Fig. 1

(a, b) The fundus evaluation revealed widespread outer retinal atrophy with visible large choroidal vessels. Marked temporal sub-retinal fibrosis was noted with foveal atrophy which was more marked in the left eye. (c, d) OCT revealed evidence of outer retinal atrophy with associated sub-foveal scarring. RPE discontinuity can be visualised in the left eye. (e, f) Fundus fluorescein angiogram noted a choroidal hyperfluorescence due to extensive windrow defects, suggestive of RPE loss