A chondromyxoid fibroma of the orbit with fundus autofluorescence in choroidal folds

Abstract

A 35-year-old woman presented at Advanced Eye Centre, PGIMER, Chandigarh, with left, painless proptosis and diminution of vision of 8 months duration. On examination, left inferior dystopia, proptosis of 4 mm on exophthalmometry, and gross upgaze restriction were noted. Palpation in the left superotemporal orbit revealed a firm, nontender orbital mass. Slit lamp fundus examination revealed multiple curvilinear lines that crossed the macula, superior to the left optic disc (A), and fundus autofluorescence revealed a hypo-hyper autofluorescent “zebra” pattern of lines highlighting the level beneath the retinal pigment epithelium choroid (B). A diagnosis of superior orbital mass with choroidal folds was made. Orbital computed tomography (C) revealed scleral indentation by the orbital mass (15 × 13 mm). Histopathology of the excised mass revealed a rare chondromyxoid fibroma of the orbit. The pathophysiology of chorioretinal folds includes wrinkling of Bruch’s membrane, retinal pigment epithelium, and adjacent choroid layers.