External carotid-cavernous fistula in infancy: Case report and review of the literature

A case of external carotid-cavernous fistula in a 7-week-old infant is presented. Unlike post-traumatic internal carotid-cavernous fistulas, most of these dural fistulas are low flow, low pressure shunts with a benign and chronic clinical course, sometimes ending in spontaneous closure. In adults and older children, meticulous follow-up of visual symptoms and signs may be adequate management. The only indication for aggressive therapy is progressive visual failure, which occurs rarely. In infants and visually immature children, however, persistent proptosis and intraocular hypertension can lead to anisometropic amblyopia, the prevention of which deserves urgent therapeutic intervention. In our patient, therapeutic embolization led to an uncomplicated clinical and angiographic cure. The vascular anatomy, the pathophysiology of associated visual failure, and the treatment modalities are reviewed.