Optical coherence tomography and optical coherence tomography angiography findings in optic nerve hypoplasia and their relationships with visual acuity.

This study aimed to quantitatively assess the thickness of the peripapillary retinal nerve fiber layer (pRNFL) thickness, as well as the microvascular alterations in the macula and peripapillary regions, in optic nerve hypoplasia (ONH) patients compared to normal controls. This was achieved through the utilization of spectral-domain optical coherence tomography (SD-OCT) and optical coherence tomography angiography (OCTA), with a specific focus on elucidating the association between these structural alterations and visual acuity. We included a total of 17 eyes of 12 ONH patients, and 34 eyes of age-matched 34 healthy controls. The pRNFL thickness was quantified using SD-OCT, while OCTA facilitated the visualization and measurement of the microvascular structure images of the superficial retinal capillary plexus (SRCP), deep retinal capillary plexus (DRCP), and radial peripapillary capillary (RPC) segment in the macula and peripapillary area. pRNFL thickness was measured for eight sectors (superior, temporal, inferior, nasal, superotemporal, superonasal, inferotemporal, and inferonasal). SRCP, DRCP, and RPC were measured for four sectors (superior, temporal, inferior, and nasal). Age, gender, and spherical equivalent refractive errors were statistically adjusted for the analysis. Associations of structural parameters with visual acuity in ONH patients were analyzed using Spearman correlation analysis. pRNFL thickness was significantly thinner in ONH patients than in controls for all sectors. Vessel densities of temporal and nasal sectors in DRCP were significantly higher in ONH patients, but vessel densities of the inferior sector in RPC were significantly lower than those in controls. For all sectors, pRNFL thickness was strongly associated with visual acuity in ONH patients. ONH patients showed significant pRNFL thinning and microvascular alterations compared to controls, and pRNFL thickness was strongly associated with visual function. OCT and OCTA are useful tools for evaluating optic disc hypoplasia and its functional status.

Nonarteritic anterior ischemic optic neuropathy and incidence of Parkinson's disease based on a nationwide population based study.

This study aimed to investigate the association between nonarteritic anterior ischemic optic neuropathy (NAION) and Parkinson's disease (PD) using a retrospective, nationwide, population-based cohort in South Korea. This study utilized data from the Korean National Health Insurance database, including 43,960 NAION patients and 219,800 age- and sex-matched controls. Cox proportional hazards regression models were used to assess the risk of developing PD in the NAION group compared to the control group after adjusting for various confounding factors. Subgroup analyses were conducted based on sex, age, and comorbidities. The incidence rate of PD was higher in the NAION group (1.326 per 1000 person-years) than in the control group (0.859 per 1000 person-years). After adjusting for confounding factors, the risk of developing PD was significantly higher in the NAION group (adjusted hazard ratio [aHR] 1.516, 95% confidence interval [CI] 1.300-1.769). Subgroup analyses did not reveal a significant difference in the risk of PD development based on sex, age, or comorbidities. This retrospective, nationwide, population-based cohort study revealed a significant association between NAION and an increased risk of developing PD in a South Korean population. The incidence rate of PD was observed to be higher in individuals diagnosed with NAION than in age- and sex-matched controls even after adjusting for potential confounding variables, with the risk being approximately 51.6% higher in the NAION group. Further research is necessary to elucidate the underlying pathophysiological mechanisms linking NAION to PD and to determine whether similar associations exist in other ethnic and geographical populations.

Deep learning-based optic disc classification is affected by optic-disc tilt.

We aimed to determine the effect of optic disc tilt on deep learning-based optic disc classification. A total of 2507 fundus photographs were acquired from 2236 eyes of 1809 subjects (mean age of 46 years; 53% men). Among all photographs, 1010 (40.3%) had tilted optic discs. Image annotation was performed to label pathologic changes of the optic disc (normal, glaucomatous optic disc changes, disc swelling, and disc pallor). Deep learning-based classification modeling was implemented to develop optic-disc appearance classification models with the photographs of all subjects and those with and without tilted optic discs. Regardless of deep learning algorithms, the classification models showed better overall performance when developed based on data from subjects with non-tilted discs (AUC, 0.988 ± 0.002, 0.991 ± 0.003, and 0.986 ± 0.003 for VGG16, VGG19, and DenseNet121, respectively) than when developed based on data with tilted discs (AUC, 0.924 ± 0.046, 0.928 ± 0.017, and 0.935 ± 0.008). In classification of each pathologic change, non-tilted disc models had better sensitivity and specificity than the tilted disc models. The optic disc appearance classification models developed based all-subject data demonstrated lower accuracy in patients with the appearance of tilted discs than in those with non-tilted discs. Our findings suggested the need to identify and adjust for the effect of optic disc tilt on the optic disc classification algorithm in future development.

Optical Coherence Tomography Parameters and Optimal Cut-off Values for Predicting Visual Prognosis in Ethambutol-Induced Optic Neuropathy.

BACKGROUND: The objective of this study was to evaluate the prognostic value of optical coherence tomography (OCT) parameters in patients with ethambutol-induced optic neuropathy (EON) and establish their optimal cut-off values for predicting visual acuity outcomes. METHODS: A retrospective cross-sectional study was conducted on 64 eyes of 32 patients with EON who underwent OCT. Peripapillary retinal nerve fiber layer (pRNFL) and macular ganglion cell-inner plexiform layer (mGCIPL) thickness were measured using Cirrus high-definition OCT (HD-OCT) within 3 months after EON diagnosis. Visual acuity of patients was recorded and analyzed at the first visit, the 1-year visit, and the latest visit. Prognostic capacities of OCT parameters for visual prognosis were evaluated and their optimal cut-off values for predicting final visual acuity were established. RESULTS: Increased pRNFL thickness was significantly associated with better visual acuity at 1 year postdiagnosis and the latest visit. A significant association was established between increased pRNFL thickness and a higher rate of recovery to visual acuity >20/25 at 1 year postdiagnosis. Receiver-operating characteristic curves identified ideal cut-off values for OCT parameters as follows: pRNFL thickness of 83 µm (sensitivity 100%, specificity 48.3%) and mGCIPL thickness of 74 µm (sensitivity 100%, specificity 83.3%) for visual acuity >20/25 at 1 year, mGCIPL thickness of 61 µm (sensitivity 85.7%, specificity 71.4%) for visual acuity >20/40 at 1 year, with corresponding AUCs exceeding 0.7. CONCLUSIONS: Both pRNFL and mGCIPL thickness possess potential values for predicting visual outcomes in patients with EON. Future research should continue to explore the utility of OCT parameters in EON prognosis.

Risk of central nervous system demyelinating attack or optic neuritis recurrence after pediatric optic neuritis in Korea.

PURPOSE: To investigate the rate of development of symptomatic central nervous system (CNS) demyelinating attacks or recurrent optic neuritis (ON) after the first episode of ON and its risk factors for Korean pediatric patients. METHODS: This multicenter retrospective cohort study included the patients under 18 years of age (n=132) diagnosed with ON without previous or simultaneous CNS demyelinating diseases. We obtained the clinical data including the results of neuro-ophthalmological examinations, magnetic resonance images (MRIs), antibody assays, and laboratory tests. We investigated the chronological course of demyelinating disease with respect to the occurrence of neurological symptoms and/or signs, and calculated the 5-year cumulative probability of CNS demyelinating disease or ON recurrence.  RESULTS: During the follow-up period (63.1±46.7 months), 18 patients had experienced other CNS demyelinating attacks, and the 5-year cumulative probability was 14.0±3.6%. Involvement of the extraorbital optic nerve or optic chiasm and asymptomatic lesions on the brain or spinal MRI at initial presentation were significant predictors for CNS demyelinating attack after the first ON. The 5-year cumulative probability of CNS demyelinating attack was 44.4 ± 24.8% in the AQP4-IgG group, 26.2±11.4% in the MOG-IgG group, and 8.7±5.9% in the double-negative group (P=0.416). Thirty-two patients had experienced a recurrence of ON, and the 5-year cumulative probability was 24.6±4.0%. In the AQP4-IgG group, the 5-year cumulative probability was 83.3±15.2%, which was significantly higher than in the other groups (P<0.001). CONCLUSIONS: A careful and multidisciplinary approach including brain/spinal imaging and antibody assay can help predict further demyelinating attacks in pediatric ON patients.

Intraretinal microvascular alterations in indirect traumatic optic neuropathy using optical coherence tomography angiography.

PURPOSE: To quantitatively evaluate macular and peripapillary microvascular alterations in patients with indirect traumatic optic neuropathy (TON) compared to normal controls using optical coherence tomography angiography (OCT-A) and determine their associations with other ocular parameters. METHODS: We enrolled 33 eyes of 33 patients with TON and 34 eyes of 34 healthy controls. OCT-A was used to generate microvascular structure images of the superficial retinal capillary plexus (SRCP), deep retinal capillary plexus (DRCP), and radial peripapillary capillary (RPC) segment in the macula and peripapillary area. Functional and structural parameters such as best-corrected visual acuity, visual field, peripapillary retinal nerve fibre layer (pRNFL) thickness, macular ganglion cell-inner plexiform layer (mGCIPL) thickness, OCT-A variables were compared between TON patients and controls. Age, gender, and spherical equivalent refractive errors were statistically adjusted for the analysis. RESULTS: OCT-A revealed a significant reduction of the average vessel density in the RPC segment in TON patients compared to controls (48.5% ± 6.28 vs. 57.88% ± 3.06%, P < 0.0001, corrected P < 0.0001). When comparing sectors, the vessel density of the RPC segment in TON patients was also significantly lower in all four quadrants compared to healthy controls. The inferior sector vessel density of the RPC segment was significantly associated with visual field defects (P = 0.0253) and visual acuity (P = 0.0369). The temporal sector vessel density of DRCP was also associated with visual field defects (P = 0.0377). The RPC segment in the superior and inferior sector vessel density displayed a significant association with the corresponding regional pRNFL thickness (P = 0.0248 and <0.0001, respectively). CONCLUSIONS: Patients with indirect TON exhibit significant microvascular alterations compared to controls. This study confirms that TON can induce intraretinal microvascular changes and suggests that OCT-A may serve as a useful biomarker for assessing visual functional and structural changes.

Central retinal microvasculature damage is associated with orthostatic hypotension in Parkinson's disease.

Orthostatic hypotension (OH) is a common non-motor symptom in Parkinson's disease (PD). OH can cause cerebral and retinal hypoperfusion and is associated with microvascular damage in PD. Optical coherence tomography angiography (OCTA) is a non-invasive technology that can be used to visualize the retinal microvasculature and detect microvascular damage in PD. In the present study, 51 PD patients (OH+, n = 20, 37 eyes; OH-, n = 32, 61 eyes) and 51 healthy controls (100 eyes) were evaluated. The Unified Parkinson's Disease Rating Scale III, Hoehn and Yahr scale, Montreal Cognitive Assessment, levodopa equivalent daily dose, and vascular risk factors, including hypertension, diabetes, and dyslipidemia, were investigated. PD patients underwent a head-up tilt (HUT) test. The PD patients had a lower superficial retinal capillary plexus (SRCP) density in the central region than control patients. The PDOH+ group had lower vessel density in the SRCP of the central region compared with the control group and lower vessel density in the DRCP of the central region than the PDOH- and control groups. The changes in systolic and diastolic blood pressure during the HUT test in PD patients showed a negative correlation with the vessel density in the DRCP central region. The presence of OH was a critical factor associated with central microvasculature damage in PD. These findings indicate that OCTA can be a useful and non-invasive tool for detecting microvasculature damage in PD patients.

PARAFOVEAL INNER RETINAL THINNING AS THE BIOMARKER PREDICTING LESS RECURRENCE OF MACULAR EDEMA IN CENTRAL RETINAL VEIN OCCLUSION AFTER DISCONTINUING ANTIVASCULAR ENDOTHELIAL GROWTH FACTOR.

PURPOSE: To identify the predictive factors for the recurrence of macular edema after the cessation of antivascular endothelial growth factor treatment in eyes with central retinal vein occlusion (CRVO). METHODS: This retrospective study included participants who had discontinued intravitreal bevacizumab injections for complete resolution of macular edema related to CRVO at 3 months after the last injection. Fifty-two eyes were enrolled in this study and classified into two groups based on the recurrence of macular edema within 1 year after the stopping point, when the decision to discontinue injections was made. Clinical characteristics and optical coherence tomographic parameters at baseline and at the stopping point were investigated. RESULTS: Multivariate logistic regression analysis demonstrated that, at baseline, old age was associated with a significantly higher risk of macular edema recurrence (odds ratio, 1.092; P = 0.022). At the stopping point, parafoveal inner retinal thickness (odds ratio: 1.043, P = 0.014) and the presence of ellipsoid zone disruption (odds ratio: 5.922, P = 0.032) were predictive factors for recurrence. The receiver operating characteristic curve showed that parafoveal inner retinal thinning of >7 µm compared with that in the fellow eye was significantly associated with decreased recurrence of macular edema. CONCLUSION: Parafoveal inner retinal thinning and intact ellipsoid zone after resolution of macular edema by antivascular endothelial growth factor treatment were predictive of a lower risk of recurrence of macular edema in CRVO. These intuitive biomarkers may help predict future disease courses and design optimal treatment strategies.

Myasthenia gravis with ocular symptoms following a ChAdOx1 nCoV-19 vaccination: A case report.

Purpose: We report on the case of a 35-year-old man who developed myasthenia gravis with ocular symptoms following a ChAdOx1 nCoV-19 vaccine injection. Observations: A 35-year-old man complained of binocular diplopia one month following ChAdOx1 nCoV-19 vaccination. He had weak infraduction of the left eye. Upper and lower extremity strength was normal on presentation. A serum antiacetylcholine receptor antibody titer was elevated at 1.60 nmol/L. His diplopia improved temporarily following the application of an ice pack for 2 min. Conclusions and importance: This case report describes a rare occurrence of myasthenia gravis with ocular symptoms as a potential complication of ChAdOx1 nCoV-19 vaccination.

OCULAR MANIFESTATIONS OF ASP38ALA AND THR59LYS FAMILIAL TRANSTHYRETIN AMYLOIDOSIS.

PURPOSE: To describe the ophthalmic manifestations of familial transthyretin amyloidosis (FTA) mutations, including Asp38Ala and Thr59Lys, which have not been previously reported to have ocular involvement. METHODS: This is an observational case series of prospectively collected data of 16 patients with FTA who were taking tafamidis for mild peripheral neuropathy and underwent a comprehensive ophthalmic examination at a single tertiary center, between January 2013 and March 2020. The ocular involvement of each FTA mutation type and the specific manifestations were the main outcome measures. RESULTS: Six of 16 patients with FTA manifested ocular involvement. Ocular involvement was noted in two of three patients with Glu89Lys mutations having retinal deposits, retinal hemorrhages, and corneal opacity. Three of nine patients with Asp38Ala mutations and one of two patients with Thr59Lys mutations showed ocular involvement that had not been previously described. The ophthalmic findings included glaucoma, anterior lens capsule opacity, vitreous opacity, and retinal deposits. The decrease in vascular flow due to perivascular cuffing of the amyloid deposits was detected by optical coherence tomography angiography. CONCLUSION: The current study newly described that two transthyretin mutation types of FTA, Asp38Ala and Thr59Lys, may manifest with ocular findings such as anterior lens capsule opacity and retinal deposits.

Deep learning models for screening of high myopia using optical coherence tomography.

This study aimed to validate and evaluate deep learning (DL) models for screening of high myopia using spectral-domain optical coherence tomography (OCT). This retrospective cross-sectional study included 690 eyes in 492 patients with OCT images and axial length measurement. Eyes were divided into three groups based on axial length: a "normal group," a "high myopia group," and an "other retinal disease" group. The researchers trained and validated three DL models to classify the three groups based on horizontal and vertical OCT images of the 600 eyes. For evaluation, OCT images of 90 eyes were used. Diagnostic agreements of human doctors and DL models were analyzed. The area under the receiver operating characteristic curve of the three DL models was evaluated. Absolute agreement of retina specialists was 99.11% (range: 97.78-100%). Absolute agreement of the DL models with multiple-column model was 100.0% (ResNet 50), 90.0% (Inception V3), and 72.22% (VGG 16). Areas under the receiver operating characteristic curves of the DL models with multiple-column model were 0.99 (ResNet 50), 0.97 (Inception V3), and 0.86 (VGG 16). The DL model based on ResNet 50 showed comparable diagnostic performance with retinal specialists. The DL model using OCT images demonstrated reliable diagnostic performance to identify high myopia.

ASSOCIATIONS WITH RECURRENCE OF MACULAR EDEMA IN BRANCH RETINAL VEIN OCCLUSION AFTER THE DISCONTINUATION OF ANTI VASCULAR ENDOTHELIAL GROWTH FACTOR.

PURPOSE: To identify factors predicting the recurrence of macular edema after the discontinuation of intravitreal antivascular endothelial growth factor injection in patients with branch retinal vein occlusion. METHODS: This retrospective study included only subjects who had discontinued injections at 3 months after the final bevacizumab injection due to fully resolved macular edema. Fifty-two eyes meeting the criteria were included in the study and divided into two groups (recurrence and no recurrence). Clinical features and measurements of retinal thickness at the time of the diagnosis and when the decision to stop injections was made (stopping point) were analyzed. RESULTS: At the stopping point, the no recurrence group showed a thinner parafoveal inner retina, better best-corrected visual acuity, and lower incidence of ellipsoid zone disruption in multivariate logistic regression analysis (all P < 0.05). Similarly, parafoveal inner retinal thinning of more than 30 µm, when compared with the corresponding region of the fellow eye or the unaffected region of the affected eye, was significantly related to less recurrence of macular edema. CONCLUSION: Thinning of the parafoveal inner retina as well as better vision and intact outer retinal layers are associated with a lack of recurrence of macular edema. These findings suggest that inner retinal atrophy after branch retinal vein occlusion may result in a reduction in oxygen demand in the affected retinal tissue and less production of vascular endothelial growth factor.

Factors affecting prediction error after cataract surgery with implantation of various multifocal IOLs in patients with previous refractive laser surgery.

BACKGROUND: This study aimed to compare the clinical outcomes of implantation of various multifocal intraocular lenses (mIOLs) and the prediction accuracy of two intraocular lens (IOL) power calculation formulas for eyes that underwent previous corneal refractive surgery. METHODS: Four types of mIOLs [TECNIS Symfony (Group I), AcrySof IQ PanOptix (Group II), LENTIS Mplus (Group III), and TECNIS ZLB00 (Group IV)] were used and the IOL power was calculated with the two no-history methods, Shammas-PL and Barrett True-K. Visual acuity and refractive outcomes including manifest refraction, prediction error (PE), absolute error (AE), and median absolute error (MedAE) were evaluated at three months after the cataract surgery. RESULTS: For all groups the Barrett True-K formula produced a narrower range of PEs and lower MedAE than Shammas-PL. Eyes of lower predictive accuracy (group B, AE >0.5D) showed weak uncorrected distance visual acuity resulting from myopic refractive error and target refraction when compared to that of higher predictive accuracy (group A, AE ≤0.5 D). CONCLUSIONS: Targeting emmetropia using the Barrett True-K, which considers both anterior and posterior corneal curvature is recommended in patients undergoing mIOL implantation with prior corneal refractive surgery. Additionally, history of prior large amount of laser ablation seems to be an important factor related to low predictive accuracy.

CHOROIDAL NEOVASCULARIZATION SECONDARY TO PHOTODYNAMIC THERAPY FOR CENTRAL SEROUS CHORIORETINOPATHY: Incidence, Risk Factors, and Clinical Outcomes.

PURPOSE: To investigate the incidence, risk factors, and clinical outcomes of complicated choroidal neovascularization (CNV) after reduced-fluence photodynamic therapy (PDT) for central serous chorioretinopathy. METHODS: This is a retrospective consecutive interventional study including 559 eyes from 520 patients treated by reduced-fluence PDT for central serous chorioretinopathy. The incidence of complicated CNV within 3 months after PDT was calculated. Baseline demographics and ocular findings were investigated to identify risk factors. The clinical course of complicated CNV was analyzed. RESULTS: Seven cases experienced development of active CNV after PDT within 3 months with a median interval of 4 weeks (range, 2-12 weeks). The incidence was estimated as 1.25% (95% CI, 0.55-2.62%). Complicated CNV developed in 6 among 138 eyes (4.35%) with flat irregular pigment epithelial detachment and in 1 among 421 eyes (0.24%) without flat irregular pigment epithelial detachment (P < 0.001, Fisher's exact test). The median best-corrected visual acuity recovered from 20/100 at the time of CNV development to 20/20 after anti-vascular endothelial growth factor injections (median, 3 times). CONCLUSION: In approximately 1%, reduced-fluence PDT for central serous chorioretinopathy may be complicated by CNV, which can be well controlled with anti-vascular endothelial growth factor injection. Flat irregular pigment epithelial detachment increases the risk of secondary CNV following PDT.

Computer-aided recognition of myopic tilted optic disc using deep learning algorithms in fundus photography.

BACKGROUND: It is necessary to consider myopic optic disc tilt as it seriously impacts normal ocular parameters. However, ophthalmologic measurements are within inter-observer variability and time-consuming to get. This study aimed to develop and evaluate deep learning models that automatically recognize a myopic tilted optic disc in fundus photography. METHODS: This study used 937 fundus photographs of patients with normal or myopic tilted disc, collected from Samsung Medical Center between April 2016 and December 2018. We developed an automated computer-aided recognition system for optic disc tilt on color fundus photographs via a deep learning algorithm. We preprocessed all images with two image resizing techniques. GoogleNet Inception-v3 architecture was implemented. The performances of the models were compared with the human examiner's results. Activation map visualization was qualitatively analyzed using the generalized visualization technique based on gradient-weighted class activation mapping (Grad-CAM++). RESULTS: Nine hundred thirty-seven fundus images were collected and annotated from 509 subjects. In total, 397 images from eyes with tilted optic discs and 540 images from eyes with non-tilted optic discs were analyzed. We included both eye data of most included patients and analyzed them separately in this study. For comparison, we conducted training using two aspect ratios: the simple resized dataset and the original aspect ratio (AR) preserving dataset, and the impacts of the augmentations for both datasets were evaluated. The constructed deep learning models for myopic optic disc tilt achieved the best results when simple image-resizing and augmentation were used. The results were associated with an area under the receiver operating characteristic curve (AUC) of 0.978 ± 0.008, an accuracy of 0.960 ± 0.010, sensitivity of 0.937 ± 0.023, and specificity of 0.963 ± 0.015. The heatmaps revealed that the model could effectively identify the locations of the optic discs, the superior retinal vascular arcades, and the retinal maculae. CONCLUSIONS: We developed an automated deep learning-based system to detect optic disc tilt. The model demonstrated excellent agreement with the previous clinical criteria, and the results are promising for developing future programs to adjust and identify the effect of optic disc tilt on ophthalmic measurements.