Abstract

Background Purpose: Vein of Galen aneurysmal malformations (VGAM) may carry life-threatening and catastrophic sequelae in children. Their clinical presentations are differently variable between cardiac and cerebral syndromes. There may be a possible relationship between the Galenic angioarchitecture and the clinical presentation. We aimed to study the effect of the venous outflow impairment on the incidence of high-flow heart failure and the incidence of hydrocephalus. Materials and Methods: The angiographies of 21 patients with true VGAM have been studied. MR angiography, 3-dimensional CT angiogram (3D CTA), and conventional digital subtraction angiography were performed for all patients with true VGAM. Transarterial embolization was done in 1 or more sessions for all cases. Results: Among the 21 cases, 14 cases were mural type, 5 cases were choroidal type, and 2 cases were mixed. Hydrocephalus was observed more in infants (92.3%), mural types (92.9%), giant and supergiant (87.5%) aneurysms, and in cases with persistent limbic circle (90.91%). All cases of hydrocephalus were associated with significant stenosis (>70%) of the Falcine sinus draining system (100%). Conclusion: Significant stenosis (>70%) of the draining sinus is a significant factor for VGAM aneurysmal enlargement and occurrence of hydrocephalus. Probably, venous outflow impairment decreases the incidence of high-flow heart failure and increases the incidence of hydrocephalus.

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