The Impact of Angiographic Geometry on the Clinical Presentation of True Vein of Galen Aneurysmal Malformations

Pediatr Neurosurg. 2020;55(6):344-350. doi: 10.1159/000512213. Epub 2021 Jan 13.

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.

Keywords: Falcine sinus; Heart failure; Hydrocephalus; Stenosis; Vein of Galen aneurysm; Venous outflow impairment.

MeSH terms

  • Angiography, Digital Subtraction
  • Cerebral Angiography
  • Cerebral Veins* / diagnostic imaging
  • Child
  • Embolization, Therapeutic*
  • Humans
  • Infant
  • Vein of Galen Malformations* / diagnostic imaging
  • Vein of Galen Malformations* / therapy