Evaluation, management, and long-term follow up of vein of Galen malformations

Abstract

Object Vein of Galen malformations (VGMs) are extremely rare intracranial lesions. Clinical presentation and management strategies vary significantly in different areas of the world. The authors report their experience in evaluation, management, and long-term follow up of these lesions in India. Methods Between October 1983 and June 2003, 25 patients with VGMs were referred to the authors’ institution for evaluation and management. Ten children younger than 2 years of age presented with rapidly increasing head size as the chief complaint. Among 11 children 2 years of age or older, the most common presenting symptom was chronic headache. Four patients who presented during adulthood had chronic headache for many years before presentation. Angiographic evaluation of the lesion was performed in 21 patients. Fifteen patients were treated using endovascular techniques. Injection of the embolic material was performed after induction of systemic hypotension when the flow in the fistula was high. Complete occlusion of the arteriovenous shunt could be achieved in two patients with vein of Galen aneurysmal dilation (100% of patients with this type of malformation) and in five of the six patients with the mural type of malformation (83%). Among patients with the choroidal type of malformation, complete obliteration of the shunt could be achieved in three patients. In three patients with high-flow choroidal malformations, embolization carried out in a single sitting resulted in shunt reduction of nearly 90%. These patients received clinical follow up. Conclusions The authors’ experience in evaluation and management of VGMs reveals that in areas of the world where access to dedicated specialist care is limited, the clinical presentation of VGMs can differ appreciably from the classic descriptions in the literature. Endovascular management of these lesions results in excellent angiographic and clinical results.