Cranial Doppler ultrasound in Vein of Galen malformation.

Abstract

Vein of Galen malformation (VGM) is the severest paediatric neurovascular disease usually requiring multiple staged embolisations. In the high-risk group of children with high-flow arteriovenous shunts, timing of treatment is uncertain. Low Doppler resistance index (RI) is known to be associated with adverse outcome in hypoxic-ischaemic brain injury in children. In this study, we want to present our long-term results of cranial transfontanellar Doppler ultrasound in children with VGM. We identified and retrospectively analysed 264 transfontanellar Doppler measurements in 19 endovascular-treated true VGM (five females, 14 males) between 2000 and 2013. The recordings were obtained from the internal carotid arteries (ICA), the anterior cerebral arteries (ACA) and the basilar arteries (BA). Maximal systolic velocity (Vs), end-diastolic velocity (Ved) and the RI were measured before and after embolisation. Untreated, nearly all cases showed pathologic high systolic (up to >1.0m/s), very high diastolic velocities (up to >0.5m/s) and low RI (<0.6). There were statistically significant differences (p = 0.012) between the pre-embolisation RI and the post-embolisation RI with pathologic low RI before and nearly normal RI after successful shunt reduction. Successful endovascular shunt reduction in VGM leads to significant changes of cranial Doppler RI from pathologic low values to normal values. We propose cranial Doppler ultrasound as an adjunctive technique to other screenings in the management of VGM. Further research is warranted to evaluate the role of the RI in the treatment timing decision.