Abstract

Six neonates with aneurysms of the vein of Galen are described: five were associated with an intracerebral arteriovenous fistula and presented with heart failure in the neonatal period; one had hydrocephalus at birth. Two infants died from intractable cardiac failure within 48 hours of age and two from progressive hydrocephalus and cardiac failure at 8 months and 8 years respectively. Two of the infants had copper coil embolisation of the aneurysm--one has since died of an intracerebral haemorrhage. The diagnosis of an intracerebral arteriovenous fistula should be considered in any infant presenting with unexplained heart failure. Close attention to the volume of all arterial pulses, and the venous pulse in the neck as well as auscultation of the head for a cranial bruit may suggest the diagnosis, which can then be substantiated by cerebral ultrasound.