Association of Disease Severity and Cerebral Interventional Complications With Long-Term Neurodevelopmental Outcome in Neonates With Vein of Galen Malformation.

BACKGROUND

Vein of Galen malformation represents a rare but life-threatening intracranial arteriovenous shunting lesion with high mortality and poor long-term neurodevelopmental outcome in neonatal presentation. At present, predictive parameters for individual prognosis of neurological outcome are lacking.

METHODS

This is a retrospective single-center analysis of the association between disease severity, determined by the percentage of retrograde volume flow in the aortic isthmus (aortic steal), occurrence of cerebral interventional complications, and neurodevelopmental outcomes (motor development, cognitive function, and language) at age two years in a consecutive cohort of neonates with vein of Galen malformation. The overall outcome was a composite of the three subcategories categorized as favorable, intermediate, and poor.

RESULTS

Twelve neonates were included, of which five (42%) exhibited a favorable outcome, two (17%) an intermediate outcome with sequelae, and five (42%) a poor outcome. There was a strong correlation of aortic steal (%) with cerebral interventional complications (Spearman ρ = 0.78) and neurodevelopmental outcomes at age two years (Spearman ρ = 0.93). Occurrence of cerebral interventional complications showed a strong correlation with long-term neurodevelopmental outcomes (Spearman ρ = 0.89).

CONCLUSIONS

Disease severity as indicated by aortic steal (%) is associated with cerebral interventional complications and two-year neurodevelopmental outcomes in neonates with vein of Galen malformation. Cerebral interventional complications are strongly associated with outcomes at age two years and may be a direct and mediating risk factor between aortic steal (%) and neurodevelopment outcomes.