Doppler Ultrasound Flow Reversal in the Superior Sagittal Sinus to Detect Cerebral Venous Congestion in Vein of Galen Malformation

BACKGROUND AND PURPOSE: Vein of Galen malformation (VGAM) is a rare congenital cerebrovascular malformation in which increased cerebral venous pressure is a key etiologic factor for brain parenchymal damage. This study investigated whether serial cerebral venous Doppler measurements can detect and monitor increased cerebral venous pressure. MATERIALS AND METHODS: Retrospective monocentric analysis of cerebral ultrasound within the first 9 months of life in neonates with VGAM admitted at <28 days of life. Perfusion waveforms in superficial cerebral sinuses and veins were categorized into 6 patterns based on anterograde and retrograde flow components. Flow profiles were analyzed over time and correlated with disease severity, clinical interventions, and venous congestion damage on cerebral MR imaging (cMRI). RESULTS: Forty-four Doppler examinations of the superior sagittal sinus (SSS) and 36 of cortical veins from 7 patients were analyzed. Pre-interventional SSS Doppler flow profiles correlated with disease severity by Bicêtre Neonatal Evaluation Score (BNES) (Spearman r = −0.97, P < .001). Before embolization, 4/7 (57.1%) had retrograde flow in the SSS; after embolization, none of 6 treated patients had retrograde SSS flow. Only patients with a high retrograde flow component (≥ one-third retrograde flow; n = 2) showed severe venous congestion damage on cMRI. CONCLUSIONS: Flow profiles in the superficial cerebral sinuses and veins appear useful to noninvasively detect and monitor cerebral venous congestion in VGAM.