Case Report: Self-Resolved Macular Edema Secondary to Congenital Retinal Macrovessels

PurposeTo report a case of macular edema secondary to congenital retinal macrovessels (CRMs), which resolved spontaneously without any treatment.ObservationsA 39-year-old female presented with blurry vision of the right eye for one day. Fundus examination revealed a branch of artery and vein of the inferior retinal arcade crossing the horizontal raphe. Optical coherence tomography (OCT) through the fovea showed cystoid macular edema in the outer plexiform layer. However, no leakage of the vessels was noticed by fundus fluorescein angiography (FFA). Observation was recommended with close follow-up. Two weeks later, the patient returned with good visual acuity, and the macular edema was resolved spontaneously.ConclusionsMacular edema is a possible complication of CRM by increasing retinal capillary hydrostatic pressure. Treatment is not necessary for this kind of macular edema if no leakage of the vessels is noticed on FFA.

Interocular symmetry of optical coherence tomography parameters in healthy children and adolescents

Evaluation of interocular asymmetry of optical coherence tomography (OCT) parameters is important for the glaucoma and optic neuropathies. This study was performed to evaluate the interocular asymmetry of OCT parameters in healthy children and adolescents. The circumpapillary retinal nerve fiber layer (RNFL) thickness, optic nerve head (ONH) parameters, and macular ganglion cell-inner plexiform layer (GCIPL) thickness were measured in 620 eyes of 310 healthy children and adolescents using Cirrus HD-OCT. The interocular asymmetry (right eye–left eye) in the OCT parameters was analyzed. The mean ± standard deviation age was 10.3 ± 3.7 years (range 5–17). The right eyes showed thinner superior quadrant RNFL, thicker nasal and temporal quadrant RNFL, lesser rim and disc areas, and thinner average, superior, and superonasal GCIPL than the left eyes (P < 0.05). The 2.5th and 97.5th percentile interocular difference tolerance limits were − 9.0 μm and 11.0 μm for average RNFL thickness, − 0.21 and 0.18 for average cup-to-disc ratio, and − 4.0 μm and 4.0 μm for average GCIPL thickness, respectively. Interocular differences were found in RNFL thickness, ONH parameters, and GCIPL thickness in healthy children and adolescents. These findings should be considered when comparing OCT parameters between the right and left eyes.

Preservation of Retinal Function Through Synaptic Stabilization in Alzheimer's Disease Model Mouse Retina by Lycium Barbarum Extracts

In Alzheimer's disease (AD), amyloid β deposition-induced hippocampal synaptic dysfunction generally begins prior to neuronal degeneration and memory impairment. Lycium barbarum extracts (LBE) have been demonstrated to be neuroprotective in various animal models of neurodegeneration. In this study, we aimed to investigate the effects of LBE on the synapse loss in AD through the avenue of the retina in a triple transgenic mouse model of AD (3xTg-AD). We fed 3xTg-AD mice with low (200 mg/kg) or high (2 g/kg) dose hydrophilic LBE daily for 2 months from the starting age of 4- or 6-month-old. For those started at 6 month age, at 1 month (though not 2 months) after starting treatment, mice given high dose LBE showed a significant increase of a wave and b wave in scotopic ERG. After 2 months of treatment with high dose LBE, calpain-2, calpain-5, and the oxidative RNA marker 8-OHG were downregulated, and presynaptic densities in the inner plexiform layer but not the outer plexiform layer of the retina were significantly increased, suggesting the presynaptic structure of retina was preserved. Our results indicate that LBE feeding may preserve synapse stability in the retina of 3xTg-AD mice, probably by decreasing both oxidative stress and intracellular calcium influx. Thus, LBE might have potential as a neuroprotectant for AD through synapse preservation.

Retinal Development and Pathophysiology in Kcnj13 Knockout Mice

Purpose: We constructed and characterized knockout and conditional knockout mice for KCNJ13, encoding the inwardly rectifying K+ channel of the Kir superfamily Kir7.1, mutations in which cause both Snowflake Vitreoretinal Degeneration (SVD) and Retinitis pigmentosa (RP) to further elucidate the pathology of this disease and to develop a potential model system for gene therapy trials.Methods: A Kcnj13 knockout mouse line was constructed by inserting a gene trap cassette expressing beta-galactosidase flanked by FRT sites in intron 1 with LoxP sites flanking exon two and converted to a conditional knockout by FLP recombination followed by crossing with C57BL/6J mice having Cre driven by the VMD2 promoter. Lentiviral replacement of Kcnj13 was driven by the EF1a or VMD2 promoters.Results: Blue-Gal expression is evident in E12.5 brain ventricular choroid plexus, lens, neural retina layer, and anterior RPE. In the adult eye expression is seen in the ciliary body, RPE and choroid. Adult conditional Kcnj13 ko mice show loss of photoreceptors in the outer nuclear layer, inner nuclear layer thinning with loss of bipolar cells, and thinning and disruption of the outer plexiform layer, correlating with Cre expression in the overlying RPE which, although preserved, shows morphological disruption. Fundoscopy and OCT show signs of retinal degeneration consistent with the histology, and photopic and scotopic ERGs are decreased in amplitude or extinguished. Lentiviral based replacement of Kcnj13 resulted in increased ERG c- but not a- or b- wave amplitudes.Conclusion: Ocular KCNJ13 expression starts in the choroid, lens, ciliary body, and anterior retina, while later expression centers on the RPE with no/lower expression in the neuroretina. Although KCNJ13 expression is not required for survival of the RPE, it is necessary for RPE maintenance of the photoreceptors, and loss of the photoreceptor, outer plexiform, and outer nuclear layers occur in adult KCNJ13 cKO mice, concomitant with decreased amplitude and eventual extinguishing of the ERG and signs of retinitis pigmentosa on fundoscopy and OCT. Kcnj13 replacement resulting in recovery of the ERG c- but not a- and b-waves is consistent with the degree of photoreceptor degeneration seen on histology.

Optical Coherence Tomography Angiography Compared With Optical Coherence Tomography for Detection of Early Glaucoma With High Myopia

Purpose: To investigate the diagnostic abilities of the perfusion density (PD) and structural thickness parameters in the peripapillary and macular regions measured by optical coherence tomography angiography (OCTA) and optical coherence tomography (OCT) and to test if their diagnostic abilities of early glaucoma are different between highly myopic (HM) and non-highly myopic (NHM) patients.Methods: A total of 75 glaucoma patients and 65 controls were included in the analyses. The glaucoma detection abilities of macular PD and peripapillary PD, along with macular ganglion cell-inner plexiform layer (mGCIPL) thickness and peripapillary retinal nerve fiber layer (pRNFL) thicknesses were compared between the HM and NHM group. Diagnostic ability was assessed by area under the receiver operating characteristics (AUC) curves, adjusted by age, axial length, and signal strength.Results: The diagnostic ability of macular PD and mGCIPL thickness had no significant difference in both HM and NHM groups. However, the diagnostic ability of peripapillary PD except in the temporal section was significantly lower in the HM group than in the NHM group (all p &lt; 0.05). The diagnostic ability of the superior, nasal, and average pRNFL thickness was also significantly lower in the HM group than in the NHM group (all p &lt; 0.05).Conclusion: This study demonstrated that although peripapillary PD and macular PD were both significantly reduced in patients with highly myopia, the diagnostic ability of peripapillary PD in HM patients was significantly lower than that in NHM patients, while macular PD was not. Macular OCTA along with OCT imaging should be included in the imaging algorithm in early glaucoma diagnosis in highly myopic patients.

Quantitative Optical Coherence Tomography for Longitudinal Monitoring of Postnatal Retinal Development

A better study of the postnatal retinal development is not only essential for the in-depth understanding of the nature of the vision system but also may provide insights for treatment developments of eye conditions, such as retinopathy of premature (ROP). To date, quantitative analysis of postnatal retinal development is primarily limited to endpoint histological examination. This study is to validate in vivo optical coherence tomography (OCT) for longitudinal monitoring of postnatal retinal development in developing mouse eyes. Three-dimensional (3D) frame registration and super averaging were adopted to investigate the fine structure of the retina. Interestingly, a hyporeflective layer (HRL) between the nerve fiber layer (NFL) and inner plexiform layer (IPL) was observed in developing eyes and gradually disappeared with aging. To interpret the observed retinal layer kinetics, a model based on eyeball expansion, cell apoptosis, and retinal structural modification was proposed.

Fingerprint sign in Vogt-Koyanagi-Harada disease: a case series

Background The tomographic finding, which has been called the "fingerprint sign" in en face reconstructions, seems to be the result of a variety of processes that cause distension of the outer plexiform layer (OPL) and the Henle fiber layer (HFL). The aim of this paper is to describe the appearance of concentric rings at the OPL/HFL interface visualized using en face reconstructions of cross-sectional optical coherence tomography images of patients with Vogt-Koyanagi-Harada disease. Methods Retrospective analysis of images of six eyes of three patients obtained by cross-sectional OCT imaging and en face reconstruction at the level of the OPL/HFL interface. Results All eyes presented with a dentate or saw-tooth pattern of the OPL/HFL interface on cross-sectional OCT with corresponding concentric rings on en face OCT reconstruction, consistent with the recently published “fingerprint sign”. Initial OPL/HFL interface changes were observed between the first and fourth months after treatment and resolution of VKHD associated serous retinal detachments. These OPL/HFL interface changes have persisted for many years following the resolution of the active inflammation. Conclusions Changes in the OPL/HFL interface can be identified following successful treatment of VKHD. These included both a dentate or saw-tooth pattern on cross-sectional imaging and concentric rings or the “fingerprint sign” on en face reconstructions. These changes persisted for many years despite disease quiescence.

Optical Coherence Tomography Identifies Visual Pathway Involvement Earlier than Visual Function Tests in Children with MRI-Verified Optic Pathway Gliomas

This study investigates whether optical coherence tomography (OCT) could add useful information in the examination of children with optic pathway glioma (OPG) at high risk of developing vision loss. For this purpose, the relationship between ganglion cell-inner plexiform layer (GC-IPL) thickness and visual function, evaluated with tests of visual acuity (VA) and visual field (VF), as well as tumor site according to magnetic resonance imaging (MRI), were examined in a geographically defined group of children with OPG. Methods: Children aged <18 years with OPG underwent ophthalmic examination including VA, VF (Zeiss HFA perimetry) and OCT imaging (Zeiss Cirrus HD-OCT). Results: Out of 51 patients included, 45 provided 77 eyes with MRI-verified OPG, and 19 patients provided 25 eyes without OPG. Significant correlations were found between GC-IPL, VF and VA (p < 0.001). The GC-IPL pattern loss corresponded in 95% to VF defects and in 92% to MRI findings. Conclusions: Our study indicates that GC-IPL measures could serve as an early marker of vision-threatening changes related to OPG and as a valuable link between MRI and visual function tests. Thinning of GC-IPL and differences in topography between eyes are strong indicators of and predictive of vision loss related to OPG.

Retinal Development and The Expression Profiles of Opsins Genes During Larvae Development in Takifugu Rubripes

Vision is the dominant sensory modality in fish and critical for the survival of fish larvae to detect predators or capture prey. The visual capacity of fish larvae is determined by the structure of the retina and the opsins expressed in the retinal and non-retinal photoreceptors. In this study, the retinal structure and opsin expression patterns during the early development stage of Takifugu rubripes larvae were investigated. At around two days after hatching days (dah), the yolk sac of T. rubripes disappeared, the mouth was clearly visible and the larvae started swimming and feeding on rotifers. Histological examination showed that at 1 dah, six layers were observed in the retina of T. rubripes larva, including the pigment epithelial layer (RPE), photoreceptor layer (PRos/is), outer nuclear layer (ONL), inner nuclear layer (INL), inner plexiform layer (IPL) and ganglion cell layer (GCL). At 2 dah, all eight layers were visible in the retina in T. rubripes larva, including RPE, PRos/is, ONL, outer plexiform layer (OPL), INL, IPL, GCL and optic fiber layer (OFL). By measuring the thickness of each layer, opposing developmental trends were found in the thickness of ONL, OPL, INL and IPL, GCL and OFL. The nuclear density of ONL, INL and GCL and ratio of ONL/INL, ONL/GCL and INL/GCL were also measured and the ratio of ONL/GCL ranged from 1.9 at 2 dah to 3.4 at 8 dah and no significant difference was observed between the different developmental stages (p > 0.05). No significant difference was observed for the INL/GCL ratio between the different developmental stages, which ranged from 1.2 at 2 dah to 2.0 at 18 dah (p > 0.05). The results of quantitative real-time PCR showed that the expression of rhodopsin, LWS, SWS2, green opsin, rod opsin, opsin3 and opsin5 could be detected from 1 dah. These results suggested that the maturation of eye of T. rubripes occurred during the period of transition from endogenous to mixed feeding, explaining the need for vision-based survival skills during the early life stages after hatching and for the overall ecology and fitness of T. rubripes.