Abstract

Our aim is to study the varied posterior segment manifestations, level of visual impairment (VI) and its causes in carotid cavernous fistula (CCF) patients. A retrospective study was done, wherein data was obtained from 48 digital subtraction angiogram (DSA) proven CCF patients. CCF was classified according to Barrow et al., based on DSA into type A (high flow) and types B, C and D (low flow). High flow CCF was present in 8 (16.7%) and low flow CCF was present in 42 (83.3%). Compared to low flow group, patients in high flow group were younger and had a history of trauma (p < 0.05). Posterior segment findings ranged from familiar stasis retinopathy and optic neuropathy (both, glaucomatous and ischemic) to uncommon findings of central retinal artery occlusion, Terson syndrome and combined retinal and choroidal detachment. Retinal vein dilatation was the most common finding in both groups. The high flow CCF group had 6 (75%) patients that had VI. This was acute in 4 (50%) patients and delayed in 2 (25%). In the low flow group 10 (23.8%) of patients had delayed VI. The identification of “3 point sign” is a novel finding of this study, not described before. While none of three findings (disc hyperaemia, retinal vein dilatation and intra-retinal haemorrhage) in isolation were predictive of visual loss, but when present together results in visual loss. Posterior segment changes were varied, some are uncommon and can occur in various combinations. “3 point sign” must be identified at the earliest to prevent visual impairment. The incidence of VI in CCF patients is high.

Highlights

  • Carotid cavernous fistulas (CCFs), spontaneous or traumatic, is an abnormal communication between the cavernous sinus and the carotid arterial system[1]

  • We report the posterior segment manifestations in 48 consecutive cases of CCFs which were proven and classified on Digital subtraction angiography (DSA)

  • Tan et al found that the high flow group constituted 18% of the total 45 angiograms proven CCFs patients cohort[10]

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Summary

Introduction

Carotid cavernous fistulas (CCFs), spontaneous or traumatic, is an abnormal communication between the cavernous sinus and the carotid arterial system[1]. Because of the diverse clinical signs and symptoms with a long list of differentials, CCFs remains undiagnosed in many patients[4]. This can lead to significant morbidity and mortality including visual impairment (VI). These changes have been described, these are either part of an isolated case report or smaller series where the diagnosis had not been confirmed on DSA7,8 Their incidence and contribution to VI in CCF patients were not analyzed in a large series. We studied the posterior segment manifestations in 48 consecutive cases of CCF, which were proven and classified on Digital subtraction angiography (DSA). We excluded patients who were suspected to have CCF (on clinical evaluation and initial imaging studies) but did not undergo a DSA and www.nature.com/scientificreports/

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