Pigmented Paravenous Chorioretinal Atrophy: A fortuitous finding in a patient with fronto-ethmoidal mucocoele

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Date

2023

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Wolters Kluwer Medknow

Abstract

A 56-year-old Nigerian male patient presented to the eye clinic with a six-month history of progressive deterioration of vision in the right eye which was preceded by a one-month history of swelling above the right eye. He had no visual complaints in the left eye and no concurrent systemic illnesses. The best corrected visual acuity was hand movement with accurate light projection in the right eye and 6/6 in the left eye. On ocular examination, a right proptosis with inferotemporal displacement was present. A right relative afferent pupillary defect was noted while the left anterior segment examination was normal. Binocular indirect ophthalmoscopy of the right eye revealed a cup-to-disc ratio of 0.6, temporal disc pallor, peripapillary atrophy, and clumps of pigmentation along the venous arcades with generalized chorioretinal atrophy involving the area of the fovea. The cup-to-disc ratio was 0.4 with mild temporal disc pallor and iridescent spots at the macula with similar features of bony spicule-like clumps in the right eye present along the superotemporal arcade in the left eye. Optical coherence tomography (OCT) scan revealed disorganization of the inner retinal layers with intraretinal reflective foci with back-shadowing and both retinal pigment epithelium and choroidal atrophy. The OCT of the left eye showed a few intraretinal reflective foci and retinal pigment epithelium and choroidal atrophy. From the clinical and OCT findings, a diagnosis of pigmented paravenous chorioretinal atrophy and right fronto-ethmoidal mucocele is made.

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Bone spicule-like clumps, chorioretinal atrophy, fronto-ethmoidal mucocele, iridescent spots, paravenous pigmentation

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