Macular edema and neovascularization in a case of Stargardt macular dystrophy

Abstract

Purpose To present a case of macular edema and neovascularization in a patient who was diagnosed with Stargardt macular dystrophy. Methods We present the case of a 22 year old male who experienced visual deterioration in his left eye. At presentation his visual acuity was OD 8‐9/10 and OS 6‐7/10. OCT scan, fluoroangiography (FA) and electroretinografy (ERG), were performed. Results OCT scan revealed cystoid macular edema in the right eye, and increased reflectivity of the pigment epithelium and some subretinal fluid in the left eye. FA findings were consistent with Stargardt dystrophy. However in the right eye an area of increasing hyperfluorescence was observed (presence of neovascularisation). ERG: subnormal photopic and scotopic responses. The color vision was impaired mainly in the left eye. Following the experience of other investigators acetazolamide was administered orally (300 mg per day) and resolution of the cystoid edema was obvious in OCT scan after 20 days. The visual acuity increased (OD 10/10 and OS 8/10). Two weeks after the discontinuation of acetolozamide the macular edema reappeared and the visual acuity diminished: OD 8‐9/10 and OS 6‐7/10. Conclusion This is a peculiar case of cystoid macular edema and neovascularization in Stargardt macular dystrophy. Our findings suggest, in consistency with other investigators, that administration of oral acetozolamide may be beneficial for the resolution of macular edema. The neovascularization in the left eye remained stable during the follow up period and it was considered that any intervention was not necessary at that time.