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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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Statistical data of adaptive optics analyses. Relationship between cone density and axial length was obtained from 16 normal control samples. There was a statistically significant negative correlation between cone density and axial length (R2 = 0.6532). Upper and lower limits of 95 % confidence intervals, 95 % prediction intervals, and regression line of normal controls are shown. The results of estimated cone density and axial length of the patient are plotted as indicated marks
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Fig7: Statistical data of adaptive optics analyses. Relationship between cone density and axial length was obtained from 16 normal control samples. There was a statistically significant negative correlation between cone density and axial length (R2 = 0.6532). Upper and lower limits of 95 % confidence intervals, 95 % prediction intervals, and regression line of normal controls are shown. The results of estimated cone density and axial length of the patient are plotted as indicated marks

Mentions: We also examined the cone density at 600 μm nasal from the fovea and the axial length of 16 normal control eyes. The values were compared to the findings in the cone density at 600 μm nasal from the fovea in the patient (Fig. 7). It is well known that there is a significant negative correlation between the axial length and cone density [24, 25], and the correlation coefficient was 0.6532 for the normal controls. The mean and standard deviation of the cone density of this normal control group were 262.8 ± 38.5 (×100 cones/mm2). The cone densities of both eyes in the patient were severely decreased and well outside the standard deviation and 95 % prediction interval of the normal controls (Fig. 7).Fig. 7


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)

Statistical data of adaptive optics analyses. Relationship between cone density and axial length was obtained from 16 normal control samples. There was a statistically significant negative correlation between cone density and axial length (R2 = 0.6532). Upper and lower limits of 95 % confidence intervals, 95 % prediction intervals, and regression line of normal controls are shown. The results of estimated cone density and axial length of the patient are plotted as indicated marks
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4555344&req=5

Fig7: Statistical data of adaptive optics analyses. Relationship between cone density and axial length was obtained from 16 normal control samples. There was a statistically significant negative correlation between cone density and axial length (R2 = 0.6532). Upper and lower limits of 95 % confidence intervals, 95 % prediction intervals, and regression line of normal controls are shown. The results of estimated cone density and axial length of the patient are plotted as indicated marks
Mentions: We also examined the cone density at 600 μm nasal from the fovea and the axial length of 16 normal control eyes. The values were compared to the findings in the cone density at 600 μm nasal from the fovea in the patient (Fig. 7). It is well known that there is a significant negative correlation between the axial length and cone density [24, 25], and the correlation coefficient was 0.6532 for the normal controls. The mean and standard deviation of the cone density of this normal control group were 262.8 ± 38.5 (×100 cones/mm2). The cone densities of both eyes in the patient were severely decreased and well outside the standard deviation and 95 % prediction interval of the normal controls (Fig. 7).Fig. 7

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