Vein of Galen malformation: What to do when vascular access is not feasible?

Abstract

Abstract Background: The vein of Galen aneurysmal malformation (GVAM) is a rare congenital vascular lesion, with high morbidity and mortality without treatment, endovascular management is the best alternative available today. Aim: The purpose of this work is to report the case of a female patient with imaginological and angiographical diagnosis of GVAM, which was difficult for endovascular managent alone, due to this was decided an alternative endovascular direct access guided by stereotactic assistance. Case report: We report the case of an infant with imaging and angiographic diagnosis of GVAM, with absence of the vein access through classical embolization could be done, complicating and making difficult for endovascular management alone, hence was decided an alternative endovascular direct access guided by stereotactic assistance, ensuring total embolization of the lesion with coils. The patient progressed satisfactorily. Discussion: VGAM generally represents only 1% or less of total cerebral vascular malformations, but in pediatrics may be as high as 30%. The malformation is occupying the subarachnoid space of the velum interpositum and the quadrigeminal cistern. It is characterized by the presence of the vein embryonic precursor of the vein of Galen, and abnormal arterial shunts from arteries normally developed but extremely dilated. Mortality rate is high in these patients without treatment, developing hydrocephalus and cerebral complications related to the occupying mass that could drive to epilepsy, serious cognitive sequelae, intracerebral emorrhage and death within the possibilities. Heart failure is often a common complication. To our knowledge this is the first report of the use of stereotactic assistance in the endovascular therapeutic management of VGAM. Conclusions: In cases where clasical venous access cannot be achieved as in the case we report, further technical assistance can be obtained with stereotactic guidance, making easier the procedure, remembering that without therapy, VGAM result in lifethreatening hydrocephalus and/or intracranial hemorrhage, rapidly driving to death.