Electrophysiological findings in extensive macular atrophy with pseudodrusen.

PURPOSE: To present electroretinogram findings in extensive macular atrophy with pseudodrusen (EMAP) and describe associated systemic factors. DESIGN: Retrospective case series. METHODS: Data on medical history, visual symptoms, multimodal imaging findings, and visual field were collected from the medical records of patients with extensive macular atrophy with pseudodrusen who attended a visual electrophysiology laboratory. Electrophysiological tests, including full-field electroretinogram, multifocal electroretinogram and photopic negative response, were performed. RESULTS: Eighteen patients (10 [56%] females, age 49-66 years) were included. Of these, 17 (94%) had a history of rheumatic fever in childhood and/or adolescence, 7 (39%) had cardiovascular disease, 4 (22%) had autoimmune disease, and 10 (56%) had inflammatory conditions. The primary visual complaints were nyctalopia (95%), followed by visual field loss (67%) and dyschromatopsia (67%). The key retinal findings included retinal pigmented epithelium atrophy in the macular region and subretinal drusenoid deposits. Regarding electrophysiological results, 100% of patients had abnormalities on multifocal electroretinogram, 94% displayed alterations in photopic negative response, and 78% showed changes in the full-field electroretinogram. CONCLUSIONS: In this cohort, electrophysiologic evaluation demonstrated diffuse retinal dysfunction affecting all layers of the retina in patients with EMAP. The disease is associated with immune-mediated systemic conditions, chiefly rheumatic fever.

Contribution of objectively measured grating acuity by sweep visually evoked potentials to the diagnosis of unexplained visual loss.

PURPOSE: To investigate the diagnostic contribution of grating visual acuity (GVA) measured by sweep pattern-reversal visually evoked potentials (SPRVEP) in unexplained visual loss (UVL). METHODS: This case-control study included adult patients under suspicion of UVL referred to SPRVEP and transient pattern-reversal visually evoked potentials (TPRVEP) testing. Optotype visual acuity (OVA) was measured by ETDRS 4-meter chart and GVA by SPRVEP. UVL patients were assigned into three distinctive categories, according to the presence of ocular disease, motivation, and electrophysiological evaluation, as follows: exaggerators, malingerers, and psychogenic. Healthy controls and patients with organic visual loss were also tested. Receiver operating characteristic (ROC) curve was constructed to evaluate the diagnostic performance of GVA and TPRVEP parameters. RESULTS: A total of 76 patients with UVL were analyzed: 60 (79.0%) exaggerators, 11 (14.4%) malingerers, and 5 (6.6%) psychogenic. Controls were 49 subjects evaluated for TPRVEP and 28 subjects for SPRVEP. There were 13 patients with organic visual loss enrolled. Mean difference between OVA and GVA was 1.19±0.67 (median=0.84; 95% CI: 1.04 to 1.34) in UVL and 0.14 ±0.09 (median= 0.14; 95% CI: 0.08 to 0.20) in organic visual loss. The area under the ROC curve (AUC) of GVA to distinguish UVL from healthy controls was 0.998 with a cutoff of 0.09 logMAR showing specificity of 100% and sensitivity of 96.0%. CONCLUSIONS: GVA measured by SPRVEP had good diagnostic validity to discriminate patients with unexplained visual loss from healthy controls and patients with organic visual loss, demonstrating its contribution to the diagnosis of this condition.

CLINICAL AND ELECTROPHYSIOLOGICAL EVALUATION AFTER INTRAVITREAL ZIV-AFLIBERCEPT FOR EXUDATIVE AGE-RELATED MACULAR DEGENERATION.

PURPOSE: To evaluate the 6-month safety and efficacy of ziv-aflibercept intravitreal injections for treating exudative age-related macular degeneration. METHODS: Fifteen patients with unilateral exudative age-related macular degeneration were enrolled. The best-corrected visual acuity was measured and spectral domain optical coherence tomography was performed at baseline and monthly. Full-field electroretinography and multifocal electroretinography were obtained at baseline and 4, 13, and 26 weeks after the first injection. All patients received three monthly intravitreal injections of ziv-aflibercept (1.25 mg) followed by as-needed treatment. RESULTS: Between baseline and 26 weeks, the mean logMAR best-corrected visual acuity improved (P = 0.00408) from 0.93 ± 0.4 (20/200) to 0.82 ± 0.5 (20/160) logarithm of the minimum angle of resolution, respectively; the central retinal thickness decreased significantly (P = 0.0007) from 490.3 ± 155.1 microns to 327.9 ± 101.5 microns; the mean total macular volume decreased significantly (P < 0.0001) from 9.51 ± 1.36 mm to 8.08 ± 1.34 mm, and the a-wave implicit time increased, with no differences in the other full-field electroretinography parameters. The average multifocal electroretinography macular responses within the first central 15° showed significantly (P < 0.05) increased P1 amplitudes at 26 weeks. No systemic or ocular complications developed. CONCLUSION: Intravitreal ziv-aflibercept significantly improved the best-corrected visual acuity, multifocal electroretinography amplitudes, central retinal thickness, and total macular volume from baseline to 26 weeks. No retinal toxicity on full-field electroretinography or adverse events occurred during the follow-up period.

Weight-adjusted caffeine and ß-blocker use in novice versus senior retina surgeons: a self-controlled study of simulated performance.

BACKGROUND/OBJECTIVES: Tremor and expertise are potentially influenced variables in vitreoretinal surgery. We investigated whether surgeon experience impacts the association of microsurgical performance with caffeine and ß-blockers weight-adjusted intake. SUBJECTS/METHODS: Novice and senior surgeons (<2 and >10 practice years, respectively) were recruited in this self-controlled, cross-sectional study. A simulator's task sequence was repeated over 2 days, 30 min after the following exposures: day 1, placebo, 2.5 mg/kg caffeine, 5.0 mg/kg caffeine, and 0.6 mg/kg propranolol; and day 2, placebo, 0.2 mg/kg propranolol, 0.6 mg/kg propranolol, and 5.0 mg/kg caffeine. Outcomes were total score (0-700, worst-best), simulation time (minutes), intraocular trajectory (centimeters), and tremor-specific score (0-100, worst-best). RESULTS: We recruited 15 novices (9 men [60%], 1.33 ± 0.49 practice years) and 11 seniors (8 men [72.7%], 16.00 ± 4.24 practice years). Novices performed worse after 2.5 mg/kg caffeine and improved following 0.2 mg/kg propranolol in total score (557 vs. 617, p = 0.009), trajectory (229.86 vs. 208.07, p = 0.048), time (14.9 vs. 12.7, p = 0.048), and tremor-score (55 vs. 75, p = 0.009). Surgical performance improved with propranolol post-caffeine but remained worse than 0.2 mg/kg propranolol in total score (570 vs. 617, p = 0.014), trajectory (226.59 vs. 208.07, p = 0.033), and tremor-score (50 vs. 75, p = 0.029). Seniors' tremor-score was lower after 2.5 mg/kg caffeine compared to 0.2 mg/kg propranolol (8 vs. 37, p = 0.015). Tremor-score following propranolol post-caffeine remained inferior to 0.6 mg/kg propranolol alone (17 vs. 38, p = 0.012). CONCLUSION: While caffeine and propranolol were associated with performance changes among novices, only tremor was affected in seniors, without dexterity changes. The pharmacologic exposure impact on surgical dexterity seems to be offset by increased experience.

Trends in cataract surgical treatment within the Brazilian national public health system over a 20-year period: Implications for Universal Eye Health as a global public health goal.

Cataract is a highly prevalent, treatable, and sight threatening condition considered one of the main focuses of public health policies addressing visual impairment and blindness towards Universal Eye Health. We aimed to investigate the trends on number of cataract surgical procedures performed through the Brazilian national health system (SUS) from 2000 to 2019 while also evaluating costs associated with it. The Brazilian Public Health System Information Database (DATASUS) was used as the primary data source for procedures including extracapsular cataract extraction (ECCE) and phacoemulsification. Trends along the years were evaluated through generalized linear models. A total of 8,424,521 cataract procedures were performed from 2000 to 2019, with a significant increase along the years from 228,145 in 2000 to 663,186 in 2019 (p<0.001), a cataract surgical procedure rate change from 13.15 to 32.28 procedures per 10,000 people. It was observed a significant increase on the number of phacoemulsification (p<0.001) and a significant decrease on the number of ECCE (p<0.001). A shift on the predominant technique has occurred between 2007 and 2008 with phacoemulsification increasing its percentual representativity from 34.3% to 69.7% of all procedures, reaching 96.1% in 2019. Phacoemulsification costs per procedure increased 30.5% from from USD$119.00 to USD$155.33 (p = 0.007) and the ECCE costs per procedure increased 29.1% from USD$78.57 to USD$101.43 (p = 0.001). There is an increasing trend of procedures related to cataract treatment performed through SUS along the 20-years period and a switch on the technique predominance from ECCE to phacoemulsification was observed after 2007. The costs associated with both techniques have increased but have not followed the country's overall inflation. Data derived from DATASUS is important to understand the overall panorama of ocular health offered by the national health system and to provide information to guide healthcare leaders on management and planning of public health policies within the system.

Retinal function in patients treated with tamoxifen.

Tamoxifen, an effective treatment of breast cancer, has been shown to cause ocular toxic effects. The purpose of this study was to determine retinal toxicity by full-field and focal electroretinograms (ERGs) in patients treated with tamoxifen. Fullfield and focal ERGs were obtained from three groups: Tamoxifen-14 females (47-72 years, mean 58.3 + or - 9.1) with normal fundus, treated with tamoxifen from 2 to 37 months; No Treatment-10 females (39-65 years, mean 50.1 + or - 8.7) with previous breast cancer diagnosis and before tamoxifen treatment; Control-13 normal female volunteers (41-81 years, mean 52.7 + or - 12.1). Peak-to-peak amplitude and b-wave implicit time were measured and statistically analyzed.Mean peak-to-peak amplitudes and implicit time from full-field and focal ERGs were comparable for the three different groups. Low-dosage tamoxifen showed no retinotoxic effect assessed by full-field and focal ERG in this small group of women with breast cancer.

Impaired Ganglion Cell Function Objectively Assessed by the Photopic Negative Response in Affected and Asymptomatic Members From Brazilian Families With Leber's Hereditary Optic Neuropathy.

Purpose: The photopic negative response (PhNR) is an electrophysiological method that provides retinal ganglion cell function assessment using full-field stimulation that does not require clear optics or refractive correction. The purpose of this study was to assess ganglion cell function by PhNR in affected and asymptomatic carriers from Brazilian families with LHON. Methods: Individuals either under suspicion or previously diagnosed with LHON and their family members were invited to participate in this cross-sectional study. Screening for the most frequent LHON mtDNA mutations was performed. Visual acuity, color discrimination, visual fields, pattern-reversal visual evoked potentials (PRVEP), full-field electroretinography and PhNR were tested. A control group of healthy subjects was included. Full-field ERG PhNR were recorded using red (640 nm) flashes at 1 cd.s/m2, on blue (470 nm) rod saturating background. PhNR amplitude (µV) was measured using baseline-to-trough (BT). Optical coherence tomography scans of both the retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) were measured. PhNR amplitudes among affected, carriers and controls were compared by Kruskal-Wallis test followed by post-hoc Dunn test. The associations between PhNR amplitude and OCT parameters were analyzed by Spearman rank correlation. Results: Participants were 24 LHON affected patients (23 males, mean age=30.5 ± 11.4 yrs) from 19 families with the following genotype: m.11778G>A [N = 15 (62%), 14 males]; m.14484T>C [N = 5 (21%), all males] and m.3460G>A [N = 4 (17%), all males] and 14 carriers [13 females, mean age: 43.2 ± 13.3 yrs; m.11778G>A (N = 11); m.3460G>A (N = 2) and m.14484T>C (N = 1)]. Controls were eight females and seven males (mean age: 32.6 ± 11.5 yrs). PhNR amplitudes were significantly reduced (p = 0.0001) in LHON affected (-5.96 ± 3.37 µV) compared to carriers (-16.53 ± 3.40 µV) and controls (-23.91 ± 4.83; p < 0.0001) and in carriers compared to controls (p = 0.01). A significant negative correlation was found between PhNR amplitude and total macular ganglion cell thickness (r = -0.62, p < 0.05). Severe abnormalities in color discrimination, visual fields and PRVEPs were found in affected and subclinical abnormalities in carriers. Conclusions: In this cohort of Brazilian families with LHON the photopic negative response was severely reduced in affected patients and mildly reduced in asymptomatic carriers suggesting possible subclinical abnormalities in the latter. These findings were similar among pathogenic mutations.

Population-Based Cataract Surgery Complications and Their Impact on Visual Status in the Brazilian Amazon Region.

PURPOSE: To investigate cataract surgery prevalence, complications, visual outcomes, and coverage in a population of older adults from the Brazilian Amazon region. DESIGN: Population-based cross-sectional study. METHODS: Individuals ≥45 years of age from urban and rural areas of Parintins City, Brazil, were identified by cluster random sampling. Participants underwent ophthalmic examination, including uncorrected visual acuity, acuity with presenting correction (APC), and best-corrected visual acuity (BCVA) from each eye. In those with previous cataract surgery, surgical technique and complications including its contribution to vision impairment were noted. The association of surgical complications and surgical coverage with sociodemographic variables was assessed by multiple logistic regression. RESULTS: A total of 2,384 persons were determined to be eligible, and 2,041 (85.6%) were examined. Overall, prevalence of cataract surgery was 8.6%, with 11.3% urban and 5.0% rural. Surgical complications were associated with males, older age, and urban residency and were found in 60.4%, with posterior capsule opacification in 29.6% and posterior capsule rupture in 16.3%. Among the 270 eyes having cataract surgery, APC ≥20/32 was found in 44.4%, APC 20/40 to 20/63 in 20.8%, APC <20/63 to 20/200 in 14.4%, APC <20/200 to 20/400 in 2.6%, and APC <20/400 in 17.8%. Low surgical coverage was found with 57.9% of those with visual impairment due to cataract not being treated surgically. CONCLUSIONS: Although a reasonable prevalence of cataract surgery was found, a high complication rate, poor visual outcomes, and low cataract surgery coverage indicated that actions to improve quality and appropriate postsurgical management should be planned and implemented by public health authorities.

Multifocal electroretinography, color discrimination and ocular toxicity in tamoxifen use.

PURPOSE: To study prospectively retinal function, color discrimination, and ocular toxicity in women treated with standard-dosage tamoxifen for breast cancer. METHODS: Thirty visually asymptomatic patients with at least 2 years of continuous tamoxifen therapy underwent multifocal electroretinography (ERG), color discrimination testing, and ophthalmic examination. The results were compared with 17 patients who were not taking tamoxifen after breast cancer surgery and to an additional age-matched group of 21 healthy women. RESULTS: Multifocal electroretinogram amplitudes and latencies were comparable among the three studied groups, and individual recordings were within age norms from our own lab. In the treated group, mild diffuse color vision loss was found in two patients with normal fundi. Three other patients had ocular toxic effects, with two cases of refractile retinal crystals and one case of keratopathy. CONCLUSIONS: The aspects of central retinal function that are assessed by multifocal ERG were not affected even after at least 2 years of tamoxifen use, suggesting that the multifocal ERG is not sufficiently sensitive to detect tamoxifen-associated change that might occur. Some degree of color vision loss and ocular toxic effects were found in a few cases of this cohort suggesting that women using tamoxifen should receive an eye exam at least as often as recommended for middle-aged people.

Pattern-reversal visual evoked potentials as a diagnostic tool for ocular malingering.

PURPOSE:: To investigate the contributions of transient pattern-reversal visual evoked potentials in the diagnosis of ocular malingering at a Brazilian university hospital. METHODS:: Adult patients with suspected malingering in one or both eyes were referred for visual evoked potential testing. Data from patients' medical records were reviewed and analyzed retrospectively. Data analysis included the distance optotype visual acuity based on a ETDRS retro-illuminated chart and the transient pattern-reversal visual evoked potential parameters of latency (milliseconds) and amplitude (microvolts) for the P100 component, using checkerboards with visual subtenses of 15' and 60'. Motivations for malingering were noted. RESULTS:: The 20 subjects included 11 (55%) women. Patient ages ranged from 21 to 61 years (mean= 45.05 ± 11.76 years; median= 49 years). In 8 patients (6 women), both eyes exhibited reduced visual acuity with normal pattern-reversal visually evoked potential parameters (pure malingerers). The remaining 12 patients (7 men) exhibited reduced vision in only 1 eye, with simulated reduced vision in the contralateral eye (exaggerators). Financial motivation was noted in 18 patients (9 men). CONCLUSION:: Normal pattern-reversal visually evoked potential parameters with suspected ocular malingering were observed in a 20 patient cohort. This electrophysiological technique appeared to be useful as a measure of visual pathway integrity in this specific population.

Pattern-reversal visual evoked potentials as a diagnostic tool for ocular malingering / Potenciais visuais evocados por padrões reversos como uma ferramenta diagnóstica de simulação

ABSTRACT Purpose: To investigate the contributions of transient pattern-reversal visual evoked potentials in the diagnosis of ocular malingering at a Brazilian university hospital. Methods: Adult patients with suspected malingering in one or both eyes were referred for visual evoked potential testing. Data from patients' medical records were reviewed and analyzed retrospectively. Data analysis included the distance optotype visual acuity based on a ETDRS retro-illuminated chart and the transient pattern-reversal visual evoked potential parameters of latency (milliseconds) and amplitude (microvolts) for the P100 component, using checkerboards with visual subtenses of 15' and 60'. Motivations for malingering were noted. Results: The 20 subjects included 11 (55%) women. Patient ages ranged from 21 to 61 years (mean= 45.05 ± 11.76 years; median= 49 years). In 8 patients (6 women), both eyes exhibited reduced visual acuity with normal pattern-reversal visually evoked potential parameters (pure malingerers). The remaining 12 patients (7 men) exhibited reduced vision in only 1 eye, with simulated reduced vision in the contralateral eye (exaggerators). Financial motivation was noted in 18 patients (9 men). Conclusion: Normal pattern-reversal visually evoked potential parameters with suspected ocular malingering were observed in a 20 patient cohort. This electrophysiological technique appeared to be useful as a measure of visual pathway integrity in this specific population.

RESUMO Objetivo: Investigar a contribuição dos potenciais visuais evocados por padrões reversos no diagnóstico de simulação de baixa de visão em um hospital universitário do Brasil. Métodos: Um grupo de pacientes adultos com suspeita de simulação de baixa de visão em um ou ambos os olhos foi avaliado e os dados analisados retrospectivamente. Foram medidos: acuidade visual de optotipos informada para longe utilizando a tabela ETDRS, parâmetros dos potenciais visuais evocados por padrões reversos de latência (milissegundos) e amplitude (microvolts) para o componente P100 com estímulos de ângulos visuais de 15' e 60'. A motivação do paciente para a simulação foi anotada. Resultados: Os participantes foram 20 indivíduos com 11 (55%) do sexo feminino. A idade variou de 21 a 61 anos (média= 45,05 ± 11,76 anos; mediana= 49 anos). Em 8 pacientes (6 mulheres) ambos os olhos tinham acuidade visual reduzida com parâmetros dos potenciais visuais evocados por padrão reverso normais para ambos os olhos (simuladores puros). Uma subsérie separada de 12 pacientes (7 homens) tinha visão reduzida em apenas um olho e estavam simulando redução da visão no outro olho (exacerbadores). A motivação financeira foi observada em 18 pacientes (9 homens). Conclusões: Parâmetros dos potenciais visuais evocados por padrões reversos normais foram encontrados neste grupo de 20 pacientes com suspeita de simulação. Esta técnica eletrofisiológica pode ser útil como uma medida da integridade do sistema visual nesta população de doentes.