Kinetic Perimetry Research Articles

Delineating the Clinical Phenotype of Patients With the c.629C>G, p.Pro210Arg Mutation in Peripherin-2.

PurposeMore than 200 different mutations in peripherin-2 (PRPH2) are associated with multiple subtypes of inherited retinal diseases (IRDs), including retinitis pigmentosa and cone or macular diseases. Our goal was to understand how the poorly characterized PRPH2 mutation p.Pro210Arg (P210R) affects visual function and retinal structure as well as gain insight into the mechanism driving the clinical pathology.MethodsEleven patients had clinical assessments including best-corrected visual acuity (BCVA), full field and multifocal electroretinography (ERG), static (spot size V) and kinetic perimetry (Octopus 900), and dark-adapted chromatic (DAC; Medmont; spot size V) perimetry. Images were acquired with the Optos ultra-wide field camera and spectral-domain optical coherence tomography (SD-OCT). Molecular characteristics of the P210R mutant protein were evaluated in vitro.ResultsPatients with the P210R mutation had BCVA (Snellen) ranging from 20/15 to 20/80. Perimetry showed a reduction in sensitivity, while ERG findings suggested that cone function was more impaired than rod function. Scotomas were identified corresponding to atrophic retinal lesions. Imaging revealed heterogeneous outer retinal changes such as hyperfluorescent flecks, hypo-autofluorescence (AF) regions of atrophy, and thinning of the photoreceptor layer on SD-OCT. In vitro findings suggested that P210R-Prph2 retains the ability to interact with binding partner Rom1 but abnormally accumulates in the endoplasmic reticulum (ER), suggesting the protein does not fold properly.ConclusionsRod and cone sensitivities were decreased in subjects with the P210R mutation in PRPH2. There was scotomatous vision loss that occurred within the macula, likely due to atrophy that occurs after drusen have formed and have begun to resolve. This suggests that although rod and cone photoreceptors are dependent on PRPH2, preventing blindness in this specific subgroup of patients could involve therapeutics that impede the formation or lifecycle of drusen.

Characterizing Visual Fields in RPGR Related Retinitis Pigmentosa Using Octopus Static-Automated Perimetry.

PurposePeripheral visual fields have not been as well defined by static automated perimetry as kinetic perimetry in RPGR-related retinitis pigmentosa. This study explores the pattern and sensitivities of peripheral visual fields, which may provide an important end point when assessing interventional clinical trials.MethodsA retrospective observational cross-sectional study of 10 genetically confirmed RPGR subjects was performed. Visual fields were obtained using the Octopus 900 perimeter. Interocular symmetry and repeatability were quantified. Visual fields were subdivided into central and peripheral subfields for analysis.ResultsMean patient age was 32 years old (20 to 49 years old). Average mean sensitivity was 7 dB (SD = 3.67 dB) and 6.8 dB (SD = 3.4 dB) for the right and left eyes, respectively, demonstrating interocular symmetry. Coefficient of repeatability for overall mean sensitivity: <2 dB. Nine out of 10 subjects had a preserved inferotemporal subfield, whose mean sensitivity was highly correlated to the central field (r2 = 0.78, P = 0.002 and r2 = 0.72, P = 0.002 for the right and left eyes, respectively). Within the central field, sensitivities were greater in the temporal than the nasal half (t-test, P = 0.01 and P = 0.03 for the right and left eyes, respectively).ConclusionsOctopus static-automated perimeter demonstrates good repeatability. Interocular symmetry permits use of the noninterventional eye as an internal control. In this cohort, the inferotemporal and central visual fields are preserved into later disease stages likely mapping to populations of surviving cones.Translational RelevanceA consistently preserved inferotemporal island of vision highly correlated to that of the central visual field may have significance as a possible future therapeutic site.

Quantitative and functional visual field outcomes after endoscopic trans-sphenoidal pituitary adenectomy.

Quantitative data on visual outcomes after trans-sphenoidal surgery is lacking in the literature. This study aims to address this by quantitatively assessing visual field outcomes after endoscopic trans-sphenoidal pituitary adenectomy using the capabilities of modern semi-automated kinetic perimetry. Visual field area (deg2) calculated on perimetry performed before and after surgery was statistically analysed. Functional improvement was assessed against UK driving standards. Sixty-four patients (128 eyes) were analysed (May 2016-Nov 2019). I4e and I3e isopter area significantly increased after surgery (p < 0.0001). Of eyes with pre-operative deficits: 80.7% improved and 7.9% worsened; the median amount of improvement was 60% (IQR 6-246%). Median increase in I4e isopter was 2213deg2 (IQR 595-4271deg2) and in I3e isopter 1034 deg2 (IQR 180-2001 deg2). Thirteen out of fifteen (87%) patients with III4e data regained driving eligibility after surgery. Age and extent of resection (EOR) did not correlate with visual improvement. Better pre-operative visual field area correlated with a better post-operative area (p < 0.0001). However, the rate of improvement in the visual field area increased with poorer pre-operative vision (p < 0.0001). A median visual field improvement of 60% may be expected in over 80% of patients. Functionally, a significant proportion of patients can expect to regain driving eligibility. EOR did not impact on visual recovery. When the primary goal of surgery is alleviating visual impairment, optic apparatus decompression without the aim for gross total resection appears a valid strategy. Patients with the worst pre-operative visual field often experience the greatest improvement, and therefore, poor pre-operative vision alone should not preclude surgical intervention.

Rapid Campimetry-A Novel Screening Method for Glaucoma Diagnosis.

One of the most important functions of the retina—the enabling of perception of fast movements—is largely suppressed in standard automated perimetry (SAP) and kinetic perimetry (Goldmann) due to slow motion and low contrast between test points and environment. Rapid campimetry integrates fast motion (=10°/4.7 s at 40 cm patient–monitor distance) and high contrast into the visual field (VF) examination in order to facilitate the detection of absolute scotomas. A bright test point moves on a dark background through the central 10° VF. Depending on the distance to the fixation point, the test point automatically changes diameter (≈0.16° to ≈0.39°). This method was compared to SAP (10-2 program) for six subjects with glaucoma. Rapid campimetry proved to be comparable and possibly better than 10-2 SAP in identifying macular arcuate scotomas. In four subjects, rapid campimetry detected a narrow arcuate absolute scotoma corresponding to the nerve fiber course, which was not identified as such with SAP. Rapid campimetry promises a fast screening method for the detection of absolute scotomas in the central 10° visual field, with a potential for cloud technologies and telemedical applications. Our proof-of-concept study motivates systematic testing of this novel method in a larger cohort.

Full-field sensitivity threshold and the relation to the oxygen metabolic retinal function in retinitis pigmentosa.

The aim of our study was to evaluate retinal function with white light dark-adapted full-field sensitivity threshold (FST) and find possible correlations with metabolic function measured with retinal oximetry (RO) in patients with retinitis pigmentosa (RP). In this prospective observational study (BASEC 2020-00,122), FST and RO measurements were performed on 66 RP eyes (33 subjects, 12♀ 21♂) aged between 18 and 80years (mean 43.2years); all eyes were graded for disease severity. Main outcome parameters were white FST thresholds using the Diagnosys Espion system with the ColorDomeTM LED full-field stimulator (Diagnosys LLC, Lowell, MA) as well as the main RO parameters: the mean arterial (A-SO2; %), venular (V-SO2; %) oxygen saturation, their difference (A-V SO2; %), and the corresponding mean diameters of the peripapillary retinal arterioles (D-A; μm) and venules (D-V; μm) recorded with the oxygen saturation tool of the Retinal Vessel Analyser (RVA; IMEDOS Systems UG, Jena, Germany). In addition, semi-automated kinetic perimetry (V4e, III4e, I4e, III3e isopters, Octopus 900®, Haag-Streit AG Bern, Switzerland) was performed and included in the linear mixed-effects models analysis calculated with SPSS®. Neither the oxygen saturation parameters (p > 0.21) nor the D-A and D-V (p > 0.13) showed significant correlations with the FST. However, when compared systematically with the visual field (VF) areas of the different isopters, RO parameters V-SO2 (p = 0.024) and A-V SO2 (p < 0.02) showed significant correlations. Furthermore, both V-SO2 and A-V SO2 showed gradual changes with more pronounced impairment in oxygen metabolic function in advanced stages of RP when analyzed in subgroups of disease severity grades. In contrast to standardized VF parameters, white dark-adapted FST appears not to correlate with retinal oxygen metabolic function measured with RO in patients with RP, suggesting that the two examinations may capture unrelated aspects of the retinal pathological process. However, RO showed a significant association with standardized VF testing parameters and may, therefore, offer an alternative outcome measure for interventional trials.

Three‐Year Safety Results of SAR422459 (EIAV‐ABCA4) Gene Therapy in Patients With ABCA4‐Associated Stargardt Disease: An Open‐Label Dose‐Escalation Phase I/IIa Clinical Trial, Cohorts 1‐5

To report on the safety of the first 5 cohorts of a gene therapy trial using recombinant equine infectious anemia virus expressing ABCA4 (EIAV-ABCA4) in adults with Stargardt dystrophy due to mutations in ABCA4. Nonrandomized multicenter phase I/IIa clinical trial. Patients received a subretinal injection of EIAVABCA4 in the worse-seeing eye at 3 dose levels and were followed for 3 years after treatment. The primary end point was ocular and systemic adverse events. The secondary end points were best-corrected visual acuity, static perimetry, kinetic perimetry, total field hill of vision, full field electroretinogram, multifocal ERG, color fundus photography, short-wavelength fundus autofluorescence, and spectral domain optical coherence tomography. The subretinal injections were well tolerated by all 22 patients across 3 dose levels. There was 1 case of a treatment-related ophthalmic serious adverse event in the form of chronic ocular hypertension. The most common adverse events were associated with the surgical procedure. In 1 patient treated with the highest dose, there was a significant decline in the number of macular flecks as compared with the untreated eye. However, in 6 patients, hypoautofluorescent changes were worse in the treated eye than in the untreated eye. Of these, 1 patient had retinal pigment epithelium atrophy that was characteristic of tissue damage likely associated with bleb induction. No patients had any clinically significant changes in best-corrected visual acuity, static perimetry, kinetic perimetry, total field hill of vision, full field electroretinogram, or multifocal ERG attributable to the treatment. Subretinal treatment with EIAV-ABCA4 was well tolerated with only 1 case of ocular hypertension. No clinically significant changes in visual function tests were found to be attributable to the treatment. However, 27% of treated eyes showed exacerbation of retinal pigment epithelium atrophy on fundus autofluorescence. There was a significant reduction in macular flecks in 1 treated eye from the highest dose cohort. Additional follow-up and continued investigation in more patients will be required to fully characterize the safety and efficacy of EIAV-ABCA4.

Comparative Evaluation of Two SD-OCT Macular Parameters (GCC, GCL) and RNFL in Chiasmal Compression.

PurposeTo evaluate the relationship between different macular thickness parameters analyzed by SD-OCT and the central visual field (VF) evaluated with automated kinetic perimetry in a cohort of patients with pituitary tumors.MethodsData from patients with pituitary adenoma treated at Reims University Hospital between October 1st, 2017, and May 31st, 2018 were collected. All patients underwent an automated kinetic perimetry and a SD-OCT to map the ganglion cell complex (GCC), the ganglion cell layer (GCL) thickness and the retinal nerve fiber layer (RNFL) using devices from two different manufacturers. Univariate and multivariate analysis were used to evaluate the correlation between the area of central VF in square degrees (deg2) and the SD-OCT parameters (μm).ResultsEighty-eight eyes were included in the analysis. All the thickness parameters measured in SD-OCT decreased with the visual field alteration. The best correlation was observed between superior thickness parameters (GCC, GCL) and the inferior central visual field. The most pertinent predictive factors for visual field loss were the inferior central GCL and the nasal RNFL (both AUC=0.775) with a sensitivity respectively of 86% and 70%.ConclusionThis study suggests that both GCC, GCL thickness parameters could be reliable predictors of central visual field impairment in patients with pituitary tumors. There was no significative difference between both devices.

Three-Year Changes in Visual Function in the Placebo Group of a Randomized Double-Blind International Multicenter Safety Study: Analysis of Electroretinography, Perimetry, Color Vision, and Visual Acuity in Individuals With Chronic Stable Angina Pectoris.

PurposeTo determine whether significant deteriorations in objective (electroretinography [ERG]) and subjective (standard automated and semi-automated kinetic perimetry; color discrimination; and best-corrected visual acuity) tests of visual function, potentially attributable to aging, occurred in the group randomized to placebo of a 3-year prospective multicenter ocular safety study of ivabradine for chronic stable angina pectoris.MethodsThe multicenter trial was conducted at 11 international ophthalmic centers. Changes in visual function between baseline and month 36 were analyzed by means of a two-tailed Wilcoxon signed-rank test, based on the Hodges and Lehman estimator of the median difference, with the 95% confidence intervals derived by Walsh averages.ResultsThirty-eight participants from the placebo group completed the study (mean [SD], age, 62.7 [8.1] years). The group exhibited in each eye small, but statistically significant, reductions in the amplitudes of the dark-adapted (DA) ERG 3.0 a-wave, and light-adapted (LA) 3.0 b-wave, as well as increases in peak time for the DA 0.01 b-wave, DA 3.0 a-wave, LA 3.0 b-wave, and LA 3.0 30-Hz flicker response and in the isopter area I3e of the visual field.ConclusionsStatistically significant deteriorations occurred in visual function over a period of 3 years, potentially attributable to age, within a group of individuals with chronic stable angina pectoris and unremarkable ophthalmological findings other than those attributable to age.Translational RelevanceA longitudinal correction factor for age-related change in visual function may be useful in future trials to determine whether an observed deterioration in visual function is related to intervention or to aging.

Objective quantification of the impact of blepharoplasty on the superior visual field.

BackgroundBlepharoplasty has both aesthetic and functional benefits in patients with pseudoptosis; however, previous studies could not demonstrate its beneficial effects quantitatively and objectively. The authors objectively analyzed the visual field before and after surgery and investigated whether measurements of the visual field can be applied as a suitable predictor of surgical outcomes.MethodsIn total, 18 eyelids in nine patients with pseudoptosis who had undergone simple skin excision blepharoplasty were evaluated prospectively from February to May 2016. The visual fields were analyzed preoperatively and 3 months postoperatively using the Goldmann kinetic perimetry test. The visual field test area was assessed using Adobe Photoshop.ResultsBlepharoplasty had an average 4.99-fold beneficial effect on the superior visual field. In particular, more improvement was seen in the superior temporal quadrant than in the nasal quadrant. No correlation was found between the preoperative margin-to-reflex distance 1 (MRD1) and the surgical outcome (P=0.119). However, there was a strong correlation between the preoperative superior visual field and the surgical outcome (P=0.001).ConclusionsUsing the Goldmann kinetic perimetry test, we objectively and quantitatively proved the beneficial effect of blepharoplasty on patients with pseudoptosis. Furthermore, we demonstrated that the preoperative visual field is a better preoperative surgical outcome predictive factor than the preoperative MRD1.

Dissociation between red and white stimulus perception: A perimetric quantification of protanopic color vision deficiencies.

SignificanceHorizontal visual field extension was assessed for red and white stimuli in subjects with protanopia using semi-automated kinetic perimetry. In contrast to a conventional anomaloscope, the “red/white dissociation ratio” (RWR) allows to describe protanopia numerically. For the majority of subjects with protanopia a restriction for faint red stimuli was found.PurposeComparing the horizontal visual field extensions for red and white stimuli in subjects with protanopia and those with normal trichromacy and assessing the related intra-subject intra-session repeatability.MethodsThe subjects were divided into groups with protanopia and with normal trichromacy, based on color vision testing (HMC anomaloscope, Oculus, Wetzlar/FRG). Two stimulus characteristics, III4e and III1e, according to the Goldmann-classification, were presented with semi-automated kinetic perimetry (Octopus 900 perimeter, Haag-Streit, Köniz/CH). They moved along the horizontal meridian, with an angular velocity of 3°/s towards the visual field center, starting from either the temporal or nasal periphery. If necessary, a 20° nasal fixation point offset was chosen to capture the temporal periphery of the visual field. For each condition the red/white dissociation ratio (RWR); Pat Appl. DPMA DRN 43200082D) between the extent of the isopter for red (RG610, Schott, Mainz/ FRG) and white stimuli along the horizontal meridian was determined.ResultsAll data are listed as median/interquartile range: Five males with protanopia (age 22.1/4.5 years) and six males with normal trichromacy (control group, age 30.5/15.2 years) were enrolled. The RWR is listed for the right eye, as no clinically relevant difference between right and left eye occurred. Protanopes’ RWR for mark III4e (in brackets: control group) was 0.941/0.013 (0.977/0.019) and for mark III1e 0.496/0.062 (0.805/0.051), respectively.ConclusionsIn this exploratory “proof-of-concept study” red/white dissociation ratio perimetry is introduced as a novel technique aiming at assessing and quantifying the severity of protanopia. Further effort is needed to understand the magnitude of the observed red-/white dissociation and to extend this methodology to a wider age range of the sample and to anomalous trichromacies (protanomalia) with varying magnitude.

Influence of aging on the color visual field in humans: A cross-sectional study.

Age-related narrowing of the visual field is observed in the elderly, which leads to reduced cognitive and psychomotor functions. The aim of the present cross-sectional study was to determine the influence of aging on the visual field for color vision in humans, with respect to angular thresholds for object detection and color detection.The subjects were divided into the elderly group (mean 76.1-year-old [70-89]) and the control group (25.2 [18-47]). Visual fields for different colors (blue, green, yellow, and red) were measured by manual kinetic perimetry and evaluated in terms of 2 measures of visual-field width: angular thresholds for object detection and those for color detection.While angular thresholds for object detection were significantly wider than those for color detection in the control group (P < .001), there was no difference in the elderly group (P = .06). Moreover, angular thresholds for object detection were significantly wider in the control group than in the elderly group (P = .019), but angular thresholds for color detection were not significantly different between the 2 groups (P = .903).The observed age-related changes in angular thresholds for object detection in color vision may reflect an age-related reduction in rod function. Stable cone function might explain the preserved angular thresholds for color detection in the elderly.

Amiodarone-associated bilateral optic neuropathy: a case report

Purpose: Amiodarone is a commonly prescribed anti-arrhythmic drug; however, its use is limited by many adverse effects. Amiodarone causes lung, thyroid gland, skin, central nervous system, liver, and eye disorders. Corneal microdeposits are the most common ocular adverse effects, while more serious tissue damage such as optic neuropathy can also occur. The aim of this report was to describe a case of a toxic optic neuropathy, which is one of the adverse effects of amiodarone treatment.&#x0D; Case report: This is a case report of a 51-year-old patient hospitalized at the Department of Ophthalmology University Medical Center Maribor because of blurred vision, first in his right eye and later in his left eye. Three months before admission, the patient began treatment with amiodarone because of arrhythmia after acute pericarditis. The patient underwent a complete ophthalmic examination and other tests for all known optic neuropathy causes. The patient initially presented with blurred vision in his right eye, and also in his left eye after 7 days. Except for corneal micro-deposits and optic disc edema, the results of an ophthalmic exam were normal. Static and kinetic visual field examinations showed bilateral visual field defects. Fluorescein angiography showed bilateral fluorescein dye leakage from the optic disc. Amiodarone treatment was terminated on the 7th day of hospitalization, and the patient reported improvement of vision in subsequent weeks. The edema in his left optic disc resorbed at 3.5 weeks and in his right optic disc at 5 weeks after termination of amiodarone treatment. Kinetic and static perimetry showed improvement in the visual field 10 weeks after the termination of amiodarone treatment.&#x0D; Conclusions: Toxic optic neuropathy had a similar clinical course as non-arteritic anterior ischemic optic neuropathy. Prompt diagnosis is crucial because treatment with amiodarone can prevent irreversible visual impairment. Because of the frequent clinical use of amiodarone, and despite the low incidence of toxic optic neuropathy, regular annual follow-up examinations should be performed.

Trends in Utilization of Visual Field Tests for Glaucoma Patients: A Nationwide Study Using the Korean Health Insurance Review and Assessment Database.

PurposeTo analyze 10-year trends in utilization of visual field tests for adult glaucoma or glaucoma-suspect patients using the Korean Health Insurance Review and Assessment data.MethodsHealth claims for the years 2010 to 2019, as recorded via Korea’s Health Insurance Review and Assessment service, were accessed. We identified glaucoma patients using the glaucoma diagnostic codes H40 (glaucoma) and H42 (glaucoma in other diseases classified elsewhere). For verification of the glaucoma diagnosis, information on any antiglaucoma medication prescriptions and ocular surgery history also was obtained. Visual field testing data was isolated using procedural codes E6690 (kinetic perimetry) and E6691 (standard automated perimetry [SAP]) performed in tertiary hospitals. Any changes in visual field test utilization were identified using regression trend analysis.ResultsFrom 2010 to 2019, the total number of SAP procedures performed in tertiary hospitals for either glaucoma or glaucoma-suspect patients increased gradually from 93,459 to 216,433. With regard to kinetic perimetry examinations, the total number decreased gradually from 6,364 to 3,792. The yearly average SAP number per patient showed a slight increase, from 1.168 to 1.248 (β = 0.008, R2 = 0.669, p = 0.004). Meanwhile, the yearly average number of kinetic perimeter examinations per patient showed a significant decrease, from 1.093 to 0.940 (β = −0.013, R2 = 0.580, p = 0.010).ConclusionsBetween 2010 and 2019, the yearly average number of SAP procedures performed per glaucoma or glaucoma-suspect patient increased in Korea. Meanwhile, the yearly average number of kinetic perimetry examinations per patient significantly decreased.

Periphery kinetic perimetry: clinically feasible to complement central static perimetry

BackgroundExisting evidence suggests that visual field defect in eyes with glaucoma significantly varies between individuals. The following study compared the central visual field defects with the peripheral visual field defects in patients with suspect glaucoma and primary open-angle glaucoma (POAG) and investigated whether using the central visual field test alone could result in loss of clinically valuable information.MethodsIn this prospective observational study, 167 eyes from 89 patients with suspect glaucoma or POAG were first examined with static automated perimetry (SAP), followed by a peripheral visual field test on Octopus 900 perimeter (Haag-Streit, Koeniz, Switzerland). The peripheral visual field test was performed by “Auto Kinetic Perimetry” program, in which Goldmann III4e stimuli randomly moved along 16 vectors at a constant angular velocity of 5 deg/s.ResultsGlaucomatous peripheral visual field defects were seen in 18% of the eyes with a normal central visual field. In addition, 86% of glaucoma patients with moderate-to-severe central visual field defects had corresponding peripheral visual field defects in the form of localized or diffuse depression of the isopters. Furthermore, a moderate correlation was found between the central and peripheral visual fields. The median test duration was 71 s for the peripheral test and 803 s for the central test (p < 0.001).ConclusionsOur study demonstrated the diversity of glaucomatous visual field defects, as well as the possibility of losing the clinically valuable information due to focusing on the central visual field test alone. The peripheral kinetic perimetry is clinically feasible to complement the central static perimetry for a comprehensive assessment of visual function in glaucoma patients.

Comparison of Visual Outcomes and Patient Satisfaction Following Cataract Surgery with Two Monofocal Intraocular Lenses: Clareon® vs AcrySof® IQ Monofocal

Aim: This study aimed to compare the performance of two monofocal Intraocular Lenses (IOL) platforms. Background: The Clareon® Intraocular Lens (IOL) is a relatively new monofocal lens platform designed to improve postoperative results compared to other monofocal platforms. Objective: This study aimed to assess and compare the visual and refractive outcomes, and incidence of YAG capsulotomy of the Clareon® IOL and a standard non-preloaded AcrySof® monofocal IOL following contralateral implantation in patients undergoing cataract surgery. Methods: A total of 20 patients (40 eyes; 12 female, average age 72.8±6.4 years) who had undergone contralateral implantation of an AcrySof® IQ monofocal lens (SN60WF or SN6AT; Alcon; Texas, USA) and a Clareon®monofocal lens (CNAOT0; Alcon; Texas, USA) were selected. Uncorrected Distance Visual Acuity (UDVA), Contrast Sensitivity (CS), kinetic perimetry, and refraction were measured 1 month following the second surgery and subjective vision was measured 6 months following the second surgery using a quality-of-life questionnaire. Results: There was no difference in postoperative UDVA (P=0.94), CS (P&gt;0.05), or refraction (P=0.64) between eyes that received the Clareon® and AcrySof® IQ lenses. Clareon® eyes had a higher incidence of glare/haloes and positive dysphotopsia while AcrySof® IQ eyes had a higher incidence of negative dysphotopsia. Patient satisfaction was similar between the groups (P=0.86), although 25% of patients reported more clarity in the eye that received the Clareon® lens. The incidence of posterior capsular opacification was low for both groups. Conclusion: Clareon® and AcrySof® IQ lenses perform similarly, providing good refractive, visual, and subjective outcomes. Clareon® is available as a preloaded lens option and may reduce PCO and the need for Nd: YAG capsulotomy.

CRB1-Associated Retinal Dystrophies: A Prospective Natural History Study in Anticipation of Future Clinical Trials

To investigate the natural disease course of retinal dystrophies associated with crumbs cell polarity complex component 1 (CRB1) and identify clinical end points for future clinical trials. Single-center, prospective case series. An investigator-initiated nationwide collaborative study that included 22 patients with CRB1-associated retinal dystrophies. Patients underwent ophthalmic assessment at baseline and 2 years after baseline. Clinical examination included best-corrected visual acuity (BCVA) using Early Treatment Diabetic Retinopathy Study charts, Goldmann kinetic perimetry (V4e isopter seeing retinal areas), microperimetry, full-field electroretinography, full-field stimulus threshold (FST), fundus photography, spectral-domain optical coherence tomography, and fundus autofluorescence imaging. Based on genetic, clinical, and electrophysiological data, patients were diagnosed with retinitis pigmentosa (19 [86%]), cone-rod dystrophy (2 [9%]), or isolated macular dystrophy (1 [5%]). Analysis of the entire cohort at 2 years showed no significant changes in BCVA (P=.069) or V4e isopter seeing retinal areas (P=.616), although signs of clinical progression were present in individual patients. Macular sensitivity measured on microperimetry revealed a significant reduction at the 2-year follow-up (P < .001). FST responses were measurable in patients with nonrecordable electroretinograms. On average, FST responses remained stable during follow-up. In CRB1-associated retinal dystrophies, visual acuity and visual field measures remain relatively stable over the course of 2 years. Microperimetry showed a significant decrease in retinal sensitivity during follow-up and may be a more sensitive progression marker. Retinal sensitivity on microperimetry may serve as a functional clinical end point in future human treatment trials for CRB1-associated retinal dystrophies.

Аutosomal Dominant Oculodental-Digital Dysplasia with Mutation in Gene GJA1 (Clinical Case)

The purpose: to describe clinical cases of oculodental-digital dysplasia (ODDD, OMIM #164200) with mutation in GJA1 (OMIM 121014) with molecular genetic verification of the diagnosis.Methods. The article describes the clinical case of oculodental-digital dysplasia in a 51 years old patient. Patient underwent full ophthalmic examination including autorefractometry, visual acuity testing with full correction, tonometry, biomicroscopy, fundus examination and photo as well as kinetic perimetry, autofluorescence and optical coherence tomography (OCT) of macula and optic disk were performed. Electrophysiological examination included Visual Evoked Potentials (VEP) to flash and pattern stimulation, ISCEV standard electroretinograms (ERG) and macular ERG. For the verification of the diagnosis and pathologic gene molecular genetic examination was performed with family anamnesis previously attained.Results. The patient was complaining the deterioration of vision, hearing loss and the sense of smell. Visual deterioration was associated with nyctalopia. Natural history revealed glaucoma 2а which was diagnosed when he was 48 years old. Best corrected visual acuity was 1,0. Peripheral visual field defects were revealed bilaterally. High visual acuity correlated with normal foveal structure on OCTs the retinal nerve fiber layer (RNFL) was thinner than normal in temporal half; deep excavation was visualized in both eyes. Normal MERG and bilateral decrease of scotopic, maximal full-field ERG was recorded which correlated with nyctalopia, as well as subnormal photopic responses indicating cone system involvement. The genetics revealed characteristic features of the face: a small nose with hypoplasia of the wings of the nose, unfolded nostrils and a wide bridge of the nose (pseudohypertelorism). On right-wing the ear sink was detected 2 antitraguses. Changes fingers upper extremities — operated syndactyly IV and V on the background of brachydactyly of the fingers. On the legs on both sides — syndactyly III–IV. 10 years the sense of smell has been dereriorated. In the study of DNA in proband in direct Sanger sequencing of all exons 1–2 and regions of exon-intron compounds of gene GJA1, was found the pathogenic variant in second exon c.412G&gt;A (p.Gly138Ser) in heterozygous state. Was established autosomal dominant type of disease.Conclusion. We are the first to describe rod-cone dystrophy in oculodental-digital dysplasia.

Electroretinogram and Visual Field Correlation in Birdshot Chorioretinopathy.

The aim of this study was to assess possible correlation between the visual field and electroretinogram (ERG) parameters in birdshot chorioretinopathy (BCR). Retrospective cross-sectional study. This single-center study included 21 eyes of 11 patients with BCR with prior ERG and Goldmann kinetic perimetry. Generalized estimating equation was used for statistical analysis, and isopters V4e and I4e were modeled as separate outcomes with each parameter of ERG as the main independent variable. The main outcome measure was presence or absence of correlation between the Goldmann isopters and ERG parameters including the amplitudes and implicit times of dark adapted (DA) and light adapted (LA) a-waves and b-waves (DA 0.01, DA 3.0, DA 10.0, and LA 3.0) and LA 30 Hz. There was a strong positive correlation between the size of isopter V4e and all the scotopic and photopic standard ERG amplitudes [P < 0.0001 for all, except LA 3.0 a-wave (P = 0.0019) and LA 30 Hz (P = 0.0026)]. For the implicit times, only DA 3.0 a-wave (P = 0.007) and LA 30 Hz (P < 0.0001) showed significant negative correlations. There were no correlations between the size of isopter I4e and any ERG amplitudes; however, 3 implicit times showed some negative correlation: DA 3.0 a-wave (P < 0.0001), DA 10.0 b-wave (P = 0.0251), and LA 3.0 b-wave (P < 0.0001). In this study, the largest isopter of Glodmann visual field showed a strong positive correlation with all standard ERG amplitudes in BCR. This novel finding underscores the role of ERG as an objective visual function test in monitoring the disease activity.

Kinetic and static perimetry after 16 years and additional OCT-A analysis in eyes with long-lasting optic disc drusen.

The aim of the study is to evaluate the progression of visual field (VF) defects over 16 years of observation and to assess abnormalities in vessels and retinal nerve fibre layer (RNFL) thickness in patients with optic disc drusen (ODD). Both static automated perimetry (SAP) and semi-automated kinetic perimetry (SKP) were performed in 16 eyes of 8 patients (mean age 54 years) with ODD among 26 eyes of 13 patients examined 16 years before. The area of I2e, I4e, III4e, and V4e isopters was measured in deg2. The MD and PSD parameters were estimated using SAP. Optical coherence tomography angiography (OCT-A) was additionally performed in 16 ODD eyes and 16 eyes of 8 healthy subjects to estimate the RNFL thickness and vessel density of the optic nerve disc and the macula. The differences in all isopter areas of SKP and SAP parameters after 16 years were not significant. The analysis of OCT-A showed a significant reduction of the vessel density and RNFL of the peripapillary area in each segment in patients with ODD, compared with the control group. The highest reduction of RNFL was observed in the superior segment of the optic disc area (92.56μm vs 126.63μm) also the macular thickness was decreased in ODD patients, compared with the control group. In the macula, there was a significant vascular defect in the whole superficial layer and in the parafoveal deep layer. A strong significant correlation of the parafoveal deep plexus with MD and PSD parameters was detected. In conclusion, VF loss due to ODD after 16 years of the follow-up was not significant both in SKP and SAP. ODD caused a reduced vessel density and RNFL, as well as macular thickness in OCT-A. SAP parameters were influenced by parafoveal deep plexus.

Organic visual loss measured by kinetic perimetry and retinal electrophysiology in children with functional amblyopia.

To demonstrate an organic (retinal) amblyogenic defect in functional amblyopes not responding to treatment. Twenty-four children (Mean age: 5.9 ± 1.8years; range: 4-10years) with functional amblyopia were recruited for this study. All these children underwent complete ophthalmic and orthoptic evaluation. In addition, Kinetic Goldman Visual Fields (KGVF), Spectral Domain Optical Coherence Tomography (SD-OCT), full field flash electroretinograms (ffERG) and multifocal electroretinograms (mfERG) were also performed. Ratios were subsequently derived by comparing the amplitudes obtained from the amblyopic eye (AE) to the good eye (GE) for the a- and b-waves of the ffERG, as well as for the ring analysis of the mfERG. KGVF showed a central scotoma of varying size (3°-7°) and density (absolute to relative), with increasing target size in 14/24 patients whose best post-treatment vision in the AE ranged from 20/100 to 20/40. The scotoma decreased in size and density with improving vision until a plateau of recovery was reached. The remaining 10/24 patients with a vision ≥ 20/30 showed no scotoma. SD-OCT showed no significant difference between the AE and GE. ffERG and mfERG were obtained in 18/24 patients. The ffERG AE/GE ratio was abnormal in 7 patients, 5 of which had large scotomas on KGVF. The mfERG ring 1 AE/GE ratio was significantly (p < .05) attenuated in 9/18 patients out of which 3 were no longer amblyopic. However, there was no significant difference (p > .05) in ring 1 AE/GE amplitude ratio between those who achieved 20/50-20/40 (.81 ± .26) and those with ≥ 20/25(.86 ± .25). The combined findings of central scotoma on KGVF and mfERG anomalies in patients who did not achieve optimal vision with treatment suggest an underlying organic defect impairing macular function.