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Advances in multimodal retinal imaging with widefield lenses have dramatically improved patient examination. In a paper entitled ‘‘Double Concentric Autofluorescence Ring in NR2E3p.G56R-Linked Autosomal Dominant Retinitis Pigmentosa’’ and published in this journal in 2012, we reported the presence of a double concentric hyperautofluorescent ring of fundus autofluorescence (FAF) as a highly prevalent and possibly pathognomonic early phenotypic marker for this dominant retinitis pigmentosa (RP). These observations were based on the use of a 558 lens for fundus autofluorescence imaging (Heidelberg Retina Angiograph 2; Heidelberg Engineering, Heidelberg, Germany). At present, ultra-widefield scanning laser ophthalmoscopy allows us to acquire from 1028 (Heidelberg Retina Angiograph 2; Heidelberg Engineering) to 2008 views (ultra-widefield Daytona Optomap; Optos, Dumferline, UK) of the patient’s retina in routine examination. When we recently re-examined the patient affected by NR2E3-p.G56R– linked ADRP described in Figure 7C of the 2012 paper with a 2008 ultra-widefield imaging technology (Optos), we observed a third ring of hyperautofluorescence in the periphery of the retina, in addition to the previously observed double hyperautofluorescent rings (Fig. 1). Therefore, a triple ring of hyperautofluorescence is present in this patient. In 2014, the patient was then 24 years old; imaging of fundus autofluorescence with a 558 lens on a confocal scanning laser revealed no changes in the concentric double ring of autofluorescence we observed with a 308 lens in 2011. The inner hyperautofluorescent ring was still located in the perimacular region and the outer ring around the optic disk and along the vascular arcades (Fig. 1A). The recent reexamination at the age of 26 years with the 2008 retinal imaging system revealed now this third ring of hyperautofluorescence in the periphery (Fig. 1B). We hypothesize that the rim of this hyperautofluorescent ring progresses centripetally toward the midperiphery. Indeed, we observe numerous pigment deposits in the far periphery, suggestive of retinal degeneration having already occurred there. Remarkably, in the midperiphery where the previously reported double hyperautofluorescent ring is present, the outer ring has completely separated from the inner ring in the inferior perimacular region, consistent with our hypothesis that the outer ring progresses centrifugally toward the periphery. To our best knowledge, this is the first report of a triple concentric ring of hyperautofluorescence in RP patients. With a growing number of clinics using widefield retinal imaging systems and given the relatively high incidence of the NR2E3p.G56R mutation among ADRP patients, we anticipate this triple ring to be observed more frequently.

Presence of a Triple Concentric Autofluorescence Ring inNR2E3-p.G56R–Linked Autosomal Dominant Retinitis Pigmentosa (ADRP)