Fig. 3
From: Retinal and optic nerve degeneration in α-mannosidosis
Tapeto-retinal degeneration in α-mannosidosis in two siblings. Fundus photographs (a, b) of the brother showed early partial optic nerve atrophy (blue arrow, b) but without any yellow-white or pigmented deposits, besides a more visible choroid around the optic nerve head (black stars, b). Optical coherence tomography (OCT) showed a perifoveal thinning of the outer retinal layers and retinal pigment epithelium (RPE, red arrows, c, d) with normal retinal layers in the fovea (green bracket, c, d). However, outer retina thinning progressed over time (smaller green bracket, d) with a larger perifoveal area of outer retinal atrophy (red arrows, d)