Fig. 4

Butterfly shaped macular dystrophy in a transfusion dependent 63 year old patient receiving deferoxamine chelation therapy due to sickle beta thalassemia (Patient 2). Fundus autofluorescence shows hyperautofluorescence in a branching pattern with some areas of reduced autofluorescence corresponding to the pigment clumping seen in fundoscopy OU (a, b). Fluoroscein angiography reveals central hypofluorescence with adjacent areas of focal hyperfluorescence OU, that persists as staining without leakage in the late frames (c, d)

Optical coherence tomography at the time of diagnosis showing hyperreflective material in the outer retinal layers, located at the level and above the RPE in both maculas (e, f). There is evident disruption of the ellipsoid zone by the amorphous material in the left eye. After 4 years, follow up OCT remains unchanged OS while there is a slight increase of the subfoveal debris in OD. (g, h)