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Background and Purpose: It is classically thought that the internal cerebral veins (ICV) do not communicate with the venous pouch of vein of Galen malformations (VGM). We report on the anatomy of the deep venous system in VGM with special emphasis on the drainage of the ICV and possible changes after endovascular treatment., Materials and Methods: We retrospectively analyzed DSA and 2D time-of-flight MR venograms of 55 children with VGM. We evaluated all pre- and post-operative images for the presence of the ICVs and determined their route of venous drainage., Results: Of 55 children, pre-operative 2D MRV detected the ICVs in 19 cases (35%) compared with one case (2%) for pre-embolization DSA (2%) (P<0.0001). Of the cases in which the ICVs were seen preoperatively, in 15 cases (78.9%) the ICV drained directly into the VGM while in the other four cases, the ICV used alternative venous drainage routes. On post-operative MRV, the ICVs were seen in 17 cases (31%) on MRV and 10 cases (18.2%) on DSA with drainage into an adult-like vein of Galen in 13 cases (76%), respectively (P=0.08). In four cases normal ICV drainage into the vein of Galen was seen even when the venous sac was closed. In two cases there was a change in ICV drainage from the vein of Galen to the lateral mesencephalic vein., Conclusion: The communication of the ICV with the VGM is a common phenomenon. Different changes of venous drainage routes do occur after treatment and are best seen on MRV., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2019. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Introduction: 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., Methods: 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., Results: Untreated, nearly all cases showed pathologic high systolic (up to >1.0 m/s), very high diastolic velocities (up to >0.5 m/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., Conclusion: 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.

Introduction: Knowledge of venous anatomy and drainage in children with neurovascular diseases is crucial. The primitive marginal sinuses (PMS) are embryonic sinuses forming the later superior sagittal sinus. Their angiographic persistence has not been reported before. The purpose of this study is to demonstrate the angiographic characteristics in children with Vein of Galen malformation and to discuss their embryology and clinical significance., Methods: We retrospectively reviewed digital subtraction angiography and magnetic resonance venography of 102 Vein of Galen malformations (VGM) from the database of two high-load centres for paediatric neurovascular diseases., Results: We found 10 persistent PMS in 102 children indicating a frequency of 9.8 %. Illustrative cases are shown to demonstrate the vast spectrum of venous variability. The PMS becomes visualised in situations of high-flow arteriovenous shunt in children and can even disappear or remodel after successful shunt reduction or closure., Conclusion: Knowledge and recognition of the PMS are important for neurointerventionalists in endovascular treatment planning using the transvenous route and can help neurosurgeons in avoiding severe surgical complications.

A vein of Galen malformation - a rare cause of cardiac insufficiency in neonates - is sometimes associated with coarctation of the aorta, two diseases requiring urgent therapy in the neonatal period. We report on a term neonate in whom we first palliated the coarctation by stent implantation, providing time to treat the vein of Galen malformation by endovascular embolisation. Following this, the coarctation was surgically repaired and the stent was explanted.

Introduction: Vein of Galen aneurysmal malformation (VGAM) is a severe pediatric neurovascular disease. Children often present with congestive heart failure in the neonatal period. In the last decades, endovascular treatment became the first therapeutic option. The purpose of this study is to report our results in the treatment of VGAM with a combined transvenous and transarterial method in the last ten years., Methods: In our cohort of 28 patients with VGAM, 22 patients were treated endovascularly between 1992 and 2010. In the last 10 years, a consecutive series of 14 children were treated with a combined transvenous and transarterial method. The therapeutic goal was immediate shunt reduction of the arteriovenous malformation, especially in the neonatal period. Closure of the fistulous connections was achieved by coiling using a combined transvenous and transarterial approach, called "kissing microcatheter technique"., Results: Eight of 14 children presented in the neonatal period with severe congestive heart failure. The other six patients presented between the age of 2 and 17 months. One patient died due to a non-procedural complication in another hospital 2 years after the last treatment. Complete or >90% of angiographically confirmed closure of the malformation was documented in 11 of 14 patients. Normal or near-normal outcome was achieved in 9 of 13 surviving children, a non-favorable outcome was observed in four children. Control of heart failure was achieved in all patients., Conclusion: Endovascular treatment of VGAM using a combined transvenous and transarterial method is a safe procedure with a low complication rate. The overall outcome can be improved, especially in the high-risk neonatal group with congestive heart failure.