Three-dimensional Distribution of the Vitelliform Lesion, Photoreceptors, and Retinal Pigment Epithelium in the Macula of Patients With Best Vitelliform Macular Dystrophy

Human Photoreceptor Outer Segments Shorten During Light Adaptation

“…All normal subjects exhibited the same pattern of thickening in the dark and thinning in the light; and, in the one subject who was imaged repeatedly over the course of several months, these measurements were reproducible over time. Third, histological analysis of an eye from a Best disease donor revealed an increased OSEL in the macular area outside the vitelliform lesion, which agrees with our previous 3-D SD-OCT analyses of multiple subjects affected by Best disease 28 (Fig. 1).…”

Human Photoreceptor Outer Segments Shorten During Light Adaptation

“…All normal subjects exhibited the same pattern of thickening in the dark and thinning in the light; and, in the one subject who was imaged repeatedly over the course of several months, these measurements were reproducible over time. Third, histological analysis of an eye from a Best disease donor revealed an increased OSEL in the macular area outside the vitelliform lesion, which agrees with our previous 3-D SD-OCT analyses of multiple subjects affected by Best disease 28 (Fig. 1).…”

Clinical and genetic heterogeneity in Slovenian patients with BEST disease

“…Vitelliform material in our two youngest patients with the vitelliform stage of the disease (Fig. 5) showed vitelliform material above the RPE and below the outer segment tips, which was in accordance with another study (Kay et al 2012). In the pseudohypopyon and vitelliruptive stages (Fig.…”

“…5) showed vitelliform material above the RPE and below the outer segment tips, which was in accordance with another study (Kay et al 2012). Vitelliform material in our two youngest patients with the vitelliform stage of the disease (Fig.…”

Outer Retinal Structure in Best Vitelliform Macular Dystrophy

“…This photoreceptor structure is capable of supporting rather good visual acuity, as visual acuity in the eyes imaged here ranged from 20/20 to 20/50. In addition, our SD-OCT and AOSLO data show normal photoreceptor structure in retinal areas outside the clinically visible lesion, in contrast to previous reports 21 , but consistent with previous findings with AOSLO. 48 This may represent a specific feature of the mutation studied here, or be due to different imaging and measurement procedures.…”

Human Photoreceptor Outer Segments Shorten During Light Adaptation

“…All normal subjects exhibited the same pattern of thickening in the dark and thinning in the light; and, in the one subject who was imaged repeatedly over the course of several months, these measurements were reproducible over time. Third, histological analysis of an eye from a Best disease donor revealed an increased OSEL in the macular area outside the vitelliform lesion, which agrees with our previous 3-D SD-OCT analyses of multiple subjects affected by Best disease 28 (Fig. 1).…”

Clinical and genetic heterogeneity in Slovenian patients with BEST disease

“…Vitelliform material in our two youngest patients with the vitelliform stage of the disease (Fig. 5) showed vitelliform material above the RPE and below the outer segment tips, which was in accordance with another study (Kay et al 2012). In the pseudohypopyon and vitelliruptive stages (Fig.…”

“…5) showed vitelliform material above the RPE and below the outer segment tips, which was in accordance with another study (Kay et al 2012). Vitelliform material in our two youngest patients with the vitelliform stage of the disease (Fig.…”

Outer Retinal Structure in Best Vitelliform Macular Dystrophy

“…This photoreceptor structure is capable of supporting rather good visual acuity, as visual acuity in the eyes imaged here ranged from 20/20 to 20/50. In addition, our SD-OCT and AOSLO data show normal photoreceptor structure in retinal areas outside the clinically visible lesion, in contrast to previous reports 21 , but consistent with previous findings with AOSLO. 48 This may represent a specific feature of the mutation studied here, or be due to different imaging and measurement procedures.…”

Human Photoreceptor Outer Segments Shorten During Light Adaptation

“…All normal subjects exhibited the same pattern of thickening in the dark and thinning in the light; and, in the one subject who was imaged repeatedly over the course of several months, these measurements were reproducible over time. Third, histological analysis of an eye from a Best disease donor revealed an increased OSEL in the macular area outside the vitelliform lesion, which agrees with our previous 3-D SD-OCT analyses of multiple subjects affected by Best disease 28 (Fig. 1).…”

Clinical and genetic heterogeneity in Slovenian patients with BEST disease

“…Vitelliform material in our two youngest patients with the vitelliform stage of the disease (Fig. 5) showed vitelliform material above the RPE and below the outer segment tips, which was in accordance with another study (Kay et al 2012). In the pseudohypopyon and vitelliruptive stages (Fig.…”

“…5) showed vitelliform material above the RPE and below the outer segment tips, which was in accordance with another study (Kay et al 2012). Vitelliform material in our two youngest patients with the vitelliform stage of the disease (Fig.…”

Outer Retinal Structure in Best Vitelliform Macular Dystrophy

“…This photoreceptor structure is capable of supporting rather good visual acuity, as visual acuity in the eyes imaged here ranged from 20/20 to 20/50. In addition, our SD-OCT and AOSLO data show normal photoreceptor structure in retinal areas outside the clinically visible lesion, in contrast to previous reports 21 , but consistent with previous findings with AOSLO. 48 This may represent a specific feature of the mutation studied here, or be due to different imaging and measurement procedures.…”