The Treatment of Indirect Carotid Cavernous Fistula in a Pediatric Patient With Deep Recurrent Ophthalmic Artery and Hemophilia

The treatment of indirect carotid-cavernous fistula (CCF) poses a unique challenge. Currently, endovascular interventions remain the principal treatment option with high cure rates and acceptable safety profiles. The anatomical characteristics of individual cases determine the optimal vascular access routes (transvenous vs. transarterial), and the devices utilized to achieve therapeutic goals. We present a rare case of an indirect CCF in a 13-year-old patient with an anatomical variant of the ophthalmic artery and hemophilia. CCFs are infrequent in the pediatric population, and an association with a deep recurrent ophthalmic artery (DROA) and hemophilia presents a unique treatment challenge. The patient had been diagnosed with type A hemophilia in 2013. In November 2022, he had presented with insidious conjunctival hyperemia and developed gradual ocular proptosis. He had been referred to Ophthalmology in October 2023, with mild symptoms and no ocular bruit or visual acuity deterioration. Angio CT, angio MRI, and cerebral angiography at that time had shown an indirect CCF. The association of an anatomical variant with the indirect CCF led to adverse anatomy and failed transvenous/transarterial vascular access. Classic treatment options (coils, detachable balloons, and liquid embolic agents) are not feasible when selective microcatheterism is unsuccessful. A FRED flow diverter was placed in the cavernous segment of the internal carotid artery (ICA) as an off-label solution to adverse patient vascular anatomy. Successful device implantation resulted in the gradual resolution of the patient's ocular symptoms.