Vein of Galen Aneurysms: Presentation and Endovascular Management

Abstract

Background: We present our experience with managing 13 cases of vein of Galen aneurysm with a special focus on endovascular strategies. This clinical review deals with the multivariable clinical presentation of vein of Galen aneurysms and the role of transarterial endovascular treatment. Methods: Thirteen patients diagnosed with vein of Galen aneurysms have been reviewed. Clinical presentation, diagnostic modalities and treatment strategies are also documented for each. MRI and three-dimensional CT angiogram (3D CTA) were performed for all patients. Transarterial embolization with Histoacryl was performed in 8 patients proved to have true vein of Galen aneurysmal malformation (VGAM). Results: Clinical presentation was variable including hydrocephalus, headaches, heart failure, coma, epilepsy, and even left orbital swelling. MRI demonstrated large vein of Galen aneurysms in all the cases. 3D CTA demonstrated true VGAM in 10 patients. Two cases proved to have vein of Galen varices. Endovascular treatment was conducted successfully in 8 patients and resulted in arrest of head growth, resolution of headaches and improvement of cardiac condition. Conclusion: The transarterial approach proved to be a successful way of management of true VGAM that yielded better outcomes.

Outcome and complications of endovascular embolization for vein of Galen malformations: a systematic review and meta-analysis

“…The literature search identified a total of 34 studies, [3][4][5][6][7][8][9][12][13][14][15]17,20,[22][23][24][25]28,30,[32][33][34][35][36][37][38][39][40][41][42]45,46,50,51 which included 667 patients whose VGAMs were treated by endovascular embolization (Fig. 1).…”

Vein of Galen Aneurysmal Malformation Presented by Dementia and Impotence in an Adult

“…As with any other fistula encountered in the brain, the aim is to embolize the fistulous point by embolic materials, either solid (detachable coils) or liquid (Onyx or N-butyl cyanacrylate) or both, using transarterial or transvenous routes or a combination of both. 1,2,4,5,12 What is peculiar about this case is that the patient was symptom free throughout his life, until he started to complain of the symptoms previously mentioned in his late twenties and early thirties. Unlike the usual course of vein of Galen malformations, which if left untreated will eventually lead to venous hypertension causing cortical and subcortical atrophy and white matter ischemia, eventually leading to seizures and mental retardation of various degrees, the patient had normal life throughout his schooling and graduated from college and had a stable job.…”

“…It is believed that these vascular malformations are abnormal arteriovenous communications between the arterial network of the choroidal fissure and the embryonic median prosencephalic vein of Markoweski. 2 Presentations vary according to the hemodynamics of the fistula; therefore, it either manifests as early as the perinatal or early neonatal period in the form of congestive heart failure due to high velocity shunt and causing high-output cardiac failure, or it manifests later during infancy with hydrocephalus and macrocrania caused by the growing Galenic pouch obstructing the normal cerebrospinal fluid pathways and partly due to venous hypertension leading to altered cerebrospinal fluid flow dynamics. [2][3][4] When left untreated, the disease process will either lead to high-output heart failure, multiorgan failure, and death in cases of congestive heart failure in neonates, or it will cause hydrocephalus, macrocrania, supratentorial and infratentorial venous congestion leading to white matter ischemia and cerebral atrophy, and eventually leading to mental retardation and seizures.…”

The natural progression of VGAMs and the need for urgent medical attention: a systematic review and meta-analysis

“…Patient clinical outcomes were analyzed in the 19 studies in which spontaneous thromboses occurred (see online supplementary table S6) 4 20 21 25 26 42–55. Online supplementary table S7 presents the clinical outcome of patients with spontaneous thromboses for various age groups.…”