All three patients showed signs of pigment clumping that extended in a band from the vascular arcades to the mid-periphery with a characteristic corresponding band of a decreased signal in AF extending from the vascular arcades to the mid periphery, which is consistent with the literature regarding patients with recessive NR2E3 mutations [3,8,19,35,38,39,40,41]. Here, NR2E3 is linked to atrial fibrillation.