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.

Precis: Secondary ocular hypertension (OHT) is common in carotid-cavernous fistulas (CCFs). Management of elevated intraocular pressure (IOP) is possible with a multidisciplinary approach. The ipsilateral normal eyes may have higher IOP than the contralateral eyes. Purpose: To study the IOP profile of the eyes of patients with a CCF, treatment outcomes for elevated IOP, and intereye IOP asymmetry in the eyes with normal IOP. Methods: This was a retrospective case series. A total of 64 eyes of 60 patients with digital subtraction angiography-proven CCF diagnosed from the year 2000 to 2016 were included. The demographics, clinical features, management, and outcomes were recorded. The primary outcome included understanding of the cause of elevated IOP. The secondary outcomes included comparison of the IOP between contralateral eyes and ipsilateral normal eyes (IOP <21 mm Hg) and management outcomes for elevated IOP. Results: The mean age of the patients was 45.6±18.2 years. In the study population, 70% of the patients were males. Indirect CCF was present in 55% of the eyes. It was found that 64.06% (n=41) of the eyes had elevated IOP, glaucoma, or were glaucoma suspects. Among all the eyes, 40.62% (n=26) of the eyes had secondary OHT due to elevated episcleral venous pressure, whereas 7.81% (n=5) of the eyes had secondary open-angle glaucoma. The mean IOP was higher in the ipsilateral eyes than in the other eyes (22.95±7.1vs. 15.11±2.99 mm Hg; P<0.001). The mean IOP in the ipsilateral normal eyes was higher than that in the contralateral eyes, with a mean difference of 2.92±2.29 mm Hg (confidence interval of the mean difference: 1.90-3.94 mm Hg; P<0.0001). IOP reduction (<21 mm Hg) was achieved in 70.7% of the patients following CCF management with intermittent carotid massage, endovascular treatment, IOP-lowering medications, or a combination among these. Conclusions: Secondary OHT due to elevated episcleral venous pressure was more common than secondary open-angle glaucoma. Ipsilateral normal eyes had higher IOP than contralateral eyes. IOP-lowering agents and management of CCF resulted in IOP control in most patients.

Periorbital arteriovenous malformations (AVMs) are congenital lesions that may cause significant morbidity such as amblyopia, cosmetic disfigurement, or chronic pain. Due to the rarity of these lesions, they are frequently misdiagnosed and treated inappropriately. We managed a 6-year-old girl with preseptal AVM by endovascular embolization followed by complete surgical excision after 2 days. She was previously diagnosed as capillary hemangioma and was being treated with intralesional steroid injections and oral propranolol. Neuroimaging revealed an AVM fed by the branches of both external and internal carotid arteries and drained by the superior ophthalmic vein. Endovascular embolization with glue followed by complete surgical excision of the lesion was done. The resultant cosmetic and functional outcome was gratifying. Diagnosis and management of periorbital AVM remain a challenge requiring a multidisciplinary approach involving interventional radiologist and trained oculoplastic surgeons. Combined endovascular embolization followed by surgical excision proved safe and effective.