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. 2020 Feb;210:59–70. doi: 10.1016/j.ajo.2019.10.019

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© 2019 The Author(s)

This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

PMC Copyright notice

Optical coherence tomography (OCT) and fundus autofluorescence (FAF) imaging of 4 patients (P1, P4, P2B, P3). OCT findings at baseline were grouped into 4 different grades based on ellipsoid zone (EZ) integrity and retinal pigment epithelium (RPE) changes: (A) grade 1: continuous/intact EZ; (B) grade 2: focally disrupted EZ; (C) grade 3: focally disrupted EZ with RPE changes; and (D) grade 4: diffuse EZ and RPE changes. The arrowheads point to the attenuated EZ. The right column presents the corresponding FAF images: (A) normal FAF appearance; (B) central early hypoautofluorescence with small central foci of hyperautofluorescence; (C) normal FAF images apart from central small areas of hypoautofluorescence; and (D) central and mid-peripheral hypoautofluorescence. The white arrows mark the border of the corresponding OCT line scans. VA = visual acuity; yo = years old.