Fig. 3.

Disease-sparing of the peripapillary region in ABCA4-associated retinopathy. Sparing of the peripapillary region around the optic nerve (magenta arrowheads) from disease changes is a characteristic feature of ABCA4-associated retinopathy and becomes apparent as flecks extend centripetally across the posterior pole of the retina (A, B). Resistance of this region persists into the late atrophic stages and gradually becomes affected (C). The presence of residual circumpapillary tissue may be discernible despite the widespread atrophy (D).