Choroidal Type of Vein of Galen Aneurysmal Malformation in Adult Patient with Unusual Presentation of Orthostatic Headache

成人患者脉络膜型大脑大静脉瘤样畸形伴不寻常的直立性头痛

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Abstract

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.

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