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

Abstract

BACKGROUND AND PURPOSE: Vein of Galen malformation is a rare congenital cerebrovascular malformation. In affected patients, increased cerebral venous pressure constitutes an important etiologic factor for the development of brain parenchymal damage. The aim of this study was to investigate the potential of serial cerebral venous Doppler measurements to detect and monitor increased cerebral venous pressure. MATERIALS AND METHODS:This was a retrospective monocentric analysis of ultrasound examinations within the first 9 months of life in patients with vein of Galen malformation admitted at ,28 days of life. Categorization of perfusion waveforms in the superficial cerebral sinus and veins into 6 patterns was based on antero-and retrograde flow components. We performed an analysis of flow profiles across time and correlation with disease severity, clinical interventions, and congestion damage on cerebral MR imaging. RESULTS:The study included 44 Doppler ultrasound examinations of the superior sagittal sinus and 36 examinations of the cortical veins from 7 patients. Doppler flow profiles before interventional therapy correlated with disease severity determined by the Bicêtre Neonatal Evaluation Score (Spearman r ¼ À0.97, P ¼ , .001). At this time, 4 of 7 patients (57.1%) showed a retrograde flow component in the superior sagittal sinus, whereas after embolization, none of the 6 treated patients presented with a retrograde flow component. Only patients with a high retrograde flow component (equal or more than one-third retrograde flow, n ¼ 2) showed severe venous congestion damage on cerebral MR imaging.CONCLUSIONS: Flow profiles in the superficial cerebral sinus and veins appear to be a useful tool to noninvasively detect and monitor cerebral venous congestion in vein of Galen malformation.

Aortic Steal Correlates with Acute Organ Dysfunction and Short-Term Outcomes in Neonates with Vein of Galen Malformation

“…However, reliable early prediction of clinical progress would be of particular therapeutic importance, as interventional treatment in a decompensated state is associated with higher complication rates and higher mortality [4]. Further, in some patients, even after stabilization of heart failure, cerebral damage can further progress due to decreased parenchymal perfusion and venous congestion [3].…”

“…Vein of Galen (aneurysmal) malformation (VGAM) is an extremely rare disease with an incidence of about 1: 58,100 live births per year in Germany [1]. In this cerebrovascular malformation, multiple arteriovenous (AV) fistulas and/or malformations between the persistent median prosencephalic vein of Markowski and various arterial feeding vessels results in intracerebral shunts between the arterial and venous systems [2,3]. By redistributing cardiac output, these AV shunts reduce perfusion of the brain parenchyma and other vital organs, ultimately producing high-output cardiac failure.…”

“…By redistributing cardiac output, these AV shunts reduce perfusion of the brain parenchyma and other vital organs, ultimately producing high-output cardiac failure. At the same time, the high venous cardiac return from the shunt results in volume overload of the right heart and right heart failure [3]. Without intervention, this constellation progresses to refractory multiorgan failure [4,5].…”