Decreased Superior Sagittal Sinus Diameter and Jugular Bulb Narrowing Are Associated with Poor Clinical Outcome in Vein of Galen Arteriovenous Malformation

Background and purpose: Few clinical and imaging findings are known to be associated with poor outcome in neonates and infants with vein of Galen arteriovenous malformations. In the present consecutive series of 35 patients, we evaluated both the diameter of the superior sagittal sinus at onset and the diameter of the jugular bulb on follow-up as potential factors related to poor outcome.

Materials and methods: Thirty-five consecutive neonates and infants who were prospectively collected in a single-center data base were included in this review. Outcome was assessed by using the Bicêtre Outcome Score. Both the absolute diameter of the superior sagittal sinus and its ratio to the biparietal diameter were measured at onset, compared with age-matched controls, and correlated to patient outcome.

Results: The diameter of the superior sagittal sinus at onset and its ratio to the biparietal diameter were significantly smaller in the vein of Galen arteriovenous malformation population compared with the matched population (P = .0001) and were correlated significantly with a risk of poor clinical outcome (P = .008). Development of jugular bulb narrowing was also related to poor clinical outcome (P < .0001).

Conclusions: Decreased superior sagittal sinus diameter may reflect a decrease of cerebral blood flow due to cerebral arterial steal and intracranial hydrovenous disorders. This finding may be considered cerebral blood flow deterioration and thus taken into consideration in the management decisions for patients with vein of Galen arteriovenous malformations. Likewise, our data suggest that progressive jugular bulb narrowing may indicate earlier intervention to prevent severe narrowing.