Case of persistent corneal epithelial damage after cataract surgery leading to diagnosis of vitamin A deficiency.

PURPOSE: To report our findings of reduced full-field electroretinograms (ff-ERGs) and abnormal optical coherence tomographic (OCT) images in a patient with poor visual acuity after cataract surgery who was eventually diagnosed with vitamin A deficiency (VAD). METHODS: This was a clinical study of a patient who complained of blurred vision after cataract surgery. To determine the cause of the reduced vision, we recorded full-field electroretinograms (ff-ERGs) to determine the scotopic and photopic status of the retina. We also performed optical coherence tomography to assess the changes in the retinal structure. Serological tests were performed. RESULTS: A 74-year-old man presented with persistent corneal epithelial damages and reduced vision that developed after conventional cataract surgery. OCT showed an interrupted ellipsoid zone, and fundus autofluorescence (FAF) showed a severe hypofluorescence in the retina of the left eye. The scotopic ff-ERGs were severely reduced, and the photopic ff-ERGs were mildly reduced. Serological examinations revealed a vitamin A concentration < 7 IU/dL (normal, 97-316 IU/dL). Based on these findings, we diagnosed the patient with VAD and started treatment with oral vitamin A supplements. After three months, his visual acuity, ff-ERGs, and OCT findings recovered to normal levels. The amplitudes and implicit times of the RETeval flicker ERGs increased to be within the normal range, and the hypofluorescence of the left eye disappeared. The length of the photoreceptor outer segments increased after the vitamin A supplementation. CONCLUSION: Our findings indicate that the ERGs are helpful for diagnosing patients with VAD associated with persistent corneal epithelial damages.

Subclinical Ocular Changes after Intravitreal Injections of Different Anti-VEGF Agents for Neovascular Age-Related Macular Degeneration.

Intraocular inflammations (IOIs) have been reported to occur after intravitreal injections of brolucizumab, and one of their causes has been suggested to be drug-specific features. We evaluated the anterior chamber by the aqueous flare value (AFV) and the retina by flicker electroretinography (ERG) after the initial intravitreal injection of aflibercept (IVA), brolucizumab (IVBr), or faricimab (IVF) for neovascular age-related macular degeneration (nAMD). The AFV and flicker ERGs were determined before, 2 weeks after, and 4 weeks after the injections in 14 eyes of 14 patients for each drug. After the injections, none of the patients had an IOI, but the AFV increased significantly in the IVA and IVF groups. The increase in the IVF group was +4.6 photon count/ms, which was significantly greater than in the other groups, but was not clinically significant. The implicit time was significantly prolonged in the IVBr group but unchanged in the IVA and IVF groups. These results suggest that brolucizumab, administered at high molar doses, may cause transient retinal disturbances that are not detectable by general ophthalmologic examinations but affect the implicit ERG times.

Transient Increase of Flicker Electroretinography Amplitudes after Cataract Surgery: Association with Postoperative Inflammation.

Purpose: To determine the characteristics and cause of the increase in the amplitude of flicker electroretinography (ERG) after cataract surgery. Design: Prospective, observational clinical study. Participants: Thirty patients who underwent cataract surgery. Methods: Flicker ERGs were recorded with the RETeval system without mydriasis. The central macular thickness (CMT) was measured by OCT and the aqueous flare value (AFV) by laser flare-cell photometry. These examinations were performed before surgery and 1 day, 1 week, 1 month, 2 months, and 3 months after surgery. Linear regression analysis through the origin was used to compare the correlations between the relative changes in flicker ERG amplitudes and the changes in the CMT and AFV at different times after the surgery. Main Outcome Measures: The amplitude of flicker ERGs, CMT, and AFV. Results: The mean amplitude of flicker ERGs increased significantly by 31% at 1 week after surgery (P < 0.001); a significant increase in the amplitudes was not present at 3 months after the surgery. The mean AFV was significantly increased at 1 day after surgery (P < 0.001), and the CMT was significantly increased at 1 to 3 months after surgery (P < 0.001). The changes in flicker ERG amplitudes at 1 week after surgery were significantly associated with the changes in the CMT at 1 to 3 months after surgery (P < 0.05), and they were weakly associated with the changes in AFV at 1 day after surgery (P = 0.05). Conclusions: These results suggest that the increase in the amplitude of flicker ERGs after cataract surgery is a transient phenomenon that has a peak at 1 week after surgery. The increase of flicker ERG amplitude was associated with measures that are frequently used to evaluate postoperative inflammation. Financial Disclosures: Proprietary or commercial disclosure may be found after the references.

Case of cystoid macular edema induced by systemic administration of paclitaxel: evaluations with electroretinograms.

PURPOSE: To report abnormal full-field electroretinograms (ERGs) in a patient with cystoid macular edema (CME) induced by systemic paclitaxel. METHODS: This is an observational case report. Full-field ERGs were recorded to evaluate the retinal function using the RETeval system and conventional ERGs using contact lens electrodes with built-in white light-emitting diodes. Optical coherence tomography (OCT) was also used to assess the retinal morphology. RESULTS: A 70-year-old man, who was diagnosed with gastric cancer, had undergone gastrectomy. Subsequently, systemic paclitaxel was administered once a week as an adjuvant therapy. After the tenth course of paclitaxel, he experienced blurred vision in both eyes and visited our department of ophthalmology. OCT revealed the presence of CME in both eyes, and the RETeval flicker ERGs showed a marked reduction in the amplitudes and a prolongation of the implicit times in both eyes. Conventional ERGs showed that the amplitudes of the oscillatory potentials (OPs) were also severely attenuated. The abnormal OCT findings and reduced visual acuity recovered to normal at 1 and 2 months, respectively, after the discontinuation of paclitaxel. However, the flicker ERGs did not recover to normal values until 4 months after the discontinuation of paclitaxel. CONCLUSION: These results suggest that the ERGs can be used to monitor the changes in the overall retinal function in patients receiving paclitaxel.

Effects of recording sequence on flicker electroretinographics recorded with natural pupils corrected for pupil area.

BACKGROUND: A new handheld electroretinographic (ERG) recording system can record ERGs without mydriasis. However, this RETeval system cannot record ERGs from both eyes simultaneously. Thus, the purpose of this study was to determine whether the sequence of the ERG recordings will alter the results. METHODS: We studied 30 eyes of 30 healthy subjects. The flicker ERGs were recorded with the RETeval system without mydriasis and were elicited by 8, 16 and 32 photopic Td-s. The flicker ERGs were recorded at two sessions. Session 1, the ERGs were recorded from the right eye and then the left eye, and Session 2, ERGs were recorded from the left eye then the right eye. We compared the implicit times, amplitudes and pupil diameters of the right eye between these two sessions. RESULTS: The implicit time of the flicker ERGs was significantly shorter (p < 0.001), and the pupil diameters were significantly smaller (p = 0.013) at Session 2 than Session 1 but only for the lower stimulus intensity of eight Td-s. There was a significant correlation of the differences in the implicit times and the differences in the pupil diameter between the two sessions (r = 0.406, p = 0.026). CONCLUSIONS: The results indicate that the implicit times of the fundamental components of RETeval flicker ERGs can be affected by the sequence of recordings for lower stimulus intensities. This was most likely due to the differences of the pupil diameter during the recordings. We recommend that stronger stimuli be used to record the RETeval flicker ERGs to minimize the effects of the sequence of recordings.

Case of lens-induced uveitis associated with supernormal flicker ERG amplitudes after cataract surgery.

PURPOSE: To report our findings in a case of lens fragment-induced uveitis associated with supernormal flicker electroretinograms (ERGs) twenty months after the cataract surgery. METHODS: This is an observational case report. Full-field flicker ERGs were recorded with the RETeval system. Optical coherence tomography (OCT) and slit-lamp biomicroscopy were used to assess the uveitis during the follow-up period. RESULTS: A 70-year-old man, who had undergone cataract surgery 20 months earlier, visited our hospital with a complaint of decreased vision in his right eye. Slit-lamp biomicroscopy revealed corneal edema and a lens fragment was detected in the inferior part of the anterior chamber. OCT showed cystoid macular edema, and flicker ERGs showed a marked increase in the amplitude and a delay in the implicit time in the right eye. These abnormalities of the flicker ERGs improved gradually after the removal of lens fragment and application of topical anti-inflammatory medications. CONCLUSION: Our case of lens-induced uveitis had supernormal flicker ERG amplitudes. Clinicians should be aware that eyes with uveitis can have larger-than-normal ERG amplitudes.

Factors Affecting Photopic Negative Response Recorded with RETeval System: Study of Young Healthy Subjects.

Purpose: To determine whether there is a significant correlation between the amplitude of the photopic negative response (PhNR) and the peripapillary retinal nerve fiber layer thickness (pRNFLT) in eyes of young, healthy subjects. Methods: We analyzed 136 eyes of 136 young, healthy subjects (89 males and 47 females; age, 20-29 years). The PhNRs were recorded with the RETeval system without mydriasis using red flashes on a blue background. PhNR amplitude was measured at two points: at 72 ms (P72) and at the negative trough following the b-wave (Pmin). Univariate and multivariable linear regression analyses were performed to identify the independent variables that were significantly correlated with P72 and Pmin. The variables included age, sex, axial length, pRNFLT, intraocular pressure (IOP), a-wave amplitude, b-wave amplitude, and pupillary area during the electroretinogram recordings. Results: The amplitudes of P72 and Pmin were significantly larger in female subjects (P = 0.021 and P = 0.001, respectively). Univariate analyses showed that PhNR amplitudes were significantly correlated with pRNFLT (P72: r = 0.246, P = 0.004; Pmin: r = 0.219, P = 0.011). Female sex was significantly and negatively correlated with P72 (r = -0.206; P = 0.016) and Pmin (r = -0.271; P = 0.001). Multivariable regression analyses showed that greater pRNFLT was an independent factor significantly associated with a larger P72 (r = 0.283; P = 0.004) and Pmin (r = 0.299; P = 0.002). Female sex was an independent factor that was significantly associated with a larger Pmin (r = -0.208; P = 0.022). Conclusions: These findings indicate that PhNR amplitude is significantly associated with pRNFLT and female sex in young, healthy subjects. Translational Relevance: The amplitude of the PhNR recorded with RETeval is smaller in subjects with thinner pRNFLT not only in glaucoma patients but also in young healthy subjects.

RDH5-Related Fundus Albipunctatus in a Large Japanese Cohort.

Purpose: To investigate clinical characteristics of RDH5-related fundus albipunctatus (FAP) in a Japanese cohort. Methods: Twenty-five patients from 22 pedigrees with RDH5-related FAP were studied. Ophthalmic medical records were reviewed. For genetic analysis, either Sanger sequencing of the RDH5 gene or whole-exome sequencing was performed. Results: Genetic analysis identified eight different RDH5 variants, including seven known RDH5 variants (p.G35S, p.G107R, p.R167H, p.A240GfsX19, p.R278X, p.R280H, and p.L310delinsEV) and a novel variant: c.259C>T (p.Q87X). The most frequently observed variant was p.L310delinsEV (65.2%, 30/46 alleles). Of 50 eyes examined, 44 eyes (88.0%) showed logMAR best-corrected visual acuity (BCVA) of 0.10 or better. In optical coherence tomography, macular involvement was observed in 12 patients (24 eyes). Ten patients (83.3%) who had good BCVA (0.10 or better) exhibited diffuse disruption of the outer retina with foveal sparing, and two patients (16.7%) exhibited diffuse disruption throughout the macula and decreased BCVA. Among the 24 eyes, ring-or crescent-shaped hyperautofluorescence or irregular autofluorescence around the fovea was observed in 15 eyes (83.3%) of 18 eyes examined by fundus autofluorescence imaging. Full-field electroretinography showed extinguished or severely decreased rod responses in all 23 examined patients, whereas decreased cone responses were seen in 17 patients (73.9%). Conclusions: Multimodal imaging and electroretinography of RDH5-related FAP revealed high frequencies of macular involvement in older patients and decreased cone responses. Our findings suggest that progressive macular/cone dysfunction, as well as delayed rod function, may be key phenotypic features of RDH5-related FAP.

Factors Affecting Mydriasis-Free Flicker ERGs Recorded With Real-Time Correction for Retinal Illuminance: Study of 150 Young Healthy Subjects.

Purpose: A small, full-field flicker electroretinogram (ERG) recording system was recently developed to record flicker ERGs without mydriasis (RETeval). The device delivers a stimulus with constant retinal illuminance by adjusting the retinal luminance to compensate for changes in the pupillary area. The purpose of this study was to determine what factors affect the fundamental components of the flicker ERGs recorded by RETeval in young healthy subjects. Methods: Flicker ERGs were recorded with the RETeval system from 150 eyes of 150 young healthy subjects (age, 20-29 years). Univariate and multivariate linear regression analyses were performed to identify the factors that affected the implicit times and amplitudes of the fundamental component of the flicker ERGs. The independent variables included age, sex, refractive error, axial length, and pupillary area. Results: Multivariate regression analyses indicated that a longer axial length (P = 0.03) and larger pupillary area (P = 0.008) were independent factors that were significantly associated with longer implicit times of the fundamental component of the flicker ERGs. Multivariate regression analyses also showed that the female sex (P = 0.03) was an independent factor, which was significantly associated with larger amplitude fundamental component of the flicker ERGs. Conclusions: These results indicate that the fundamental components of the RETeval flicker ERGs are significantly affected by the axial length, pupillary area, and sex of young healthy subjects. The results also suggest that it would be better to compensate for the Stiles-Crawford effect when flicker ERGs are recorded with natural pupils.