Figure 1.
(A) Adaptive optics scanning laser ophthalmoscopy (AO-SLO) image registered on color fundus photograph. (B) Fundus autofluorescence (FAF) imaging. (C) Montage of a series of high-resolution images from the central fovea outward to 1.5 mm from the center of the fovea, obtained by AO-SLO. Asterisk = foveal center. First, 3 different field of view images (L [yellow box]: 1700×1700 µm, M [green box]: 820×820 µm, S [white box]: 340×340 µm) centered on the center of the fovea were obtained, followed by 2 field of view images (M and S) centered 0.5 mm and 1.0 mm from the center of the fovea at each direction (superior, nasal, inferior, temporal) (D and E) Representative images of areas 0.5 mm (D) and 1.0 mm (E) from the center of the fovea (340 µm×340 µm area).
Figure 2.
Cone labeling and cone density/arrangement measurement.
(A) A cone mosaic image 0.5 mm from the center of the fovea in a normal eye. (B) Cone labeling results. (C) Voronoi diagram. The colors indicate the number of sides of each Voronoi polygon (pink, 4; blue, 5; green, 6; yellow, 7; and orange, 8). (D) A cone mosaic image 0.5 mm from the center of the fovea in an eye with retinitis pigmentosa (RP). (E) Cone labeling results. (F) Voronoi diagram. The colors indicate the number of sides of each Voronoi polygon (pink, 4; blue, 5; green, 6; yellow, 7; and orange, 8). The cone densities are 27,275 and 13,102 cones/mm2 in the normal eye and the eye with RP, respectively. Proportions of 6-sided Voronoi polygons were 54.1% and 40.0% in the normal eye and the eye with RP, respectively. The ratio of observed average nearest-neighbor distance (NND) for each subject divided by expected NND was 0.795 and 0.723 in the normal eye and the eye with RP, respectively.
Figure 3.
Spectral-domain optical coherence tomography measurement.
Measurements included the thickness of the outer nuclear layer (ONL), which was measured between the vitreoretinal interface and external limiting membrane (ELM), the photoreceptor inner segment and outer segment (IS+OS) thickness, measured between the ELM and retinal pigment epithelium (RPE), and the total central foveal thickness.
Table 1.
Clinical characteristics of patients with retinitis pigmentosa.
Figure 4.
Retinitis Pigmentosa Case (Case 8).
Images of the left eye of a 33-year-old man with RP (Case 8). Snellen equivalent best-corrected visual acuity (BCVA) was 20/15. (A) Fundus photograph shows attenuation of retinal vessels and mottling and granularity of the retinal pigment epithelium. (B) FAF image shows hypofluorescenct lesions outside the macula, but normal within the macula. (C) Infrared image with green arrows indicating the directions of scans shown in D and E, and a white box indicating the area scanned by AO-SLO. (D) Total deviation of Humphrey Field Analyzer (10-2 SITA standard program). Blue box indicates the central 4 points. (E) Horizontal SD-OCT line scan through the fovea. (F) Vertical SD-OCT line scan through the fovea. Blue arrowheads indicate 0.5 mm from the center of the fovea, and yellow arrowheads indicate 1.0 mm area from the center of the fovea. IS ellipsoid is remaining in the area between arrows. Red double-headed arrows indicate the area corresponding to the area scanned by AO-SLO.
Figure 5.
Adaptive Optics Scanning Laser Ophthalmoscopy Image of Case 8.
(A) OCT image in a high magnification view of horizontal scan corresponding to the area scanned by AO-SLO. (B) OCT image in a high magnification view of vertical scan corresponding to the area scanned by AO-SLO. Blue arrowheads indicate 0.5 mm from the center of the fovea, and yellow arrowheads indicate 1.0 mm area from the center of the fovea. (C) AO-SLO montage image. The images show cones with a mostly regular mosaic pattern with small dark areas. Small dark areas are seen even in the area where the IS ellipsoid is continuous on SD-OCT (Fig. 4). (D) A high-magnification image at 0.5 mm in the nasal direction from the center of the fovea. (E) A high-magnification image at 0.5 mm in the inferior direction from the center of the fovea. (F) A high-magnification image at 1.0 mm in the inferior direction from the center of the fovea. The asterisk indicates the foveal center.
Figure 6.
Retinitis Pigmentosa Case (Case 14).
Images of the right eye of a 35-year-old female with RP (Case 14). Snellen equivalent BCVA was 20/15. (A) Fundus photograph shows attenuation of retinal vessels and mottling and granularity of the retinal pigment epithelium. (B) FAF image shows swirls of hyperautofluorescence in the macula. (C) Infrared image with green arrows indicating the directions of scans shown in E and F, and a white box indicating the area scanned by AO-SLO. (D) Total deviation of Humphrey Field Analyzer (10-2 SITA standard program). Blue box indicates the central 4 points. (E) Horizontal SD-OCT line scan through the fovea. (F) Vertical SD-OCT line scan through the fovea. Blue arrowheads indicate 0.5 mm from the center of the fovea, and yellow arrowheads indicate 1.0 mm from the center of the fovea. Note that the IS ellipsoid is almost continuous in each scan. Red double-headed arrows indicate the area corresponding to the area scanned by AO-SLO.
Figure 7.
Adaptive Optics Scanning Laser Ophthalmoscopy Image of Case 14.
Images of Case 14. (A) OCT image in a high magnification view of horizontal scan corresponding to the area scanned by AO-SLO. (B) OCT image in a high magnification view of vertical scan corresponding to the area scanned by AO-SLO. Blue arrowheads indicate 0.5 mm from the center of the fovea, and yellow arrowheads indicate 1.0 mm area from the center of the fovea. (C) AO-SLO images of Case 14. The images show cones with patchy dark areas representing cone loss. Dark areas are seen even in the area where the IS ellipsoid is continuous on SD-OCT (Fig. 6). (D) A high-magnification image at 0.5 mm in the inferior direction from the center of the fovea. (E) A high-magnification image at 0.5 mm in the temporal direction from the center of the fovea. (D) A high-magnification image at 1.0 mm in the temporal direction from the center of the fovea. The asterisk indicates the foveal center.
Figure 8.
Retinitis Pigmentosa Case (Case 12).
Images of the left eye of a 63-year-old female with RP (Case 12). Snellen equivalent BCVA was 20/15. (A) Fundus photograph shows attenuation of retinal vessels and mottling and granularity of the retinal pigment epithelium. (B) FAF image shows a hyperautofluorescent ring surrounded by a hypoautofluorescent ring in the macula. (C) Infrared image with green arrows indicating the directions of scans shown in E and F, and a white box indicating the area scanned by AO-SLO. (D) Total deviation of Humphrey Field Analyzer (10-2 SITA standard program). Blue box indicates the central 4 points. (E) Horizontal SD-OCT line scan through the fovea. (F) Vertical SD-OCT line scan through the fovea. Blue arrowheads indicate 0.5 mm from the center of the fovea, and yellow arrowheads indicate 1.0 mm from the center of the fovea. The IS ellipsoid is remaining in the area between arrows. Red double-headed arrows indicate the area corresponding to the area scanned by AO-SLO.
Figure 9.
Adaptive Optics Scanning Laser Ophthalmoscopy Image of Case 12.
Images of Case 12. (A) OCT image in a high magnification view of horizontal scan corresponding to the area scanned by AO-SLO. (B) OCT image in a high magnification view of vertical scan corresponding to the area scanned by AO-SLO. Blue arrowheads indicate 0.5 mm from the center of the fovea, and yellow arrowheads indicate 1.0 mm area from the center of the fovea. (C) AO-SLO montage images of Case 12. The images show a large dark annular lesion (arrows) where cones are missing, which corresponds to the area where IS ellipsoid is disrupted on SD-OCT (Fig. 8). (D) A high-magnification image at 0.5 mm in the superior direction from the center of the fovea. (E) A high-magnification image at 0.5 mm in the inferior direction from the center of the fovea. (F) A high-magnification image at 1.0 mm in the temporal direction from the center of the fovea. The asterisk indicates the foveal center.
Table 2.
The inter-later reliability of intraclass correlation coeffients for the two observers on AO-SLO measurements.
Table 3.
Cone density and arrangement in eyes with retinitis pigmentosa vs. normal eyes.
Table 4.
Cone density in each region in eyes with retinitis pigmentosa vs. normal eyes.
Table 5.
Cone density and arrangement on AO-SLO vs. photoreceptor status on SD-OCT in eyes with retinitis pigmentosa (0.5 mm from center of the fovea).
Table 6.
The inter-later reliability of intraclass correlation coeffients for the two observers on SD-OCT measurements.
Table 7.
Correlation between the result of AO-SLO or SD-OCT and retinal sensitivity in eyes with retinitis pigmentosa (0.5 mm from center of the fovea).
Table 8.
Correlation between cone density/arrangement and OCT findings in eyes with retinitis pigmentosa.