PMC3659265_01
Photographs of the fundus (A and B) and fluorescein angiography (C and D) of both eyes of a patient with occult macular dystrophy.. Abbreviations: R, right eye; L, left eye.
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C chart. Color fundus photographs and fluorescein angiography showed normal findings in both eyes (Figure 1).
Images of multifocal electroretinograms (ERGs) (A, B, D and E), and full-field ERG (C and F). In the full-field ERG (C and F), 1 shows the graph for the rod ERG, 2 that for the flash ERG, 3 that for the 30 Hz flicker ERG, and 4 that for the cone ERG.. Note: The circles of A and D show the response of the central 8 areas. The scales (C and F) represent 10 ms (horizontal axis) and 100 muV (vertical axis).. Abbreviations: R, right eye; L, left eye; N, nasal; T, temporal.
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Multifocal ERG showed reduced amplitudes in the central ring of both eyes (right eye 25.62 nV/deg2, left eye 11.80 nV/deg2) (Figure 2A, B, D and E), while scotopic and photopic full-field ERGs showed normal responses (Figure 2C and F). The responses of the central eight areas of multifocal ERG (circles in Figure 2A and D) showed a similar decrease between the nasal and the temporal retina in both eyes.
Images of spectral domain optical coherence tomography (A and C) and adaptive optics (B and D) at the fovea of the OMD patient.. Notes: Images for the right eye are shown in A and B, and those for the left eye are presented in C and D. Panoramic adaptive optics images of B the right eye (6.25 x 0.95 mm), and D the left eye (6.0 x 1.04 mm). The arrowheads in A and C show areas of observable IS/OS line and COST line at the fovea. The arrowheads in B and D show the corresponding areas in AO images. The long arrow in B and D shows foveal center. The short arrows in A and C show the areas with a reduced IS/OS line and unclear COST line. An asterisk in C shows defect of IS/OS line and COST line. Bar = 500 mum.. Abbreviations: OMD, occult macular dystrophy; IS/OS, inner- and outer-segment; COST, cone outer-segment tips; AO, adaptive optics.
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An SD-OCT image revealed a relatively normal IS/OS line and cone outer-segment tips (COST) line at the fovea in the right eye (arrowheads in Figure 3A), with a reduced IS/OS line and unclear COST line in the outside of the fovea (arrows in Figure 3A). A panoramic AO image of the right eye (6.25 x 0.95 mm) showed an extensive loss of cone photoreceptors in the entire area examined, with a ring-like area of residual cone photoreceptors at the fovea (arrowheads in Figure 3B). However, cone photoreceptors were observed at the foveal center (arrow in Figure 3B). SD-OCT image revealed a discontinuous IS/OS line and COST line at the foveal center in the left eye (asterisk in Figure 3C). Similar to the findings in the right eye, the IS/OS line and the COST line in the left eye were observed at the fovea (arrowheads in Figure 3C), with a reduced IS/OS line and unclear COST line in the outside of the fovea (arrows in Figure 3C). AO image of the left eye (6.0 x 1.04 mm) showed an extensive loss of cone photoreceptors in the entire area, with a ring-like region of residual cone photoreceptors at the fovea (arrowheads in Figure 3D). A dark area was observed at the foveal center without cone mosaics (arrow in Figure 3D), and this area corresponded with the area exhibiting loss of the IS/OS line and COST line in the SD-OCT image (asterisk in Figure 3C).
Panoramic adaptive optics images of the left eye of a healthy control (A, 6.51 x 0.96 mm), the right eye of the OMD patient (C, 6.3 x 1.03 mm), left eye of the OMD patient (E, 6.51 x 0.96 mm), and the corresponding cone-density maps (B, D and F), respectively. Eight 100 x 100 mum areas at the nasal or temporal retina were selected (A, C and E).. Notes: The foveal center is indicated by the asterisk in B. The arrowheads in D and F show a ring-like dark area around the fovea with lower cone densities than the peripheral areas in the nasal or temporal retina. Bar = 500 mum. A color scale of cone density is shown at the bottom.. Abbreviations: OMD, occult macular dystrophy; N, nasal; T, temporal.
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Eight 100 x 100 mum areas at the nasal or temporal retina were selected (avoiding large retinal vessels) in the panoramic AO images (Figure 4A, C and E). AO image of a healthy control left eye (6.51 x 0.96 mm) are shown in Figure 4A, and the corresponding cone-density panoramic map is shown in Figure 4B. Table 1. The data were expressed as the mean +- standard deviation. As shown in Figure 4B, the cone densities at the foveal center were lower than those at the outside of the foveal center (asterisk in Figure 4B). This was because the resolution of the AO fundus camera was not sufficient to distinguish each cone photoreceptor at the foveal center. Therefore, we did not perform cone analysis at the foveal center in this study. AO images of both eyes. A panoramic AO image (6.3 x 1.03 mm) of the right eye is shown in Figure 4C, and the corresponding cone-density panoramic map is shown in Figure 4D. High cone densities were observed at the fovea, while a ring-like area around the fovea showed lower cone densities than the peripheral areas in the nasal or temporal retina (arrowheads in Figure 4D). A panoramic AO image (6.51 x 0.96 mm) of the left eye is shown in Figure 4E, and the corresponding cone-density panoramic map is shown in Figure 4F. The left eye showed a ring-like area of low cone densities around the fovea, and this area was similar to, but larger than, that of the right eye (arrowheads in Figure 4F).
Cone analysis in the eight selected areas of the OMD patient. (A right eye, B left eye).. Notes: Color maps of cone density, cone spacing, and Voronoi analysis are shown. Color scales for each analysis are shown at the bottom.. Abbreviations: OMD, occult macular dystrophy; N, nasal; T, temporal.
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Eight areas were analyzed with the software, and the color maps and results are shown in Figure 5A (right eye) and B (left eye) and Table 1.