underscores the practical aspects related to counseling, prognosis, and the multidisciplinary management of these entities.