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Multimodal imaging of a case of peripheral cone dystrophy.

Ito N, Kameya S, Gocho K, Hayashi T, Kikuchi S, Katagiri S, Gekka T, Yamaki K, Takahashi H, Tsuneoka H - Doc Ophthalmol (2015)

Bottom Line: Fluorescein and indocyanine green angiographies did not show any hyper- or hypofluorescent areas of the retina.The scotopic full-field ERGs were normal, but the photopic ERGs were markedly reduced.The parafoveal cone densities were severely decreased in both eyes.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, The Jikei University School of Medicine, 3-25-8 Nishi-shimbashi, Minato-ku, Tokyo, 105-8461, Japan.

ABSTRACT

Purpose: To characterize the peripheral cones in the images obtained by spectral-domain optical coherence tomography (OCT), swept source OCT, and adaptive optics fundus camera in a patient with peripheral cone dystrophy.

Methods: A 28-year-old Japanese man underwent detailed ophthalmic evaluations including high-resolution imaging of the fundus of both eyes.

Results: The decimal best-corrected visual acuity was 1.2 in both eyes. The results of slit-lamp biomicroscopy and ophthalmoscopy were essentially normal. Fluorescein and indocyanine green angiographies did not show any hyper- or hypofluorescent areas of the retina. Goldmann perimetry showed full peripheral visual fields but relative central scotomas within the central 20°. The results of the Humphrey Visual Field Analyzer showed a limited preservation of the central sensitivity. Color vision tests showed no errors in both eyes. Spectral-domain OCT showed attenuation of both the ellipsoid and interdigitation zones throughout the macular region except the center of the fovea. The scotopic full-field ERGs were normal, but the photopic ERGs were markedly reduced. Regular cone mosaics were not observed especially more than 450 μm radius from the fovea in the adaptive optics retinal images. The parafoveal cone densities were severely decreased in both eyes.

Conclusions: Our findings indicate that the peripheral cone dystrophy diagnosed by full-field ERGs and perimetry is due to a reduction in the density of parafoveal and peripheral cones.

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SD-OCT and high-magnification AO images. Magnified SD-OCT images of a normal control (a) and PCD patient (b) are shown. Yellow boxes in a and b are nasal areas at 600, 450, and 300 μm away from the foveal center. A montage of low-magnification AO image of normal control (c) and the patient (d) is shown. Yellow boxes in c and d are also nasal areas at 600, 450, and 300 μm away from the foveal center (f). (e–j) Magnified view of the area outlined in c and d is shown. The area at 600 μm (e), 450 μm (f), and 300 μm (g) nasal from the foveal center in normal control is shown. The area at 600 μm (h), 450 μm (i), and 300 μm (j) nasal from the foveal center in the patient is shown. Regular cone mosaics are not observed especially more than 450 μm from the fovea in the patient, while AO image of normal control shows well-ordered cone mosaic. Bar 100 μm
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Fig6: SD-OCT and high-magnification AO images. Magnified SD-OCT images of a normal control (a) and PCD patient (b) are shown. Yellow boxes in a and b are nasal areas at 600, 450, and 300 μm away from the foveal center. A montage of low-magnification AO image of normal control (c) and the patient (d) is shown. Yellow boxes in c and d are also nasal areas at 600, 450, and 300 μm away from the foveal center (f). (e–j) Magnified view of the area outlined in c and d is shown. The area at 600 μm (e), 450 μm (f), and 300 μm (g) nasal from the foveal center in normal control is shown. The area at 600 μm (h), 450 μm (i), and 300 μm (j) nasal from the foveal center in the patient is shown. Regular cone mosaics are not observed especially more than 450 μm from the fovea in the patient, while AO image of normal control shows well-ordered cone mosaic. Bar 100 μm

Mentions: High-resolution en face AO imaging did not detect regular cone mosaics especially more than 450 μm from the fovea, while the AO images of normal control showed well-ordered cone mosaic in this area (Figs. 5b, 6c–j). Similar results were observed in both eyes. The oval area of residual cone photoreceptors in the AO image was similar to the shape of residual IZ in the en face OCT image (Fig. 3f and between blue arrows in Fig. 5b).Fig. 5


Multimodal imaging of a case of peripheral cone dystrophy.

Ito N, Kameya S, Gocho K, Hayashi T, Kikuchi S, Katagiri S, Gekka T, Yamaki K, Takahashi H, Tsuneoka H - Doc Ophthalmol (2015)

SD-OCT and high-magnification AO images. Magnified SD-OCT images of a normal control (a) and PCD patient (b) are shown. Yellow boxes in a and b are nasal areas at 600, 450, and 300 μm away from the foveal center. A montage of low-magnification AO image of normal control (c) and the patient (d) is shown. Yellow boxes in c and d are also nasal areas at 600, 450, and 300 μm away from the foveal center (f). (e–j) Magnified view of the area outlined in c and d is shown. The area at 600 μm (e), 450 μm (f), and 300 μm (g) nasal from the foveal center in normal control is shown. The area at 600 μm (h), 450 μm (i), and 300 μm (j) nasal from the foveal center in the patient is shown. Regular cone mosaics are not observed especially more than 450 μm from the fovea in the patient, while AO image of normal control shows well-ordered cone mosaic. Bar 100 μm
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Related In: Results  -  Collection

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Fig6: SD-OCT and high-magnification AO images. Magnified SD-OCT images of a normal control (a) and PCD patient (b) are shown. Yellow boxes in a and b are nasal areas at 600, 450, and 300 μm away from the foveal center. A montage of low-magnification AO image of normal control (c) and the patient (d) is shown. Yellow boxes in c and d are also nasal areas at 600, 450, and 300 μm away from the foveal center (f). (e–j) Magnified view of the area outlined in c and d is shown. The area at 600 μm (e), 450 μm (f), and 300 μm (g) nasal from the foveal center in normal control is shown. The area at 600 μm (h), 450 μm (i), and 300 μm (j) nasal from the foveal center in the patient is shown. Regular cone mosaics are not observed especially more than 450 μm from the fovea in the patient, while AO image of normal control shows well-ordered cone mosaic. Bar 100 μm
Mentions: High-resolution en face AO imaging did not detect regular cone mosaics especially more than 450 μm from the fovea, while the AO images of normal control showed well-ordered cone mosaic in this area (Figs. 5b, 6c–j). Similar results were observed in both eyes. The oval area of residual cone photoreceptors in the AO image was similar to the shape of residual IZ in the en face OCT image (Fig. 3f and between blue arrows in Fig. 5b).Fig. 5

Bottom Line: Fluorescein and indocyanine green angiographies did not show any hyper- or hypofluorescent areas of the retina.The scotopic full-field ERGs were normal, but the photopic ERGs were markedly reduced.The parafoveal cone densities were severely decreased in both eyes.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, The Jikei University School of Medicine, 3-25-8 Nishi-shimbashi, Minato-ku, Tokyo, 105-8461, Japan.

ABSTRACT

Purpose: To characterize the peripheral cones in the images obtained by spectral-domain optical coherence tomography (OCT), swept source OCT, and adaptive optics fundus camera in a patient with peripheral cone dystrophy.

Methods: A 28-year-old Japanese man underwent detailed ophthalmic evaluations including high-resolution imaging of the fundus of both eyes.

Results: The decimal best-corrected visual acuity was 1.2 in both eyes. The results of slit-lamp biomicroscopy and ophthalmoscopy were essentially normal. Fluorescein and indocyanine green angiographies did not show any hyper- or hypofluorescent areas of the retina. Goldmann perimetry showed full peripheral visual fields but relative central scotomas within the central 20°. The results of the Humphrey Visual Field Analyzer showed a limited preservation of the central sensitivity. Color vision tests showed no errors in both eyes. Spectral-domain OCT showed attenuation of both the ellipsoid and interdigitation zones throughout the macular region except the center of the fovea. The scotopic full-field ERGs were normal, but the photopic ERGs were markedly reduced. Regular cone mosaics were not observed especially more than 450 μm radius from the fovea in the adaptive optics retinal images. The parafoveal cone densities were severely decreased in both eyes.

Conclusions: Our findings indicate that the peripheral cone dystrophy diagnosed by full-field ERGs and perimetry is due to a reduction in the density of parafoveal and peripheral cones.

Show MeSH
Related in: MedlinePlus