Predicting factors for the follow-up outcome and management decisions in vein of Galen aneurysmal malformations

Childs Nerv Syst. 2010 Jan;26(1):35-46. doi: 10.1007/s00381-009-0959-7. Epub 2009 Aug 7.


Purpose: Vein of Galen aneurysmal malformations (VGAMs) are choroidal arteriovenous malformations that develop during an early embryonic stage. Although recent reports have shown improved outcome for these patients, the overall outcome still is poor. In this study, we evaluated the clinical, imaging, and angiographic features that may predict the outcome in VGAM patients.

Methods: Twenty-five patients diagnosed with VGAM were reviewed for clinical symptoms, including neonatal scoring systems, imaging findings, angioarchitecture, treatment decision, initial treatment age, follow-up timing, and follow-up outcome.

Results: Factors that were significantly associated with a poor outcome (p < 0.05) included neurological symptoms at presentation, a medium-to-low overall neonatal score (<12/21), a very poor score (<2/5) in one (or more) categories, focal parenchymal changes, calcifications, tonsillar herniation, arterial steal, or more than two groups of multiple arterial feeders. The venous drainage pattern and treatment age were not significantly associated with the overall outcome.

Conclusions: The presence of multiple factors that are related with poor outcome may warrant withholding aggressive treatment, while a small subgroup of carefully selected patients without any of these factors who are clinically asymptomatic may have a good outcome even with conservative management and close follow-up. For all other patients in which treatment is considered, the optimal treatment time is at 4-5 months of age; however, urgent treatment, regardless of age, should be indicated in those that do not have permanent brain damage on imaging with deteriorating congestive heart failure, evidence of arterial steal, or progressive occlusion of the venous outflow.

MeSH terms

  • Age Factors
  • Cerebral Angiography
  • Cerebral Veins / pathology
  • Cerebrovascular Circulation
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Infant, Newborn
  • Magnetic Resonance Angiography
  • Magnetic Resonance Imaging
  • Male
  • Pregnancy
  • Prenatal Diagnosis
  • Prognosis
  • Severity of Illness Index
  • Treatment Outcome
  • Vein of Galen Malformations / diagnosis*
  • Vein of Galen Malformations / pathology
  • Vein of Galen Malformations / therapy*