Endovascular management of vein of Galen aneurysmal malformations. Influence of the normal venous drainage on the choice of a treatment strategy

Abstract

This article reviews the available endovascular options for VGAM therapy, emphasizing three points that we have identified as critical in our practice for the establishment of a treatment strategy: (1) the importance of the deep cerebral venous anatomy, in particular the existence of normal drainage through the Galenic system in spite of the VGAM; (2) the concept of treatment staging, for arterial as well as for venous interventions; and (3) the definition of a therapeutic goal that can be attained at a reasonable cost in terms of complication risks and functional outcome.

Anatomy of the deep venous system in vein of Galen malformation and its changes after endovascular treatment depicted by magnetic resonance venography

“…Understanding venous drainage patterns in VGM patients and their role on the choice of treatment strategy in VGM is crucial 9. While there are several reported strategies for treatment of VGMs, the two main strategies discussed in the literature are transarterial embolization of VGM feeders, usually performed in a staged fashion, and transvenous embolization with the aim of closing the venous aneurysm of the VGM.…”

Susceptibility-Weighted Imaging: A New Tool in the Diagnosis and Evaluation of Abnormalities of the Vein of Galen in Children

“…In 1 patient, there were multiple prominent venous aneurysms surrounding the brain stem as a result of this phenomenon. The presence of these efferent pial venous drainage pathways carrying arterialized blood may impact upon the method and approach to treatment of these malformations, 12 and it is therefore important to differentiate them from veins enlarged because of passive venous congestion. These observations on the utility of SWI in differentiating between passive venous congestion and venous dilation from AVS in our series of patients are similar to the observations reported by Letourneau-Guillon and Krings 13 in their recent small series of patients with dural arteriovenous fistulas.…”

Vein of Galen Aneurysmal Malformation Presented by Dementia and Impotence in an Adult

“…As with any other fistula encountered in the brain, the aim is to embolize the fistulous point by embolic materials, either solid (detachable coils) or liquid (Onyx or N-butyl cyanacrylate) or both, using transarterial or transvenous routes or a combination of both. 1,2,4,5,12 What is peculiar about this case is that the patient was symptom free throughout his life, until he started to complain of the symptoms previously mentioned in his late twenties and early thirties. Unlike the usual course of vein of Galen malformations, which if left untreated will eventually lead to venous hypertension causing cortical and subcortical atrophy and white matter ischemia, eventually leading to seizures and mental retardation of various degrees, the patient had normal life throughout his schooling and graduated from college and had a stable job.…”

“…The true VGAMs are fistulas between the choroidal arterial supply and the vein of Galen, and they are further classified into mural or choroidal according to the anatomic pattern of the fistula. 4,5 Adult cases are very rare and are usually neglected cases that were not managed properly. Very few case reports exist on adult cases of true vein of Galen malformations, either discovered accidentally or with first presentation in adulthood.…”

“…2 Presentations vary according to the hemodynamics of the fistula; therefore, it either manifests as early as the perinatal or early neonatal period in the form of congestive heart failure due to high velocity shunt and causing high-output cardiac failure, or it manifests later during infancy with hydrocephalus and macrocrania caused by the growing Galenic pouch obstructing the normal cerebrospinal fluid pathways and partly due to venous hypertension leading to altered cerebrospinal fluid flow dynamics. [2][3][4] When left untreated, the disease process will either lead to high-output heart failure, multiorgan failure, and death in cases of congestive heart failure in neonates, or it will cause hydrocephalus, macrocrania, supratentorial and infratentorial venous congestion leading to white matter ischemia and cerebral atrophy, and eventually leading to mental retardation and seizures. 5 Lasjaunias classified the VGAMs into true and false.…”