Aneurysmal Malformations of the Vein of Galen

Abstract

The Vein of Galen Aneurysmal Malformation (VGAM) is regarded as a lesion with high morbidity and mortality, both spontaneously and under treatment, in part due to an incomplete appreciation of the effects of the lesion on the specific physiology of young children. In addition, various techniques have been applied over a short period of time to treat the lesion rather than the disorders it creates. We report experience with 120 consecutive cases of VGAM managed over the past ten years: 24 were diagnosed antenatally; 50 presented in the neonatal period with haemodynamic disturbance; 35 in infants presented with secondary hydrovenous disorders (macrocrania, subependymal atrophy and ventricular dilatation); 12 were seen in children. We were unable to follow 10% of the patients because the referring teams decided not to follow our therapeutic advice. Treatment was contra-indicated in 17% of cases (with early brain damage and a rapidly fatal outcome). There were five (4%) which thrombosed spontaneously (but only two of these were neurologically normal). Embolisation was performed in 78 patients via a transarterial femoral approach using glue. Anatomical cure has already been achieved in 53%. Of the treated patients, even when treatment has not been completed, 80% are growing neurologically normal. Permanent neurological deficit or irreversible neurocognitive delay related to the natural history of the lesion were evident in 8.5%. Overall mortality was 9%. These figures confirm that previous views on the prognosis of VGAM should be revised. Endoarterial embolisation, is the preferred treatment to date.