Vein of Galen Aneurysmal Malformation

Abstract

In this chapter, the disease pattern “vein of Galen aneurysmal malformation” (VGAM) is outlined. In Part A, the body of the literature is summarized with sections, namely historical aspects and definitions, epidemiology and clinical presentation, pathophysiology and pathoanatomy, classification of VGAM subtypes, and treatment and outcome. Part B covers controversial issues and dedicated management strategies, whereby the perspective and proceedings in a VGAM high-volume center are presented: indications for treatment, the angioarchitecture of VGAMs and strategy of endovascular therapy, and modern techniques in diagnostic and interventional neuroradiology. The authors’ intention is to provide comprehensive clear explanations of VGAMs and its interdisciplinary management. A special focus constitutes the recent advances in the field of diagnostic and interventional neuroradiology, which is key for safe and effective embolization procedures in VGAM patients. In the emergency setting, endovascular therapy must be performed for hemodynamic stabilization with prevention of multi-organ failure and ultimately death. In the staged elective setting, endovascular therapy is applied for the neurological stabilization, with the goal of normal or near-normal neurological development as well as the prevention of intracranial hemorrhage. New images and illustrations help to understand the highly complex embolization techniques and associated hemodynamic changes of cerebral blood flow.