Vein of Galen Aneurysmal Malformation Masquerading as Carotid Cavernous Fistula in a Child with Proptosis

Purpose An infant with proptosis and dilated episcleral vessels was diagnosed with vein of Galen malformation, which is a rare condition presenting initially to an ophthalmologist. Methods An 8-month-old child presented with slowly progressive proptosis of the left eye of 5 months' duration. The proptosis was axial, nonpulsatile, with no associated bruit. Dilated corkscrew episcleral vessels were observed. The patient was referred to our center with diagnosis of carotid cavernous fistula. The child had a history of episodes of seizures. Results Contrast-enhanced computed tomographic scan of the patient showed dilated vessels, hydrocephalus, and dilated vein of Galen. Vein of Galen aneurysmal malformation was confirmed on magnetic resonance venography. Conclusions Occasionally patients with Vein of Galen aneurysmal malformation may first present to the ophthalmologist. Ophthalmologists should therefore be aware of this rare but potentially treatable condition. (Eur J Ophthalmol 2009; 19: 894–6)