Pareek Kapil, Shrivastava Trilochan, Sinha Virendra Deo
Department of Neurosurgery, SMS Medical College, Jaipur, Rajasthan, India.
Asian J Neurosurg. 2018 Apr-Jun;13(2):418-420. doi: 10.4103/1793-5482.228542.
Vein of Galen aneurysmal malformations (VGAMs) are rare vascular malformations occurring commonly in the pediatric age group. They comprise only 1% of all intracranial vascular malformations, but in pediatric population, they represent 30% of all intracranial vascular malformations. They are of two types-mural and choroidal. Choroidal type of VGAM is more primitive and most severe form of disease. It usually causes high cardiac output failure in newborn period because of multiple high flow fistulas. Adult presentation of choroidal type VGAM is very rare, and only few cases have been reported in literature so far. A 21-year-old female patient presented with an orthostatic headache. Neuroimaging showed the choroidal type of VGAM. Staged embolization was planned through transarterial route. Partial embolization was done in the first stage. Patient's symptoms were disappeared thereafter. As per our knowledge, orthostatic headache has not been described previously in literature. With a very few existing case reports of adult true vein of Galen malformations, we believe that this case can help in understanding the natural history of vein of Galen malformations and the pathophysiology of its development.
大脑大静脉动脉瘤样畸形（VGAMs）是罕见的血管畸形，常见于儿童年龄组。它们仅占所有颅内血管畸形的1%，但在儿童人群中，它们占所有颅内血管畸形的30%。它们有两种类型——壁型和脉络丛型。脉络丛型VGAM是更原始且最严重的疾病形式。由于多个高流量瘘管，它通常在新生儿期导致高心输出量衰竭。脉络丛型VGAM的成人表现非常罕见，迄今为止文献中仅报道了少数病例。一名21岁女性患者出现体位性头痛。神经影像学显示为脉络丛型VGAM。计划通过经动脉途径进行分期栓塞。第一阶段进行了部分栓塞。此后患者症状消失。据我们所知，体位性头痛此前在文献中尚未有描述。鉴于现有的成人真正大脑大静脉畸形病例报告非常少，我们认为该病例有助于了解大脑大静脉畸形的自然病史及其发展的病理生理学。
