A Rare Case of Direct Spontaneous Carotid-Cavernous Fistula in a 6-Month-Old Infant and Review of the Literature

Abstract

Direct or type A CCFs are the direct connection between the cavernous segment of the internal carotid artery and the cavernous sinus. While most direct CCFs are caused by trauma, spontaneous direct CCFs are extremely rare in infants. In this report, we describe a 6-month-old child with bulges in the right eye that had been present since 20 days after birth. On examination, there was a right eye abduction limitation with no deviation associated with proptosis. Bruit was present during auscultation. CEMRI showed an enlarged right cavernous sinus with dilatation of the superior ophthalmic vein, suggesting a carotid-cavernous fistula. The patient was referred to an advanced center where he was advised patch therapy to prevent amblyopia. He was kept under observation by a neurosurgeon until 3 years, after which he was scheduled to undergo transarterial coiling.