The persistent primitive marginal sinus - Observations in children with Vein of Galen malformation

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.