Abstract

Plain Language SummaryVein of Galen malformation is a rare, congenital intracerebral arteriovenous malformation with high morbidity and mortality in neonatal manifestation despite modern interdisciplinary strategies. Currently, there is no valid, noninvasive parameter for early prediction of clinical progression at the bedside. A typical finding of hemodynamically relevant vein of Galen malformation is flow reversal in the descending aorta (aortic steal). Therefore, we developed a new ultrasound method to quantify the percentage of reflux in the preductal aortic isthmus and integrated the measurement method into routine clinical practice. In this study, we retrospectively investigated the association between percentage of aortic steal, organ dysfunction parameters, mortality, and brain injury on magnetic resonance imaging (MRI) at baseline and follow-up in 12 neonates with vein of Galen malformation admitted between January 2021 and June 2022. Statistical analyses showed that the severity of organ dysfunction, mortality, and brain damage on MRI increased with increasing percentage of aortic steal. These correlations and associations showed a linear relationship between percentage reflux in the aortic isthmus and all outcome parameters. The potential of sonographically determined aortic steal to predict clinical progression and short- and long-term outcomes needs to be investigated in larger, prospective cohorts. If this marker proves as promising as the data from this study suggest, it could become an important diagnostic component for management and decision-making in critically ill neonates with vein of Galen malformation.

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