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. 2013 Sep 25;24(12):993–1006. doi: 10.1089/hum.2013.153

FIG. 2.

FIG. 2.

Younger BCM patients show normal RPE but abnormal thinning of the photoreceptor nuclear layer. (A) En face imaging of melanin and lipofuscin fluorophores of the RPE using NIR-RAFI and SW-RAFI, respectively, in a normal subject (age 29; myope, −6D) and three BCM patients. The small dark foveal region in the normal and P1 on SW-RAFI is the result of absorption of the excitation light by dense macular pigment; the larger dark region in the central macula of P4 on NIR-RAFI and SW-RAFI represents RPE atrophy. Retinal blood vessels and the optic nerve head also appear darker than the background. All images are shown contrast stretched for visibility of features. (B) Cross-sectional scans along the vertical meridian crossing the fovea in the same four eyes as shown in (A). ONL, where the photoreceptor nuclei reside, is highlighted (blue). Vertical dashed lines delineate the foveola dominated by cone photoreceptors, 0.8 mm superior retina with cone and rod photoreceptors, and the rod hot spot dominated by rod photoreceptors. (C) ONL thickness vertically across the central 10 mm of retina is graphically displayed for a group of normal subjects (gray represents mean±2SD; n=22; age range 8–62 years), and the BCM data (lines). (D) ONL thickness at the foveola (upper), 0.8 mm superior retina (middle), and the rod hot spot (lower) as a function of age in patients (colored symbols: green, age <10; orange, age range 10–20; brown, age >20 years) and normal controls (gray symbols). The 95% prediction interval of linear regression fit to the normal data is shown (gray dashed lines). Symbols connected with lines represent patients with multiple visits. (E) The extent of ONL abnormality along the vertical meridian as a function of age. Individual patient data are displayed as in (D). Average results for the three age groups are shown with colored circles. Inset, schematic of the average extent of ONL abnormality for the three age groups overlaid to scale on a representative retinal view. ONL, outer nuclear layer; RPE, retinal pigment epithelium.

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