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Purpose: We describe the clinical findings in a Japanese patient with unilateral traumatic optic neuropathy (TON) who underwent steroid pulse therapy followed by optic canal decompression surgery. The optic nerve function was assessed longitudinally and quantitatively by Goldmann visual fields (GVFs). This was accomplished by measuring the area of each isopter and scotoma, and the findings were compared with the visual acuities recorded during the course of the resolution of the TON. Case Presentation: A 70‐year‐old man suffered from TON in his left eye after falling from a 3 m cliff. On examination at the Saitama Medical University Hospital, his decimal best corrected visual acuity (BCVA) in his left eye was 0.2. Computed tomography revealed a fracture of the lateral wall of the left orbit, but no obvious optic canal fracture was observed. Steroid pulse therapy was started, and optic nerve decompression surgery was performed. After steroid administration, there was a slight improvement in the visual acuity, and a response in the I/3e isopter was present (area 17.76 deg2). However, the V/4e area decreased from 539.35 to 359.36 deg2. Three days after the optic canal decompression surgery, the decimal visual acuity improved to 0.6, and the V/4e area and I/3e area increased to 1122.52 and 46.88 deg2, respectively. Postoperatively, there was a marked improvement in the size of the GVFs that corresponded to the improved visual acuity. The visual acuity of the left eye was 0.8 after 6 months, and the GVF was still not completely normal. Conclusions: Our findings showed the course of recovery of the visual acuity and visual field in an eye with TON. The quantification of GVF was helpful in assessing the course of recovery after the treatments. The new quantitative index of the GVFs may be helpful in evaluating the effectiveness of treatments for optic nerve disorders. [ABSTRACT FROM AUTHOR]

Purpose: To determine the physiological status of the retina by electroretinography (ERG) using skin electrodes and the RETevalTM system in eyes that had undergone pars plana vitrectomy (PPV) with silicone oil (SO) tamponade. The vitrectomy was performed for a retinal detachment and proliferative diabetic retinopathy (PDR). Design: Retrospective case series. Methods: ERGs were recorded with the RETevalTM system (LKC Technologies Inc. Gaithersburg, MD; Welch Allyn, Inc. Skaneateles Falls, NY) from eight eyes with PDR before and after the SO removal. The amplitudes and implicit times of the a- and b-waves of the ERGs before the SO removal were compared to that after the SO removal. Results: ERGs were recordable in four eyes before and after the SO removal and the a- and b-amplitudes improved in three eyes and worsened in one eye after the SO removal. In the remaining four eyes, ERGs were non-recordable both before and after the SO removal. Conclusion: These results indicate that ERGs picked up by skin electrodes can be used to assess the physiology of the retina in eyes with a SO tamponade. The flat ERGs in the SO-filled eye indicated the presence of diffuse retinal damage which was confirmed by the flat ERGs after the SO removal. Plain Language Summary: There has been an increasing number of reports on evaluating the retinal function using electroretinography (ERG) with skin electrodes. The main advantage of this system is the ability to record ERGs with a skin electrode that does not touch the cornea and ocular surface. This reduces the risk of infection especially in the postoperative period and in clinical situations where infection is suspected. In addition, there have been only a few reports evaluating the function of the retina by ERG in SO-filled eyes. We recorded ERGs with the RETeval (LKC Technologies Inc. Gaithersburg, MD; Welch Allyn Inc. Skaneateles Falls, NY) device, a relatively new ERG recording system that uses skin electrodes and is less invasive. We recorded ERGs from eight SO-filled eyes with proliferative diabetic retinopathy (PDR). In 4 SO filled eyes, the amplitudes increased in three eyes after the SO removal. In the other four eyes, ERGs were non-recordable before and after the SO removal. These results suggest that the RETeval system that uses skin electrodes can be used to assess the retinal function in PDR eyes with a SO tamponade. We suggest that the absence of ERGs in the SO filled eyes was not due to the electrical non-conductive effects of SO but may indicate the presence of diffuse retinal damage which was confirmed after the SO removal. [ABSTRACT FROM AUTHOR]

Purpose: To determine whether 0.025% povidone-iodine (0.025% PI) in the irrigation solution during vitrectomy for endophthalmitis is safe. Methods: Two cases of bleb-associated endophthalmitis were treated with vitrectomy using 0.025% PI in the irrigation solution. The RETevel electroretinographic (ERG) system with skin electrodes was used to assess the physiology of the retina pre- and postoperatively. Case: Case 1 was a 46-year-old man who had atopic keratoconjunctivitis and underwent trabeculectomy with the creation of a bleb after there was a rise in the intraocular pressure. One month postoperatively, a mild filtering bleb-associated endophthalmitis developed, and the eye was treated with intravitreal and subconjunctival injections of vancomycin (VCM) and ceftazidime (CAZ). After three days, the fundus was not visible and B-mode echography showed an area of high brightness in the retina. Vitrectomy with irrigation with VCM and CAZ, and 0.025% PI was performed successfully. Pre- and postoperative ERGs showed an improvement in both the a- and b-wave amplitudes and the b/a ratio was stable at 2.0. Case 2 was a 63-year-old man who had undergone trabeculectomy for glaucoma. Five years later, the eye developed blebitis which was treated with topical and subconjunctival injections of VCM and CAZ. Three days later, vitreous opacities appeared and a high brightness area was seen in the B-mode echographic images. Vitrectomy with VCM and CAZ, and 0.025% PI irrigation was successfully performed. Comparisons of the pre- and postoperative ERGs found that the a- and b- wave amplitudes of the ERGs increased and the b/a ratio was stable at approximately 1.5. Conclusion: Vitrectomy with 0.025% PI irrigation is safe and ERG recordings with skin electrodes can be used to evaluate the pre- and postoperative retinal physiology safely. Plain Language Summary: We report our findings in two cases of bleb-related endophthalmitis that underwent pars plana vitrectomy (PPV) with irrigation with 0.025% povidone-iodine (PI)-Balanced Salt Solution (BSS) PLUS (Alcon Laboratories, Fort Worth, TX). The retinal function was evaluated by full-field electroretinograms (ERGs) recorded with skin electrodes before and after the vitrectomy. At present, there is no consensus on whether patients with bleb-related endophthalmitis should be treated with PPV or treated solely by intravitreal antibiotics. It was recently reported that vitrectomy using an irrigation solution containing 0.025% PI followed by postoperative antibiotics was effective and safe for the treatment of endogenous endophthalmitis and postoperative endophthalmitis. However, iodine is known to be retinotoxic depending on its concentration. Electroretinography is a useful method to evaluate the safety of medications because it represents the physiology of the entire retina. However, conventional recording procedures with contact lens electrodes cannot be used in eyes with bleb associated endophthalmitis. Therefore, we performed ERG using skin electrodes which were introduced relatively recently. As a result, electroretinographic response showed that retinal function was not altered after surgery in these two cases. [ABSTRACT FROM AUTHOR]

Lacrimal drainage pathway disease-associated keratopathy (LDAK) has been associated with corneal perforation, which arises from both infectious and non-infectious corneal disorders. However, patients with corneal perforation are often not routinely tested for LDAK, and the potential risk posed by LDAK in the development of corneal ulcers has not been investigated in detail. This study aimed to assess the proportion and characteristics of LDAK in patients with non-infectious corneal perforation using lacrimal syringing test. This study enrolled 56 patients with corneal perforation treated at Saitama Medical University Hospital between January 2016 and September 2022. The causes of corneal perforation were trauma (n = 17, 30%), infection (n = 19, 34%), non-infection (n = 16, 29%), and unknown (n = 4, 7%). A lacrimal syringing test was performed on 12 patients with non-infectious corneal perforation and 4 with an unknown diagnosis. Among the 16 patients with non-infectious corneal perforation, 13 (81%) had lacrimal drainage disease, but only 3 (19%) patients had lacrimal puncta, as revealed by slit-lamp examinations. The primary bacterial species identified in lacrimal obstructive disease and lacrimal canaliculitis were Staphylococcus spp. and Actinomycetes spp. respectively. Lower temporal and peripheral corneal perforations were common. All patients underwent lacrimal surgery, and 6 (38%) were treated for corneal perforation without corneal surgery. Interestingly, several patients with LDAK who did not exhibit any lacrimal duct obstruction on slit-lamp examination. The study findings demonstrate the significance of the lacrimal syringing test for assessing LDAK in patients with corneal perforation, indicating LDAK as a potential cause of corneal perforation. [ABSTRACT FROM AUTHOR]

purpose of this article is to present an explanation of how MH developed in this eye without vitreous traction.Patient: A 64-year-old woman visited an eye clinic with vision reduction in her right eye of 3 days duration. A VH was detected in the right eye and pars plana vitrectomy (PPV) was performed. A retinal tear was detected which was the origin of the VH. The vision was restored to a decimal visual acuity of 1.2. Eight months later, the patient noticed that her vision was distorted and was referred to our hospital.Diagnosis: Optical coherence tomographic (OCT) images showed a thin epiretinal membrane on the macula, cystoid changes in the macular area, and a full-thickness MH.Interventions: The MH closed spontaneously in two weeks, however a lamellar MH with an epiretinal proliferation (EP) developed 11 months later. Two months later, OCT showed cyst-like changes in the retina and a full-thickness MH. A second PPV was performed with the insertion of the ILM flap and EP into the MH to close the MH. Her visual acuity improved, and distorted vision was not present.Lessons: Clinicians should be aware that a MH can develop in a vitrectomized eye without vitreous traction but can close spontaneously. We conclude that careful follow-up examinations are necessary even in vitrectomized eyes.Plain Language Summary: We report our findings in a case in which a macular hole (MH) developed 8 months after vitrectomy and spontaneously closed but then reopened. Finally, the MH was closed after a second vitrectomy. Optical coherence tomographic images showed changes in the macular microstructure, eg, the presence of a thin epiretinal membrane on the retina, cystoid changes in the retina, and a full-thickness MH. We discuss the mechanism of MH development and surgical treatment. [ABSTRACT FROM AUTHOR]

Long-acting (lasting extend) diquafosol ophthalmic solution 3% (DQSLX) is administered three times daily versus six times daily for the currently approved diquafosol ophthalmic solution (DQS). We investigated the efficacy and adherence of switching from DQS to DQSLX in patients with dry eye disease. We retrospectively enrolled 54 patients (17 men and 37 women) with eye drop prescription changes from DQS to DQSLX between December 2022 and March 2023. The number of eye drops, subjective symptoms, tear breakup time (TBUT), and fluorescein staining scores from baseline to 4 weeks after starting DQSLX were evaluated. Participants then chose between DQSLX and DQS. Patients administered DQSLX three times per day, as listed on the package insert, 88.9% of the time; significantly higher than the 5.6% of patients who used DQS six times per day, as instructed. The DQSLX group showed significant improvements in symptoms and fluorescein staining scores (23.3 ± 20.1 and 0.8 ± 1.7, respectively) compared with the baseline (37.8 ± 24.1 and 1.1 ± 1.5, p = 0.01 and <0.001, respectively). The TBUT in the DQSLX group (5.0 ± 2.5 s) did not significantly improve compared to the DQS group (4.5 ± 1.7 s) (p = 0.75). Fifty-one (94.4%) patients opted to continue DQSLX because of the pleasant feeling of the eye drops, long-lasting moisture, and less frequent administration. The efficacy and adherence of DQSLX was comparable to DQS. [ABSTRACT FROM AUTHOR]

Vitreoretinal lymphomas (VRLs) present with different clinical characteristics. However, only a few case reports have been published that evaluated the retinal function and the retinal morphology. The relationship between retinal morphology and function of eyes with a vitreoretinal lymphoma (VRL) was investigated via optical coherence tomography (OCT) and electroretinography (ERG). The ERG and OCT findings in 11 eyes of 11 patients (69.4 ± 11.5 years old) who were diagnosed with VRL at the Saitama Medical University Hospital between December 2016 to May 2022 were studied. The decimal best-corrected visual acuity ranged from hand movements to 1.2 (median 0.2). Histopathological studies of the vitreous specimens showed class II VRL in one eye, class III VRL in seven eyes, class IV VRL in two eyes, and class V VRL in one eye. The IgH gene rearrangement was positive in three of the six eyes tested. The OCT images showed morphological abnormalities in 10 of the 11 (90.9%) eyes. Severe attenuation was found for the amplitudes of the b-wave of the DA 0.01 ERG in 6 of 11 eyes (54.5%), the DA 3.0 a-wave in 5 of 11 eyes (45.5%), the DA 3.0 b-wave in 36.4%, the LA 3.0 a-wave in 36.4%, the LA 3.0 b-wave in 18.2%, and flicker responses in 36.4% of the eyes. None of the DA 3.0 ERGs had a negative shape (b/a < 1.0). In the five eyes in which the a-wave was severely attenuated, hyperreflective dots were observed subretinally. The ERG analysis in eyes with a VRL indicates a relatively severe dysfunction of the outer retinal layer and was helpful in determining the site of the morphological changes in eyes with VRL. [ABSTRACT FROM AUTHOR]

The extraction of the foveal avascular zone (FAZ) from optical coherence tomography angiography (OCTA) images has been used in many studies in recent years due to its association with various ophthalmic diseases. In this study, we investigated the utility of a dataset for deep learning created using Kanno Saitama Macro (KSM), a program that automatically extracts the FAZ using swept-source OCTA. The test data included 40 eyes of 20 healthy volunteers. For training and validation, we used 257 eyes from 257 patients. The FAZ of the retinal surface image was extracted using KSM, and a dataset for FAZ extraction was created. Based on that dataset, we conducted a training test using a typical U-Net. Two examiners manually extracted the FAZ of the test data, and the results were used as gold standards to compare the Jaccard coefficients between examiners, and between each examiner and the U-Net. The Jaccard coefficient was 0.931 between examiner 1 and examiner 2, 0.951 between examiner 1 and the U-Net, and 0.933 between examiner 2 and the U-Net. The Jaccard coefficients were significantly better between examiner 1 and the U-Net than between examiner 1 and examiner 2 (p < 0.001). These data indicated that the dataset generated by KSM was as good as, if not better than, the agreement between examiners using the manual method. KSM may contribute to reducing the burden of annotation in deep learning. [ABSTRACT FROM AUTHOR]

Inherited retinal diseases (IRDs) comprise a phenotypically and genetically heterogeneous group of ocular disorders that cause visual loss via progressive retinal degeneration. Here, we report the genetic characterization of 1210 IRD pedigrees enrolled through the Japan Eye Genetic Consortium and analyzed by whole exome sequencing. The most common phenotype was retinitis pigmentosa (RP, 43%), followed by macular dystrophy/cone‐ or cone‐rod dystrophy (MD/CORD, 13%). In total, 67 causal genes were identified in 37% (448/1210) of the pedigrees. The first and second most frequently mutated genes were EYS and RP1, associated primarily with autosomal recessive (ar) RP, and RP and arMD/CORD, respectively. Examinations of variant frequency in total and by phenotype showed high accountability of a frequent EYS missense variant (c.2528G>A). In addition to the two known EYS founder mutations (c.4957dupA and c.8805C>G) of arRP, we observed a frequent RP1 variant (c.5797C>T) in patients with arMD/CORD. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this study was to determine the effect of brilliant blue G (BBG) staining of the inner limiting membrane (ILM) on macular function. Method: Fourteen eyes of 14 patients consisting of 9 men and 5 women who underwent vitreous surgery with ILM peeling were studied. The mean age of the patients was 68.8 ± 9.14 years. Three eyes had a macular hole and eleven eyes had an epiretinal membrane. The ILM was made more visible by spraying 0.25% BBG into the vitreous cavity. The macular function was assessed by recording intraoperative focal macular electroretinograms (iFMERGs) before and after the intravitreal spraying of the BBG dye. The iFMERGs were recorded three times after core vitrectomy. The first recording was performed before the BBG injection (Phase 1, baseline), the second recording was performed after the spraying of the BBG and washing out the excess BBG (Phase 2), and the third recording was performed after the ILM peeling (Phase 3). All recordings were performed after 5 min of light-adaptation and stabilization of the intraocular conditions. The iFMERGs were recorded twice at each phase. The implicit times and amplitudes of the a- and b-wave, the PhNR, and the d-wave were measured. Wilcoxon signed-rank test were used to determine the significance of differences of the findings at Phase 2 vs. Phase 1 and Phase 3 vs. Phase 1. A p value < 0.05 was taken to be statistically significant. Results: The average implicit times of the a-wave, b-wave, PhNR, and d-wave were not significantly different in Phase 1, 2, and 3. The average a-wave, b-wave, PhNR, and d-wave amplitudes at Phase 1 did not differ significantly from that at Phase 2 and at Phase 3. Conclusions: The results indicated that the intravitreal injection of BBG does not alter the physiology of the macula, and we conclude that BBG is safe. We also conclude that iFMERGs can be used to monitor the macular function safely during intraocular surgery. [ABSTRACT FROM AUTHOR]

To evaluate the automated determination of the center of an idiopathic macular hole (MH) by using swept-source optical coherence tomography (OCT) images with new macro-based algorithms in ImageJ and to compare the difference between the MH center measurements obtained automatically and manually. This cross-sectional study included 39 eyes of 39 elderly individuals (22 women, 17 men) with stage 3 and 4 MH. The MH center was automatically determined using the ImageJ macro. The foveal center was also manually identified by two masked examiners using horizontal and vertical serial B-scan OCT angiography images. The mean age was 68.8 ± 8.3 years. After adjusting for the effect of magnification, the mean distance between the MH center determined manually by Examiner 1 and that determined automatically was 15.5 ± 9.9 µm. The mean distance between the two manually determined measurements of the MH center was 20.3 ± 19.7 µm. These two mean distance values did not differ significantly (Welch t-test, p = 0.27) and was non-inferior (p < 0.0001). The automated ImageJ-based method for determining the MH center was comparable to manual methods. This study showed that automated measurements were non-inferior to manual measurements, and demonstrated a substitutable usefulness, at least for use in clinical practice. [ABSTRACT FROM AUTHOR]

Vitreous temperature has been reported to vary during intraocular surgery. We measured the temperature at three intraocular sites, just posterior to the crystalline lens (BL), mid-vitreous (MV), and just anterior to the optic disc (OD), and investigated temperature changes before and after different types of surgical procedures in 78 eyes. The mean temperature at the beginning was 30.1 ± 1.70 °C in the anterior chamber, 32.4 ± 1.41 °C at the BL, 33.8 ± 0.95 °C at the MV, and 34.7 ± 0.95 °C at the OD. It was lowest at the BL, and highest at the OD. The mean temperature after cataract surgery was slightly lower especially at an anterior location. Thus, the temperature gradient became slightly flatter. The mean temperature after core vitrectomy was even lower at all sites and a gradient of the temperature was not present. The mean temperature after membrane peeling was significantly higher than that after core vitrectomy, and there was no gradient. The mean temperature after fluid/air exchange was lower at the BL and higher at the MV and at the OD. Thus, a gradient of higher temperatures at the OD appeared. The intraocular temperature distribution is different depending on the surgical procedure which can then change the temperature gradient. The temperature changes at the different intraocular sites and the gradients should be further investigated because they may affect the physiology of the retina and the recovery process. [ABSTRACT FROM AUTHOR]

Although intraocular lymphoma (IOL) mainly has have vitreous opacity and subretinal infiltration, its clinical symptoms are diverse. We report a case of IOL that mainly showed exudative retinal detachment in which analysis of IgH gene rearrangement (AIGHR) of the collected subretinal fluid sample was useful for diagnosis. A 77-year-old woman developed decreased left visual acuity for 1 month. She had been treated for dermatomyositis, diabetes mellitus, and right parotid tumor for 3 years. Visual acuity was 0.1 OD and counting fingers OS. Slit-lamp examination showed grade 4 (Emery-Little classification) nuclear cataract in both eyes and keratoprecipitates and tan vitreous opacity in the left eye. Fundoscopy details were unclear except for a vaguely observable optic nerve head due to yellow-brown vitreous opacity, which we judged as an old vitreous hemorrhage. Phacovitrectomy was performed and almost total retinal detachment was found, except for a part of the superior periphery. Since no retinal break was found and a wide range of thin membrane-like tissue was found on the surface of the retina, the surgeon suspected primary IOL and performed unplanned biopsy. The peripheral vitreous was collected as a sample, and then the subretinal fluid was collected through an intentional break to prevent mixing with other fluids. The subretinal strand was gently removed and collected. Cytology showed class III, the IL10/IL6 ratio was low, and AIGHR was positive. Postoperatively, fundus autofluorescence showed no abnormality, no leakage was observed on fluorescein and indocyanine green angiography, and the location of typical infiltration lesions under the retina was unclear. There were no positive findings on systemic examinations and a diagnosis of primary IOL was made. The main symptoms of this case were vitreous opacity and exudative retinal detachment, and AIGHR using subretinal fluid was useful for diagnosis. [ABSTRACT FROM AUTHOR]

The center of the fovea, termed the foveola, is the area of highest visual acuity, has the highest density of cone photoreceptors. We investigated the distance between the automatically-determined center of the foveal avascular zone (FAZ) and the manually-determined highest foveal bulge (FB) point using single swept-source optical coherence tomography angiography (OCTA) instrument. This cross-sectional study included 49 eyes of 49 individuals (34 women and 15 men; median age: 68 years) with no history of ocular disorders. The FAZ in the superficial capillary plexus was automatically determined using the Kanno–Saitama macro method, and the center of the FAZ was automatically determined using ellipse approximation. Another candidate foveal center, the highest FB point, was determined manually on the serial cross-sectional B-scan images. As a result, the foveal center was manually identified as the highest FB point on B-scan OCTA images. The center of the FAZ was more likely to be located inferior to the highest FB point (p = 0.031). In participants with a total (linear) distance of more than 50 μm between the center of the FAZ and the highest FB point, the displacement was significantly more in the horizontal direction than in the vertical direction (p = 0.017). These results can be applicable to further studies regarding the spatial relationships between the center of the FAZ and the highest FB point in various macular diseases or previously-treated eyes. [ABSTRACT FROM AUTHOR]

We describe a case of brolucizumab-related intraocular inflammation (IOI) detected using vitreous haze on optical coherence tomography (OCT) at an early stage before the patient was aware of any symptom. A 69-year-old female presented with decreased right vision. The patient was diagnosed with pachychoroidal neovasculopathy and started intravitreal aflibercept (IVA) with a 3+ treat-and-extend strategy (TAE). Although the serous retinal detachment (SRD) disappeared after IVA treatment, the patient was managed with treatment every 4 weeks without extending the treatment interval To shorten the treatment interval, intravitreal brolucizumab (IVBr) was started 44 weeks after starting IVA treatment. After initiating IVBr treatment, the SRD completely disappeared. However, 16 weeks after starting IVBr, OCT showed noise in the vitreous cavity, which had not been seen before, and infrared images showed a black smoke-like shadow over the macula. Despite these findings, the patient had no subjective symptoms, and so IVBr was re-administered with an 8-week TAE interval. Five days after IVBr treatment, vitreous inflammatory cells were observed, and the noise in the vitreous cavity and the smoke-like shadow in the infrared image were further enhanced. We diagnosed the patient with brolucizumab-related IOI, and anti-inflammatory treatment was initiated. After extensive treatment, the vitreous opacity gradually disappeared, and the vitreous noise on OCT and the black smoke-like shadow on infrared images disappeared. IOI may have already been present 16 weeks after starting IVBr treatment, when we judged that there was no inflammation and IVBr was re-administered. When following patients receiving IVBr, IOI may be detected by OCT at an earlier stage by evaluating vitreous haze. [ABSTRACT FROM AUTHOR]

Purpose: Optical coherence tomography angiography (OCTA) allows noninvasive observation of the retinal vasculature, and image analysis can be used to calculate the retinal vessel density and foveal avascular zone (FAZ) area. A previous study showed that macular vessel density and the signal strength index (SSI) of OCTA images increased significantly after cataract surgery. However, the effect of aging on OCTA analysis remains unclear. This study aimed to investigate age-related changes in macular vascularization while excluding the effects of cataracts. Patients and Methods: OCTA imaging of the macula was performed in adult patients who had undergone cataract surgery between February 2018 and May 2019 and in young healthy participants between April 2017 and April 2018. Results: The median (quartiles) age of the overall study population was 59.0 (27.0, 69.0) years, and no severe refractive error was observed (axial length: 24.2 ± 1.3 [mean ± SD] mm; spherical equivalent: 0.75 [− 2.25, 0.00] diopter). When we investigated the relationship between macular vessel density and age after excluding the effects of cataracts, we found that the macular vessel density showed age-related changes even in the absence of the effects of the SSI. However, the FAZ area was not affected by age. Conclusion: Macular vessel density decreased with age, even when the effects of cataracts were excluded. The effects of age and cataracts should be considered when designing studies and interpreting OCTA findings of the retinal vasculature. [ABSTRACT FROM AUTHOR]

Purpose: To describe the characteristics of Japanese patients with hydroxychloroquine (HCQ) retinopathy developing within 3 years of treatment outset. Study design: Retrospective case series Methods: Three patients with HCQ retinopathy developing within 3 years of treatment outset have been identified in Japan since HCQ became available in 2015. Their medical charts, containing optical coherence tomography (OCT), fundus autofluorescence imaging, and visual field tests, were reviewed. Results: The treatment durations and cumulative doses until onset were 29–36 months and 182–326 g, respectively. The first patient had possible pre-existing maculopathy, although the abnormalities were ambiguous. The second and third patients had impaired renal function. The patients did not complain of severe visual disturbance at diagnosis, but visual field loss and disruption of the outer retinal segments consisting of a parafoveal pattern in the first case and a pericentral pattern (localized, 8 or more degrees from the center of the fovea) in the second and third cases were clearly observed on OCT. Even after HCQ discontinuation, their retinopathy showed slight progression on the visual field tests and OCT images. A blood sample was obtained from 1 patient on the day after HCQ discontinuation, and the whole blood level of HCQ was measured using validated liquid chromatography-tandem mass spectrometry. The HCQ level 27 h after the last dose was high, at 2240 ng/mL (suggested threshold > 1733 ng/mL). Conclusion: Ophthalmologic screening from the initiation of HCQ treatment detected 3 cases of HCQ retinopathy developing within 3 years of treatment outset, including a patient with a high blood level of HCQ. [ABSTRACT FROM AUTHOR]

We describe the electroretinographic findings of a case of primary intraocular lymphoma (PIOL) wherein the patient received intravitreal injections of methotrexate (ivMTX). A 62-year-old man developed blurred vision and complained of decreased visual acuity (VA) in his right eye. Fundus examination showed vitreous opacity and multiple subretinal yellowish lesions. Optical coherence tomography (OCT) revealed subretinal and intraretinal infiltrations. The full-field electroretinogram (ffERG) showed subnormal combined rod-cone response and multifocal electroretinogram (mfERG) recorded using skin electrodes showed severe attenuation of the response compared with the other eye. Pars prana vitrectomy, phacoemulsification, and lens implantation were performed to remove the opacity, and vitreous biopsy revealed a high ratio of interleukin 10–6 (76.0). There was no systemic malignant lesion, and the patient was diagnosed with PIOL. Treatment with ivMTX (400 μg/0.1 mL) was started. One month later, the intraretinal infiltration had disappeared, and mfERG revealed recovery of the response density from the central area. Two months later, OCT showed recovery of the foveal ellipsoid and interdigitation zones, and VA recovered to 20/17; mfERG showed maintenance of macular function. However, the amplitude of a- and b-waves in the ffERG gradually decreased. Macular function recovered, but there was also a decrease in total retinal function. mfERG and ffERG recorded using skin electrodes were useful in monitoring macular and entire retinal function with repeated examinations and showed recovery and maintenance of macular function in a case of PIOL treated with ivMTX. [ABSTRACT FROM AUTHOR]

Inherited retinal disorder (IRD) is a leading cause of blindness, and CRX is one of a number of genes reported to harbour autosomal dominant (AD) and recessive (AR) causative variants. Eighteen patients from 13 families with CRX-associated retinal disorder (CRX-RD) were identified from 730 Japanese families with IRD. Ophthalmological examinations and phenotype subgroup classification were performed. The median age of onset/latest examination was 45.0/62.5 years (range, 15–77/25–94). The median visual acuity in the right/left eye was 0.52/0.40 (range, −0.08–2.00/−0.18–1.70) logarithm of the minimum angle of resolution (LogMAR) units. There was one family with macular dystrophy, nine with cone-rod dystrophy (CORD), and three with retinitis pigmentosa. In silico analysis of CRX variants was conducted for genotype subgroup classification based on inheritance and the presence of truncating variants. Eight pathogenic CRX variants were identified, including three novel heterozygous variants (p.R43H, p.P145Lfs*42, and p.P197Afs*22). A trend of a genotype-phenotype association was revealed between the phenotype and genotype subgroups. A considerably high proportion of CRX-RD in ADCORD was determined in the Japanese cohort (39.1%), often showing the mild phenotype (CORD) with late-onset disease (sixth decade). Frequently found heterozygous missense variants located within the homeodomain underlie this mild phenotype. This large cohort study delineates the disease spectrum of CRX-RD in the Japanese population. [ABSTRACT FROM AUTHOR]

Changes in retinal vasculature and ocular circulation may play an important role in the glaucoma development and progression. We evaluated the vertical asymmetry across the temporal raphe of the deep retinal layer vessel density, using swept-source optical coherence tomography angiography (SS-OCTA), and its relationship with the central visual field (VF) loss. Thirty-four eyes of 27 patients with open-angle glaucoma were included. SS-OCTA macular scanning was performed within a 3 × 3 mm (300 × 300 pixels) volume, centred on the fovea. The relationships between the vertical asymmetrical deep retinal vessel density reduction (ADRVD) across the temporal raphe and various ocular parameters were analysed. Twenty-two glaucomatous eyes with ADRVDs had central VF loss. Contrarily, ADRVDs were not found in any of the 12 eyes without central VF loss. Thirteen eyes (59.1%) with central VF loss had ADRVDs topographically corresponding to the central VF loss and macular ganglion cell complex thinning. The glaucomatous eyes with ADRVDs exhibited inferior rather than superior central VF loss (P = 0.032). Thus, ADRVD specifically indicates the glaucomatous central visual loss. Further analysis of ADRVD may improve our understanding on glaucoma pathogenesis, offering new treatment insights. [ABSTRACT FROM AUTHOR]

Biallelic variants in the EYS gene are a major cause of autosomal recessive inherited retinal disease (IRD), with a high prevalence in the Asian population. The purpose of this study was to identify pathogenic EYS variants, to determine the clinical/genetic spectrum of EYS-associated retinal disease (EYS-RD), and to discover disease-associated variants with relatively high allele frequency (1%-10%) in a nationwide Japanese cohort. Sixty-six affected subjects from 61 families with biallelic or multiple pathogenic/disease-associated EYS variants were ascertained by whole-exome sequencing. Three phenotype groups were identified in EYS-RD: retinitis pigmentosa (RP; 85.94%), cone-rod dystrophy (CORD; 10.94%), and Leber congenital amaurosis (LCA; 3.12%). Twenty-six pathogenic/disease-associated EYS variants were identified, including seven novel variants. The two most prevalent variants, p.(Gly843Glu) and p.(Thr2465Ser) were found in 26 and twelve families (42.6%, 19.7%), respectively, for which the allele frequency (AF) in the Japanese population was 2.2% and 3.0%, respectively. These results expand the phenotypic and genotypic spectrum of EYS-RD, accounting for a high proportion of EYS-RD both in autosomal recessive RP (23.4%) and autosomal recessive CORD (9.9%) in the Japanese population. The presence of EYS variants with relatively high AF highlights the importance of considering the pathogenicity of non-rare variants in relatively prevalent Mendelian disorders. [ABSTRACT FROM AUTHOR]

Background: Macular hole (MH) is a retinal break in the fovea involving partial or complete dehiscence of the neural retinal layers affecting the visual quality by decreasing visual acuity (VA) and visual deformation. We describe a case of secondary MH associated with submacular hemorrhage (SMH) due to polypoidal choroidal vasculopathy (PCV), which showed spontaneous closure.Case Presentation: A 67-year-old man developed decreased VA in his right eye due to an SMH. The VA was 20/50, and monthly intravitreal injection of aflibercept was administered three times. The SMH gradually decreased, and 10 months later the external limiting membrane was found to be perforated, resulting in MH. The old clot disappeared, and the MH remained for 10 months. Twenty-three months later, serous retinal detachment (SRD) involving the macula appeared and the MH had disappeared. SRD gradually disappeared, and macular configuration recovered. VA gradually improved and became 20/20 38 months later.Conclusion: Dynamic change of the ultrastructure in an unusual case of secondary-developed and spontaneously closed MH was clearly observed. Although the mechanism was unknown, the small diameter size and exudative PCV are thought to have contributed to the closure. [ABSTRACT FROM AUTHOR]

Purpose: In various ophthalmic surgical procedures, 0.25% povidone-iodine (PI) solution is used to prevent infections. This study examined the bacterial colonisation before and after endoscopic nasolacrimal duct intubation irrigated with PI solution and investigated the relationship between bacterial detection and surgical failure. Methods: A retrospective study at Saitama Medical University Hospital. We enrolled 113 patients (33 men, 80 women) who underwent lacrimal intubation surgery between April 2016 and December 2018. Preoperatively, the lacrimal pathways were washed with normal saline solution, which was subsequently cultured. The sites of obstruction in the lacrimal pathway were endoscopically determined, perforated, and intubated. The tubes were afterward either irrigated with 0.25% PI or normal saline every 2 weeks. After 8 weeks, a piece of the removed lacrimal tube was cultured. We characterized the pre- and postoperative bacterial cultures. Results: We enrolled 54 and 52 patients in PI and saline groups (mean age: 69.1± 9.2 and 73.2± 7.2 years), respectively. In the PI group, Staphylococcus epidermidis was less frequently detected postoperatively than preoperatively. S. oralis and Candida parapsilosis were identified more often postoperatively (P=0.02, P=0.01, respectively). The PI group had significantly lower bacterial detection rate than the saline group (P=0.01). However, the surgical failure rates did not differ between groups. In surgical failure patients, the postoperative rate of Pseudomonas aeruginosa was very high (75%). Conclusion: PI reduces the bacterial detection rate but does not improve the surgical success rate. P. aeruginosa is associated with an increased risk of surgical failure. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the association between axial length and crystalline lens biometry with and without accommodation using the CASIA2 swept-source (SS) optical coherence tomography (OCT) system on the anterior segment. Study design: Intervention study. Methods: Individuals aged 20 to 39 years were enrolled for SS-OCT imaging at 2 separate visits within 1 week. Each eye was imaged under accommodation stimuli with different amplitudes (0 D, -1 D, -3 D, -5 D, and -7 D). The anterior and posterior lens curvature radii and lens thicknesses were measured. The participants were divided into 4 groups according to axial length. Results: Forty-eight participants (96 eyes) were initially enrolled. All 96 eyes were included in the analyses, as they met the eligibility criteria and each had proper OCT images. The mean participant age was 25.9 ± 4.3 years, and the mean spherical error was -3.5 ± 2.5 D. The mean axial length was 25.1 ± 1.2 mm. Multivariate analysis revealed that the anterior curvature radius became flatter and the lens thickness became thicker as the axial length increased (all P <.01). Furthermore, axial length independently contributed to the shape of the lens after adjusting for the effects of age and accommodation stimuli. Conclusion: Axial length was associated with lens shape after adjusting for age and accommodation stimuli. These measurements can provide a glimpse of dynamic lens biometry changes, which may help us better understand the role of lenses in various physiologic changes, including accommodation and myopia. [ABSTRACT FROM AUTHOR]

The purpose of this study was to evaluate the retinal function by electroretinograms (ERGs) recorded with the RETeval system using skin electrodes after pars plana vitrectomy (PPV) with gas tamponade in eyes with a rhegmatogeneous retinal detachment (RRD). Flicker ERGs were recorded from 17 eyes with an RRD before (baseline), within 2 weeks after the PPV when the size of the tamponade gas was approximately one-half of the vitreous cavity (P1), and when the gas had been completely absorbed (P2). The amplitudes of the flicker ERGs at each phase were compared to that at the baseline. The median (25th, 75th percentile) of the amplitude was 10.0 µV (5.5, 13.0 µV) at the baseline, 11.7 µV at P1 (7.8, 14.8 µV; P = 0.003), and 17.1 µV at P2 (11.7 23.3 μV; P < 0.001). The ratio of the amplitudes in the affected eye to that in the fellow eye at the baseline and at each phase was calculated, and the ratio of the amplitudes at P1 and P2 were significantly and positively correlated (P = 0.723, P = 0.001; Spearman's rank correlation coefficient). We conclude that recordings the flicker ERGs with skin electrodes can be used to assess the physiology of eyes even with the vitreous cavity half-filled with the gas used to tamponade the retina. [ABSTRACT FROM AUTHOR]

Purpose: To describe a Japanese girl with unilateral optic neuritis who was seropositive for the anti-myelin-oligodendrocyte glycoprotein (MOG). Serial recordings of the pattern visual evoked potentials (pVEPs) were made to follow the dynamic changes of the disease activity. Observations: A 5-year-old girl developed a sudden reduction of vision and deep ocular pain in her right eye. On examination at our university hospital, the best-corrected visual acuity (BCVA) was light perception, and a swelling of the optic disc and tortuous vessels at the posterior pole of the right eye were observed. MRI demonstrated that her right optic nerve was hyperintense on short TI inversion recovery (STIR) sequence. A diagnosis of right papillitis was made, and she was treated with steroid pulse therapy followed by a gradual tapering of oral prednisolone. The visual acuity decreased to no light perception and plasmapheresis combined with high-dose intravenous immunoglobulin therapy was performed. The decimal visual acuity rapidly improved and recovered to 1.2, and no recurrence was observed for at least 1 year. On day 19, she was found to be anti-MOG antibody positive and anti-Aquaporin 4 antibody negative. pVEPs were recorded during the course of the disease process which showed the dynamic changes of the physiology of the visual pathways. The implicit times of the N75 and P100 components were prolonged in the right eye in the acute phase. The right visual acuity remained at 1.2 for at least 1 year, but the implicit times of the N75 and P100 components of the pVEPs of the right eye were still prolonged compared to left eye. Conclusion: Our findings indicate a positive relationship between the anti-MOG antibodies-positivity and the prolonged pVEPs. Further analyses of the pVEPs and other clinical findings of the optic neuritis are needed to establish the clinical significance of the anti-MOG antibodies positivity and optic neuritis for the diagnosis, treatment, and prognosis for this disease. [ABSTRACT FROM AUTHOR]

Purpose. To determine the feasibility of performing intraocular surgeries in a heads-up position with low illuminance conditions by observing a display of the surgical field created by a three-dimensional imaging (3D) system. Methods. Seventy-four eyes of 56 patients underwent cataract surgery (72 eyes) with the heads-up 3D surgery system; 60 eyes with cataract surgery alone, 7 eyes with combined cataract and glaucoma microdevice implant surgery, 5 eyes with combined cataract and vitrectomy surgery, and two eyes with vitrectomy surgery alone were studied. The illuminance from the surgical microscope was set to be dimmer (Leica M822F40 main light 2%; otto-flex 6%) than the usual setting to minimize the discomfort and glare for the patient. The surgeries were performed under topical anesthesia. The luminance of the images observed through the eyepieces of the operating microscope and the image of a 3D system created by a high-sensitivity sensor Exmor R 3CMOS HD camera (Sony MCC-1000MD) were measured. Results. All surgeries were completed without any complications under the low illumination conditions. The surgical field on the display monitor was created by a 3D system using a high-sensitivity sensor camera and was observed in a heads-up position. The patients did not report any intolerable discomfort or glare during the surgery. Cataract surgeries were performed with a good view of the surgical field under the extremely low illumination from the surgical microscope. The high-sensitivity sensors and electronic amplifications of the image signals made the surgical field brighter and allowed the surgeon to perform the surgery confidently and safely. Conclusions. Heads-up, 3D-assisted intraocular surgeries can be performed safely and efficiently with low illuminance of the surgical field. This trial is registered with UMIN000037838. [ABSTRACT FROM AUTHOR]

The present report aimed to describe the macular structure's recovery process in a case of optic disc pit maculopathy (ODP-M) with outer layer hole following pars plana vitrectomy (PPV) with juxtapapillary laser treatment (JPL). We performed repeated optical coherence tomography (OCT) examinations to evaluate the macular structural changes. An 80-year-old man presented with distorted vision and decreased visual acuity (VA) in his left eye, experienced for 1 year, prior to presentation. Fundus examination and OCT showed intraretinal fluid (IRF) in the inner and outer retinal layers. Serous retinal detachment (SRD) with an outer layer hole in the macula was also evident. The IRF was connected to the optic disc; however, the SRD was isolated. Best-corrected VA was 20/100. PPV combined with JPL was performed. Posterior vitreous detachment creation and tamponade with sulfur hexafluoride was performed. Postoperatively, the inner retinal IRF at the fovea disappeared. The outer layer hole gradually closed and had completely disappeared 1 month postoperatively. After resolution of the outer layer hole, SRD reduced gradually and disappeared 8 months postoperatively, although the macular outer retinal IRF remained. The outer retinal IRF had partially resolved by the 18th postoperative month. Macular structure was completely recovered 31 months postoperatively, with an improved VA of 20/20. In conclusion, SRD might be associated with outer retinal IRF and outer layer holes. In cases of ODP-M, outer layer holes might induce optic disc-isolated SRD. [ABSTRACT FROM AUTHOR]

Purpose: To assess the physiology of the retina by electroretinography (ERG) with skin electrodes in eyes that had undergone vitrectomy with silicone oil (SO) tamponade. Design: Retrospective case series. Method: ERGs were recorded from eleven eyes with complex vitreoretinal disorders and from the normal fellow eyes. The affected eyes underwent pars plana vitrectomy (PPV) with SO tamponade. ERGs were recorded before and after the SO was removed. The amplitudes and implicit times of the a- and b-waves of the affected eyes were compared to those of the normal fellow eyes. In addition, the ratios of the amplitudes of the b-waves of the affected eyes to those of the fellow eyes were compared before and after the SO was removed. Results: ERGs were recordable from 7 eyes (63.6%) before the SO was removed and 11 eyes (100%) after the SO was removed. The a- and b-wave amplitudes were significantly smaller in the affected eyes than those of the fellow eyes at the baseline. The b-wave amplitude before the removal of the SO was significantly and positively correlated with that after the SO removal. The ratios of the b-waves of the affected/normal fellow eye significantly increased after the SO was removed. Conclusion: The results indicate that ERGs picked up by skin electrode can be used to assess the physiology of the retina in eyes with a SO tamponade. The amplitude of the b-waves of the ERGs in silicone-filled eyes can be used to predict the amplitude after the silicone is removed. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate microvascular changes in the macular area of eyes with rhegmatogenous retinal detachment (RRD) without macular involvement (macula-on RRD) using swept-source optical coherence tomography angiography (SS-OCTA). Patients and methods: Five patients with macula-on RRD were eligible for analysis. All patients underwent SS-OCTA examination (Triton) for the macular area. The healthy fellow eyes were included as controls. The vessel density (VD) was calculated using binarization, and the foveal avascular zone (FAZ) was measured. The VD and FAZ area were compared between the eyes with RRD and the fellow eyes using the Wilcoxon signed rank test. Results: The patients' clinical characteristics were as follows: age, 49.0 years (21.0, 54.0) (median [25, 75th percentile]); preoperative best-corrected visual acuity, -0.08 (-0.08, 0.11) for RRD and -0.08 (-0.08, -0.03) for the fellow eye (P=0.50); and axial length, 27.0 (25.1, 28.7) mm for RRD and 27.4 (25.6, 28.5) mm for the fellow eye (P=0.31). The parafoveal VD was not significantly different between the eyes with RRD and the fellow eyes (P=1.00 for the superficial retina and P=0.44 for the whole retina). The FAZ area was also similar for the eyes with RRD and the fellow eyes (P=0.31 for the superficial retina and P=0.13 for the whole retina). Conclusion: The findings of this study suggest that the macular microvasculature remains intact in eyes with macula-on RRD. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the ability of enhanced-depth imaging (EDI) optical coherence tomography angiography (OCTA) to detect vascular signals inside the glaucomatous optic disc.Patients and methods: Ten glaucomatous eyes of 8 subjects and 11 nonglaucomatous eyes of 7 subjects underwent EDI and conventional OCTA for optic disc analysis. The optic disc vessel density (VD) at maximum scan depth (2 mm) was compared between the 2 methods and between glaucomatous and nonglaucomatous eyes. Regression analysis was used to determine the factors affecting disc VD.Results: The median (25th, 75th percentile) of the visual field mean deviation was –20.5 (–25.1, –13.5) dB in glaucoma. The disc VD measured with the EDI method was significantly greater than that measured with the conventional method in glaucoma and nonglaucoma. The disc VD was also significantly lower in glaucoma than in nonglaucoma. The disc VD and mean deviation were significantly and positively correlated in both methods (conventional: R2=0.27, EDI: R2=0.22).Conclusion: The correlation between disc VD and glaucoma severity was comparable between conventional and EDI measurements. Images obtained with EDI-OCTA have the additional benefit of revealing more vascular signals in deeper layers. The disc VD, as determined by both conventional and EDI-OCTA methods, may be an indicator of glaucoma severity. [ABSTRACT FROM AUTHOR]

Background: Patients with Leber hereditary optic neuropathy (LHON) have a progressive decrease of their visual acuity which can deteriorate to <0.1. Some patients can have a partial recovery of their vision in one or both eyes. One prognostic factor associated with a recovery of vision is an early-age onset. The purpose of this study was to determine other clinical factors that are predictive of a good visual recovery.Methods: Sixty-one Japanese LHON patients, with the 11,778 mutation and a mean age of 23.1 ± 12.1 years at the onset, were studied. All patients were initially examined at an acute stage of LHON and were followed for 3 to 10 years. At 1 year after the onset, the lowest visual acuity was <0.1 in all eyes. We studied the following parameters of patients with/without a final visual acuity of ≥ 0.2: sex; heavy consumption of cigarettes and alcohol; taking idebenone; mean age at onset; mean lowest visual acuity; and distribution of the lowest and the final visual acuity.Results: Fifteen (24.6%) of the 61 patients or 25 (20.5%) of the 122 eyes had a recovery of their visual acuity to ≥ 0.2. The mean age at onset of these 15 patients with visual recovery to ≥ 0.2 was 17.5 ± 7.7 years, and that of the 46 patients without visual recovery to ≥ 0.2 was 25.0 ± 12.8 years (P = 0.02, Mann-Whitney U test). The mean lowest visual acuity of the 25 eyes with visual recovery ≥ 0.2 was 0.04, and that of the 97 eyes without visual recovery to ≥ 0.2 was 0.015 (P < 0.001, Mann-Whitney U test). Fifty percent (15/30) of the eyes whose lowest visual acuity was ≥ 0.04 during 1 year after the onset had a visual recovery to ≥ 0.2, while 11% (10/92) of the eyes whose the lowest visual acuity was ≤ 0.03 had a visual recovery to ≥ 0.2 (P < 0.001, χ 2 test). There were no significant differences in the other clinical factors.Conclusion: A final visual acuity of ≥ 0.2 was associated with a less severe reduction of the visual acuity at 1 year after the onset. Our findings can be used to predict the visual prognosis in LHON patients. [ABSTRACT FROM AUTHOR]

Study Population i **: **September 2019** Back Matter Correction There was an error in the original publication [[1]]. Reference 1 Sasaki T., Shoji T., Kanno J., Ishii H., Yoshikawa Y., Ibuki H., Shinoda K. Automatic Determination of the Center of Macular Hole Using Optical Coherence Tomography En Face Images. Automatic Determination of the Center of Macular Hole Using Optical Coherence Tomography En Face Images. [Extracted from the article]

Purpose: To describe the clinical and genetic findings in a patient with autosomal recessive bestrophinopathy (ARB) and his healthy parents. Methods: The patient and his healthy non-consanguineous parents underwent detailed ophthalmic evaluations including electro-oculography (EOG), spectral-domain optical coherence tomography (SD-OCT), and fundus autofluorescence (FAF) imaging. Mutation analysis of the BEST1 gene was performed by Sanger sequencing. Results: The FAF images showed multiple spots of increased autofluorescence, and the sites of these spots corresponded to the yellowish deposits detected by ophthalmoscopy. SD-OCT showed cystoid macular changes and a shallow serous macular detachment. The Arden ratio of the EOG was markedly reduced to 1.1 in both eyes. Genetic analysis of the proband detected two sequence variants of the BEST1 gene in the heterozygous state: a novel variant c.717delG, p.V239VfsX2 and an already described c.763C>T, p.R255W variant associated with Best vitelliform macular dystrophy and ARB. The proband's father carried the c.717delG, p.V239VfsX2 variant in the heterozygous state, and the mother carried the c.763C>T, p.R255W variant in the heterozygous state. The parents who were heterozygous for the BEST1 variants had normal visual acuity, EOG, SD-OCT, and FAF images. Conclusions: In a truncating BEST1 mutation, the phenotype associated with ARB is most likely due to a marked decrease in the expression of BEST1 promoted by the nonsense-mediated decay surveillance mechanism, and it may depend on the position of the premature termination of the codon created. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate retinal function by intraoperative electroretinograms (ERGs) before and after core vitrectomy. Design: Retrospective consecutive case series. Method: Full-field photopic ERGs were recorded prior to the beginning and just after core vitrectomy using a sterilized contact lens electrode in 20 eyes that underwent non-complicated vitreous surgery. A light-emitted diode was embedded into the contact lens, and a stimulus of 150 ms on and 350 ms off at 2 Hz was delivered. The amplitudes and latencies of the a-, b-, and d-waves, photopic negative response (PhNR), and oscillatory potentials (OPs) were analyzed. The intraocular temperature at the mid-vitreous was measured at the beginning and just after the surgery with a thermoprobe. Results: The intraocular temperature was 33.2 ± 1.3°C before and 29.4 ± 1.7°C after the vitrectomy. The amplitudes of the PhNR and OPs were significantly smaller after surgery, and the latencies of all components were prolonged after the surgery. These changes were not significantly correlated with the changes of the temperature. Conclusion: Retinal function is reduced just after core vitrectomy in conjunction with significant temperature reduction. The differences in the degree of alterations of each ERG component suggests different sensitivity of each type of retinal neuron. [ABSTRACT FROM AUTHOR]

Purpose: To describe a new technique to record focal macular electroretinograms (FMERGs) during vitrectomy to assess macular function. Methods: Intraoperative FMERGs (iFMERGs) were recorded in ten patients (10 eyes) who undergo vitrectomy. iFMERGs were elicited by focal macular stimulation. The stimulus light was directed to the macular area through a 25 gauge (25G) glass fiber optic bundle. Background light was delivered through a dual chandelier-type light fiber probe. Focal macular responses elicited with combinations of stimulus and background luminances were analyzed. Results: A stimulus luminance that was approximately 1.75 log units brighter than the background light was able to elicit focal macular responses that were not contaminated by stray light responses. Thus, a stimulus luminance of 160 cd/m2 delivered on a background of 3 cd/m2 elicited iFMEGs from only the stimulated area. This combination of stimulus and background luminances did not elicit a response when the stimulus was projected onto the optic nerve head. The iFMERGs elicited by a 10° stimulus with a duration of 100 ms and an interstimulus interval of 150 ms consisted of an a-, b-, and d-waves, the oscillatory potentials, and the photopic negative response (PhNR). Conclusions: Focal ERGs with all components can be recorded from the macula and other retinal areas during vitreous surgery. This new technique will allow surgeons to assess the function of focal areas of the retina intraoperatively. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the photoreceptor inner and outer segment layer thickness in eyes with MEWDS. Design: Prospective, non-comparative, observational case series. The follow-up duration was 4 months. Methods: Four women were diagnosed with unilateral MEWDS. The ages of the patients were 25, 24, 35, and 40 years. The retinal microstructure was assessed by spectral-domain optical coherence tomography (SD-OCT). The thickness of the photoreceptor inner (IS) and outer (OS) segments and sum of them (IS + OS) at the fovea were analyzed. Results: The visual acuity was reduced in three of four eyes at the acute phase. SD-OCT showed that the border of IS and OS (IS/OS) line and the cone outer segment tips (COST) line in the macula area were not detected in all four eyes. The IS + OS thickness was 50.3 ± 5.6 μm and that of the healthy fellow eyes was 73.5 ± 7.0 μm ( n = 4 eyes). The thickness of the IS was 27.8 ± 2.6 μm and that of the OS was 45.8 ± 7.3 μm. In all eyes, there was a spontaneous improvement of the visual acuity. SD-OCT showed a recovery of only the IS/OS line in the macular area, but the COST line was not visible in three cases. The mean IS + OS thickness increased to 56.0 ± 7.9 μm ( n = 4), IS = 26.0 ± 2.0 μm ( n = 3), and OS = 30.1 ± 8.7 μm ( n = 3) in the early recovery phase, and to 64.8 ± 9.3 μm ( n = 4), IS = 28.5 ± 1.7 μm ( n = 4), and OS = 36.3 ± 7.9 μm ( n = 4) in the late recovery phase. The mean inner and outer segment thickness remained unchanged in the fellow eyes. Conclusion: Eyes with MEWDS have changes in the photoreceptor microstructures. The change in the IS + OS thickness during the natural recovery course might be due to an increase in the OS length. [ABSTRACT FROM AUTHOR]

Objective: The purpose of this study was to investigate frequent disease-causing gene mutations in autosomal recessive retinitis pigmentosa (arRP) in the Japanese population. Methods: In total, 99 Japanese patients with non-syndromic and unrelated arRP or sporadic RP (spRP) were recruited in this study and ophthalmic examinations were conducted for the diagnosis of RP. Among these patients, whole exome sequencing analysis of 30 RP patients and direct sequencing screening of all CNGA1 exons of the other 69 RP patients were performed. Results: Whole exome sequencing of 30 arRP/spRP patients identified disease-causing gene mutations of CNGA1 (four patients), EYS (three patients) and SAG (one patient) in eight patients and potential disease-causing gene variants of USH2A (two patients), EYS (one patient), TULP1 (one patient) and C2orf71 (one patient) in five patients. Screening of an additional 69 arRP/spRP patients for the CNGA1 gene mutation revealed one patient with a homozygous mutation. Conclusions: This is the first identification of CNGA1 mutations in arRP Japanese patients. The frequency of CNGA1 gene mutation was 5.1% (5/99 patients). CNGA1 mutations are one of the most frequent arRP-causing mutations in Japanese patients. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the usefulness of a liquid crystal display (LCD) with higher driving frequency and shorter response time (2 ms) as a visual stimulator to elicit pattern reversal visually evoked potentials (p-VEPs). Method: p-VEPs were recorded from 12 eyes of 12 healthy volunteers (28.3 ± 9 years). The p-VEPs elicited by a conventional cathode ray tube (CRT) screen were compared to those elicited by a high-speed LCD screen (2-ms LCD, GD245HQbid, Acer, Taipei, Taiwan). The luminance changes of each monitor were measured with a photodiode. Results: During the reversal phase the luminance of the 2-ms LCD screen with 97% contrast was transiently reduced, which can elicit an electroretinogram (ERG) and therefore a flash VEP. The 2-ms LCD with 81% contrast checkerboard had a minimal luminance reduction during the reversal phase, and therefore no ERGs were elicited. No significant differences in the amplitude of P100 and the implicit times of N75 and P100 were observed in the p-VEPs elicited by a CRT or the 2-ms LCD screens as stimulators. Conclusion: The luminance change can elicit flash VEPs, and this artifact can be minimized by using a 2-ms LCD screen with reduced contrast of the checkerboard stimulus. © 2014 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

. Purpose: To try to recreate the images reported by patients during vitreous surgery in a model eye. Methods: A fluid-filled model eye with a posterior frosted translucent surface which corresponded to the retina was used. Three holes were made in the model eye through which an endoillumination pipe and intraocular forceps could be inserted. A thin plastic sheet simulating an epiretinal membrane and an intraocular lens (IOL) simulating a dislocated IOL were placed on the retina. The images falling on the posterior surface were photographed from the rear. The images seen through the surgical microscope were also recorded. Results: The images from the rear were mirror images of those seen through the surgical microscope. Intraocular instruments were seen as black shafts from the rear. When the plastic sheet was picked up, the tip of the forceps was seen more sharply on the posterior surface. The images of the dislocated IOL from the posterior were similar to that seen through the surgical microscope, including the yellow optics and blue haptics. Conclusion: Intravitreal objects can form images on the surface of a model eye. Objects located closer to the surface are seen more sharply, and the colour of the objects can be identified. [ABSTRACT FROM AUTHOR]

Purpose: The cathode-ray tube (CRT) screen has recently been replaced by liquid crystal display (LCD) screens as visual stimulators for pattern-reversal visually evoked potentials (p-VEPs). The aim of the study was to evaluate the usefulness of LCD screen to elicit p-VEPs. Methods: The waveforms of the p-VEPs elicited by a LCD panel were compared with those elicited by a conventional CRT screen. The changes in the luminance of each screen were measured with a photodiode, and the mean luminance change was measured with a luminance meter. VEPs and electroretinograms (ERGs) were also recorded when the monitor was covered by a diffuser. Results: The p-VEPs elicited by the LCD consisted of the N75 and P100 components of the conventional VEPs and had good reproducibility. The average latency of these components was significantly delayed by 9.8 ms for N75 and 10.2 ms for P100, and the N75-P100 amplitude was significantly larger than the conventional p-VEP elicited by the CRT screen. During the reversal phase, especially from black-to-white, the luminance of the LCD screen was transiently reduced, and it elicited a flash VEP and ERG. A reduction in the contrast of the checks minimized the transient change in the luminance, and the VEP waveform was more similar to that elicited by the CRT screen. Conclusions: The results suggest that when an LCD monitor is used as an alternative visual stimulator to elicit p-VEPs, the delay in the luminance change and the flash effect needs to be taken into account. [ABSTRACT FROM AUTHOR]

Purpose: To report an improvement of the visual acuity after transcorneal electrical stimulation (TES) in a case of Best vitelliform macular dystrophy (BVMD). Patient and methods: A 26-year-old woman diagnosed with BVMD presented with reduced vision. Her best corrected visual acuity (BCVA) was reduced to 20/200 in the right eye, and TES was performed once a month for two sessions. The current of the biphasic pulses (anodic first; duration, 10 msec; frequency, 20 Hz) was delivered using a DTL-electrode, and the duration of the TES was 30 min. Results: The BCVA in the right eye slowly improved after the TES, and 6 months later the BCVA was 20/25. The results of Humphrey visual field tests (VF) and multifocal ERGs (mfERGs) were only slightly changed. Two years later, the BCVA decreased, and it was improved again after another session of TES with the same parameters of the electrical pulses. Conclusion: The improvement of the visual acuity in our case of BVMD indicates that TES should be tried in other cases of retinal dystrophy. Further clinical and laboratory studies on TES are needed. [ABSTRACT FROM AUTHOR]

Background The aim was to determine the low luminance visual acuity in eyes with central serous chorioretinopathy. Methods Seven eyes of seven patients with central serous chorioretinopathy and six eyes of six age-matched normal volunteers were examined. Low luminance visual acuity charts were created by an Apple Power Mac G5 computer and displayed on a cathode ray tube monitor ( SONY GDM- F500). The background luminance was set at six different levels from 78.20 cd/ m2 to 0.37 cd/ m2. The visual acuities of the eyes with central serous chorioretinopathy at each of the six luminance levels were compared to those from their fellow eyes and to normal eyes. Results The mean visual acuities varied from 0.13, 0.23, 0.29, 0.42, 0.62 to 0.70 logMAR units as luminance varied from high to low. At the lowest luminance (0.37 cd/ m2), five of the seven eyes could not read any character. The mean visual acuities of the fellow eyes at the same luminance levels were 0.03, 0.06, 0.11, 0.20, 0.27 and 0.45 logMAR units and those of the normal volunteers were 0, 0.03, 0.08, 0.14, 0.23 and 0.38 logMAR units, respectively. The visual acuities of the eyes with central serous chorioretinopathy were significantly poorer than those of the normal eyes at all luminance levels except 0.37 cd/ m2 (p < 0.05 for all). Conclusions Although the eyes from all three groups had 0 logMAR units visual acuity under standard testing condition, the visual acuity of the eyes with central serous chorioretinopathy were significantly worse at low luminance levels. The low luminance visual acuity may provide information on the visual disturbances reported by central serous chorioretinopathy patients with 0 logMAR units visual acuity. [ABSTRACT FROM AUTHOR]