macular pigment optical density (MPOD) with age in the Korean population using the Macular Pigment Screener II (MPSII®).METHODS: One hundred and twenty-six eyes were retrospectively reviewed. MPOD was measured using MPSII®, which uses a heterochromatic flicker photometry method, and the estimated values were analyzed. Spearman's correlation test was used to evaluate correlations between MPOD and age. The association between MPOD and age was determined using a simple linear regression analysis. MPODs among the four groups were compared via the post hoc analysis with Bonferroni correction, MPODs between the age-related macular degeneration (AMD) group and aged-matched healthy subjects were compared via the Mann-Whitney U test. Other risk factors for AMD were identified via a logistic regression analysis.RESULTS: Estimated MPOD decreased significantly with increasing age in the general population. In the simple regression analysis, a statistically significant linear regression model was observed, and the estimated values of MPOD decreased by −0.005 as age increased by 1 year. Aged (> 50 years) showed lower MPOD than younger (30–49 years) subjects. But, in the healthy population, the estimated MPOD values exhibited a decreasing trend with age, but there were no significant differences according to age, after excluding patients with AMD. MPOD was significantly lower in patients with AMD than in aged healthy controls. Furthermore, hypertension, dyslipidemia, and smoking were identified as risk factors for AMD.CONCLUSION: MPOD measured with MPSII® reflects the MP density in healthy individuals and patients with dry AMD. Aging was not significantly associated with low MPOD in healthy population, but the presence of dry AMD was significantly associated with low MPOD. Then, low MPOD may be a risk factor for development of dry AMD. Furthermore, routine screening with MPS II® for ages 50 and older is thought to help detect early low MPOD and identify individuals who should take supplements.]]>
Aging ; Dyslipidemias ; Healthy Volunteers ; Humans ; Hypertension ; Linear Models ; Logistic Models ; Macular Degeneration ; Macular Pigment ; Mass Screening ; Methods ; Photometry ; Retrospective Studies ; Risk Factors ; Smoke ; Smoking

PURPOSE: To report a rare case of Sjögren's reticular retinal dystrophy. CASE SUMMARY: A 54-year-old male presented with blurred vision and metamorphopsia in both eyes since a few years prior to his initial visit. There was a bilateral reticular network of yellow deposits throughout the posterior pole on fundus examination, which was hyperautofluorescent in fundus autofluorescence photographs. The pigment alterations were more visible with fluorescein angiography, which showed hypofluorescent lesions with hyperfluorescent borders. Spectral-domain optical coherence tomography showed elevations of the outer retina associated with the presence of subretinal hyperreflective material. Based on the conclusive correlation with clinical features, we diagnosed Sjögren's reticular retinal dystrophy. CONCLUSIONS: Sjögren's reticular retinal dystrophy is characterized by its specific pigment changes at the level of clinical manifestations and the retinal pigment epithelium. In cases of Sjögren's reticular retinal dystrophy, close monitoring is required because it has a lifetime risk of choroidal neovascularization.
Choroidal Neovascularization ; Fluorescein Angiography ; Humans ; Macular Degeneration ; Male ; Middle Aged ; Retina ; Retinal Dystrophies ; Retinal Pigment Epithelium ; Retinaldehyde ; Tomography, Optical Coherence ; Vision Disorders

PURPOSE: To report the first case of cystoid macular edema in a retinitis pigmentosa patient with pars plana vitrectomy. CASE SUMMARY: A 43-year-old female visited our hospital with visual disturbances of both eyes. Corrected visual acuity was 20/22 in the right eye and 20/25 in the left eye. Peripheral depigmentation and atrophy of the retinal pigment epithelium, pigmentary retinal degeneration, and attenuated arterioles were observed in both eyes. Cystoid macular edema was observed on optical coherence tomography which showed that the central macular thickness was 308 µm in the right eye and 422 µm in left eye. Intravitreal aflibercept was injected into the left eye. One month after injection, the central macular thickness showed no response with a thickness of 449 µm. An intravitreal dexamethasone implant was then injected, 1 month after injection, the central macular thickness was 367 µm. Six months after injection, the patient again complained of visual disturbance of the left eye with a corrected visual acuity of 20/70. Vitreous opacity was observed and the central macular thickness was 501 µm. The patient underwent pars plana vitrectomy. Three days after surgery, the central macular thickness was 320 µm. One year after surgery, the corrected visual acuity was 20/33 and the central macular thickness was 311 µm. CONCLUSIONS: Pars plana vitrectomy due to cystoid macular edema in a retinitis pigmentosa patient has not been previously reported in the Republic of Korea. Pars plana vitrectomy can therefore be an effective treatment for cystoid macular edema in retinitis pigmentosa patients.
Adult ; Arterioles ; Atrophy ; Dexamethasone ; Female ; Humans ; Macular Edema ; Republic of Korea ; Retinal Degeneration ; Retinal Pigment Epithelium ; Retinitis Pigmentosa ; Retinitis ; Tomography, Optical Coherence ; Visual Acuity ; Vitrectomy

Stargardt-like macular dystrophy 4 (STGD4) is a rare macular dystrophy characterized by bull's eye atrophy of the macula and the underlying retinal pigment epithelium. Patients with STGD4 show decreased central vision, which often progresses to severe vision loss. The PROM1 gene encodes prominin-1, which is a 5-transmembrane glycoprotein also known as CD133 and is involved in photoreceptor disk morphogenesis. PROM1 mutations have been identified as genetic causes for STGD4 and other retinal degenerations such as retinitis pigmentosa. We report a case of STGD4 with a PROM1 p.R373C mutation in a Korean patient. Ophthalmic examinations of a 38-yr old man complaining of decreased visual acuity revealed bilateral atrophic macular lesions consistent with STGD4. Targeted exome sequencing of known inherited retinal degeneration genes revealed a heterozygous missense mutation c.1117C>T (p.R373C) of PROM1, which was confirmed by Sanger sequencing. To the best of our knowledge, this is the first case of a PROM1 mutation causing STGD4 in Koreans.
Atrophy ; Exome ; Glycoproteins ; Humans ; Macular Degeneration* ; Morphogenesis ; Mutation, Missense ; Retinal Degeneration ; Retinal Pigment Epithelium ; Retinitis Pigmentosa ; Visual Acuity

PURPOSE: To compare the outer retinal thickness in normal fellow eyes of patients with unilateral age-related macular degeneration (AMD) and normal control eyes. METHODS: We retrospectively reviewed the medical records of 60 patients with unilateral exudative AMD including polypoidal choroidal vasculopathy and 60 normal controls. Spectralis optical coherence tomography was performed in the normal fellow eyes of patients with unilateral AMD and in the normal group. The thicknesses between the retinal pigment epithelium (RPE) line and the cone outer segment tips (COST) line, between the COST line and the photoreceptor inner segment/outer segment (IS/OS) line, and between the IS/OS line and the external limiting membrane (ELM) line were measured at the fovea in both groups. RESULTS: The thickness between the RPE and COST lines was 32.4 ± 3.0 µm in normal fellow eyes of patients with unilateral AMD and 35.3 ± 3.5 µm in the normal group (p < 0.001). Total retinal thickness, thicknesses between the COST and the IS/OS lines and the IS/OS and the ELM lines in fellow eyes were not significantly different from those of normal eyes (p = 0.126, 0.615, 0.874). There was no significant difference in total retinal thickness or each outer retinal thickness measured in normal fellow eyes between patients with neovascular AMD and polypoidal choroidal vasculopathy. CONCLUSIONS: The thickness between the RPE and the COST lines was thinner in the fellow eyes of patients with unilateral AMD than in the normal eyes. We suggest that less thickness between the RPE and COST lines might indicate a greater risk of AMD.
Choroid ; Humans ; Macular Degeneration* ; Medical Records ; Membranes ; Retinal Pigment Epithelium ; Retinaldehyde* ; Retrospective Studies ; Tomography, Optical Coherence

Bestrophin-1 (Best1) is a calcium-activated anion channel identified from retinal pigment epithelium where human mutations are associated with Best's macular degeneration. Best1 is known to be expressed in a variety of tissues including the brain, and is thought to be involved in many physiological processes. This review focuses on the current state of knowledge on aspects of expression and function of Best1 in the brain. Best1 protein is observed in cortical and hippocampal astrocytes, in cerebellar Bergmann glia and lamellar astrocytes, in thalamic reticular neurons, in meninges and in the epithelial cells of the choroid plexus. The most prominent feature of Best1 is its significant permeability to glutamate and GABA in addition to chloride ions because glutamate and GABA are important transmitters in the brain. Under physiological conditions, both Best1-mediated glutamate release and tonic GABA release from astrocytes modulate neuronal excitability, synaptic transmission and synaptic plasticity. Under pathological conditions such as neuroinflammation and neurodegeneration, reactive astrocytes phenotypically switch from GABA-negative to GABA-producing and redistribute Best1 from the perisynaptic microdomains to the soma and processes to tonically release GABA via Best1. This implicates that tonic GABA release from reactive astrocyte via redistributed Best1 is a common phenomenon that occur in various pathological conditions with astrogliosis such as traumatic brain injury, neuroinflammation, neurodegeneration, and hypoxic and ischemic insults. These properties of Best1, including the permeation and release of glutamate and GABA and its redistribution in reactive astrocytes, promise us exciting discoveries of novel brain functions to be uncovered in the future.
Astrocytes ; Brain Injuries ; Brain* ; Carisoprodol ; Choroid Plexus ; Epithelial Cells ; gamma-Aminobutyric Acid ; Glutamic Acid ; Humans ; Ions ; Macular Degeneration ; Meninges ; Neuroglia ; Neuronal Plasticity ; Neurons ; Permeability ; Physiological Processes ; Retinal Pigment Epithelium ; Synaptic Transmission

PURPOSE: To analyze the anatomical characteristics on spectral-domain optical coherence tomography (SD-OCT) of patients who are legally blind (less than 20/1,000) due to end-stage exudative age-related macular degeneration (AMD) that does not require intravitreal anti-vascular endothelial growth factor (anti-VEGF) injection. METHODS: After anti-VEGF injections (active group), 120 eyes of 103 exudative AMD patients experienced visual acuity improved by at least 2 lines or improvement on SD-OCT. In addition, 55 eyes of 54 end-stage exudative AMD patients who did not respond to treatment or who were legally blind due to foveal scar at the first visit (end-stage group) were evaluated retrospectively. Changes in retinal structures of the 2 groups were analyzed by SD-OCT at the last visit. RESULTS: The mean age of the end-stage group was about 5 years older than the active group. During the follow-up period, subretinal hemorrhage, intraretinal hemorrhage and retinal pigment epithelium tear occurred more frequently in the end-stage group than in the active group (p < 0.05). Intra-retinal fluids and subretinal fluids were more frequently administered in the active group than in the end-stage group, and thick subretinal hyper-reflective materials (SRHRM), fibrovascular pigment epithelial detachment (PED) and extensive inner segment/outer segment (IS/OS) line disruption were observed in all eyes of the end-stage group. The size and thickness of PED, foveal thickness and SRHRM thickness were significantly larger in the end-stage group than in the active group (p < 0.05). Disciform retinal scars were eventually formed in most of the end-stage group. CONCLUSIONS: In end-stage exudative AMD, the presence of retinal hemorrhage and retinal pigment epithelium tear during follow-up, or the findings of thick SRHRM, fibrovascular PED, and extensive IS/OS line disruption on SD-OCT suggest weak expected effect of intravitreal anti-VEGF injection, which can act as a reference for determining the timing of treatment termination.
Cicatrix ; Endothelial Growth Factors* ; Follow-Up Studies ; Hemorrhage ; Humans ; Macular Degeneration* ; Retinal Hemorrhage ; Retinal Pigment Epithelium ; Retinaldehyde ; Retrospective Studies ; Subretinal Fluid ; Tears ; Tomography, Optical Coherence ; Visual Acuity

PURPOSE: To analyze the anatomical characteristics on spectral-domain optical coherence tomography (SD-OCT) of patients who are legally blind (less than 20/1,000) due to end-stage exudative age-related macular degeneration (AMD) that does not require intravitreal anti-vascular endothelial growth factor (anti-VEGF) injection. METHODS: After anti-VEGF injections (active group), 120 eyes of 103 exudative AMD patients experienced visual acuity improved by at least 2 lines or improvement on SD-OCT. In addition, 55 eyes of 54 end-stage exudative AMD patients who did not respond to treatment or who were legally blind due to foveal scar at the first visit (end-stage group) were evaluated retrospectively. Changes in retinal structures of the 2 groups were analyzed by SD-OCT at the last visit. RESULTS: The mean age of the end-stage group was about 5 years older than the active group. During the follow-up period, subretinal hemorrhage, intraretinal hemorrhage and retinal pigment epithelium tear occurred more frequently in the end-stage group than in the active group (p < 0.05). Intra-retinal fluids and subretinal fluids were more frequently administered in the active group than in the end-stage group, and thick subretinal hyper-reflective materials (SRHRM), fibrovascular pigment epithelial detachment (PED) and extensive inner segment/outer segment (IS/OS) line disruption were observed in all eyes of the end-stage group. The size and thickness of PED, foveal thickness and SRHRM thickness were significantly larger in the end-stage group than in the active group (p < 0.05). Disciform retinal scars were eventually formed in most of the end-stage group. CONCLUSIONS: In end-stage exudative AMD, the presence of retinal hemorrhage and retinal pigment epithelium tear during follow-up, or the findings of thick SRHRM, fibrovascular PED, and extensive IS/OS line disruption on SD-OCT suggest weak expected effect of intravitreal anti-VEGF injection, which can act as a reference for determining the timing of treatment termination.
Cicatrix ; Endothelial Growth Factors* ; Follow-Up Studies ; Hemorrhage ; Humans ; Macular Degeneration* ; Retinal Hemorrhage ; Retinal Pigment Epithelium ; Retinaldehyde ; Retrospective Studies ; Subretinal Fluid ; Tears ; Tomography, Optical Coherence ; Visual Acuity

PURPOSE: Intravitreal anti-vascular endothelial growth factor (anti-VEGF) is the first choice of treatment for age-related macular degeneration. However, quite a few eyes treated using conventional dose anti-VEGF (CDAV) have persistent pigment epithelial detachment (PED) on optical coherence tomography. This study investigated the efficacy and safety of high dose anti-VEGF (HDAV) for refractory PED. METHODS: In this retrospective study, 31 eyes of neovascular age-related macular degeneration patients with persistent PED findings despite six or more intravitreal injections of CDAV (bevacizumab 1.25 mg or ranibizumab 2.5 mg) were analyzed. Changes in visual outcome, central foveal thickness, and PED height were compared before and after HDAV (bevacizumab 5.0 mg) for these refractory PED cases. RESULTS: The mean age of patients was 67.7 years. The number of CDAV injections was 12.1. The number of HDAV injections was 3.39. Best-corrected visual acuity in logarithm of the minimum angle of resolution before and after HDAV was 0.49 and 0.41 (p < 0.001), respectively. Central foveal thickness before and after HDAV was 330.06 and 311.10 µm (p = 0.125), respectively. PED height before and after HDAV was 230.28 and 204.07 µm (p = 0.014), respectively. There were no serious adverse reactions in all the eyes. CONCLUSIONS: Increasing the dose of bevacizumab in refractory PED may be a possible treatment option.
Aged ; Angiogenesis Inhibitors/administration & dosage ; Bevacizumab/*administration & dosage ; Dose-Response Relationship, Drug ; Female ; Fluorescein Angiography ; Fundus Oculi ; Humans ; Intravitreal Injections ; Macular Degeneration/*complications/diagnosis/drug therapy ; Male ; Middle Aged ; Retinal Detachment/diagnosis/*drug therapy/etiology ; Retinal Pigment Epithelium/*diagnostic imaging/drug effects ; Retrospective Studies ; Tomography, Optical Coherence ; Vascular Endothelial Growth Factor A/antagonists & inhibitors

PURPOSE: To evaluate choroidal thickness at the outside of the fovea in patients with diabetic retinopathy using spectral-domain optical coherence tomography. METHODS: We examined 87 eyes of 87 patients with diabetic retinopathy and 40 eyes of 40 normal patients. Patients with diabetic retinopathy were divided into 3 groups according to the grade of diabetic retinopathy and macular edema. The choroidal thickness was obtained at the fovea and outside of the fovea using enhanced depth imaging of Spectralis optical coherence tomography. One foveal and 8 peripheral images were selected and choroidal thickness was measured from the outer border of the retinal pigment epithelium to the inner scleral border. RESULTS: Subfoveal choroidal thickness was thinner with increasing severity of diabetic retinopathy. However, there was no significant difference between groups without the nasal side of the fovea. A statistically significant difference was observed over the fovea at the superotemporal area. CONCLUSIONS: The choroidal thickness outside of the fovea was thinner with the severity of diabetic retinopathy and was more pronounced in the superotemporal area.
Choroid* ; Diabetic Retinopathy* ; Humans ; Macular Edema ; Retinal Pigment Epithelium ; Tomography, Optical Coherence*