The Management of Vein of Galen Aneurysmal Malformations

Abstract

OBJECTIVE: The vein of Galen aneurysmal malformation (VGAM) is a choroidal type of arteriovenous malformation involving the vein of Galen forerunner. This is distinct from an arteriovenous malformation with venous drainage into a dilated, but already formed, vein of Galen. Reports of endovascular treatment of VGAM in the literature approach the disease from a purely technical viewpoint and often fail to provide satisfactory midterm results. To focus the therapeutic challenge to a strictly morphological goal overlooks the fundamental aspects of neonatal and infant anatomy and fluid physiology. During the past 20 years, our approach to VGAM has remained the same. Our experience, based on 317 patients with VGAM who were studied in Hospital Bicêtre between October 1981 and October 2002, allows us to describe the angioarchitecture, natural history, and management of VGAM in neonates, infants, and children. METHODS: Of our cohort of 317 patients, 233 patients were treated with endovascular embolization; of these, 216 patients were treated in our hospital. The treatment method of choice was a transfemoral arterial approach to deliver glue at the fistulous zone. RESULTS: Of 216 patients, 23 died despite or because of the embolization (10.6%). Twenty out of the 193 (10.4%) surviving patients were severely retarded, 30 (15.6%) were moderately retarded, and 143 (74%) were neurologically normal on follow-up. CONCLUSION: Our data demonstrate that most treated children survive and undergo normal neurological development; an understanding of the clinical, anatomical, and pathophysiological features of VGAM has, therefore, reversed the former poor prognosis. Our level of understanding about the lesion allows us to predict most situations and remedy them by applying a strict evaluation protocol and working within an optimal therapeutic window. Patient selection and timing remain the keys in the management of this condition. It is more important to restore normal growth conditions than a normal morphological appearance, with the primary therapeutic objective being normal development in a child without neurological deficit.