Dense Central Scotoma Research Articles

Minimum Ganglion Cell Layer Thickness is the Best Structural Predictor of Visual Function in Leber Hereditary Optic Neuropathy

Background: Patients with Leber hereditary optic neuropathy, a genetic cause of severe optic atrophy and blindness, demonstrate characteristic structural changes measurable through optical coherence tomography, including initial swelling of the retinal nerve fiber layer and progressive thinning of the ganglion cell layer. After symptomatic conversion, patients experience progressive vision loss, often with dense central scotomas. Aims: This study aims to explore the predictive potential of ocular structural measurements to visual function in patients with Leber hereditary optic neuropathy. Methods: Medical records were reviewed retrospectively for patients with Leber hereditary optic neuropathy clinical testing. Structural measurements, including the average thickness of the ganglion cell layer, the retinal nerve fiber layer, and minimum thickness of the ganglion cell layer, measured through optical coherence tomography were obtained along with measures of visual function such as visual acuity and mean deviation of visual field testing. Simple and multivariable linear regressions were performed to determine correlations between structural measurements and visual functions. Analyses were conducted for all patients, and subgroup of symptomatic patients. Results: Overall, 92 eyes were included with 78 symptomatic eyes. Across all patients, the minimum ganglion cell layer thickness had the highest correlation coefficient with visual acuity (Beta -0.632, adjusted R2 0.396) and with visual field function (Beta 0.572, adj. R2 0.320) compared to the average thickness of the ganglion cell layer (Beta 0.474, adj. R2 0.216) and retinal nerve fiber layer (Beta 0.481, adj. R2 0.223). In multivariate analysis, the minimum ganglion cell layer thickness was the only significant measurement that correlated with visual acuity across all eyes (Beta -0.527, P<0.001) and symptomatic eyes (Beta -0.479, P<0.001). The minimum ganglion cell layer thickness (Beta 0.440, P<0.001) and retinal nerve fiber layer average thickness (0.258, P=0.023) were significant structural measurements correlating to visual field function. Conclusion: The minimum ganglion cell layer thickness is the best structural measure predictor for visual field and visual acuity compared to other common structural measurements in patients with Leber hereditary optic neuropathy. It is a good predictor even when evaluating only symptomatic eyes. Additional attention toward the minimum ganglion cell layer thickness may offer further insight into a patient’s visual potential and the natural history of this disease.

Clinical Overview of Leber Hereditary Optic Neuropathy.

.Leber hereditary ptic neuropathy (LHON) is a disease of young adults with bilateral, painless, subacute visual loss. The peak age of onset of LHON is in the second and third decades of life. Men are 4 times more likely to be affected than women. In about 25-50% of cases, both eyes are affected simultaneously. In unilateral cases, the other eye is usually affected 2 to 3 months later. Visual acuity deteriorates to counting fingers or worse with a dense central or centrocecal scotoma. In the subacute phase, the optic disc may appear hyperemic with swelling of the peripapillary retinal nerve fibre layer, peripapillary telangiectasias, and increased vascular tortuosity. Ocular coherence tomography of the macula shows marked thinning of the ganglion cell complex even at this stage. The diagnosis of LHON is made in a subject with a consistent clinical history and/or one of three common pathogenic mitochondrial DNA (mtDNA) variants identified by molecular genetic testing. Idebenone was approved by the European Medicines Agency under exceptional circumstances for the treatment of LHON. Current evidence suggests some benefit to vision in a subset of affected individuals treated with idebenone, particularly when treated within the first year of onset of vision loss. In this article, we discuss aetiology, clinical features, diagnosis, differential dignosis, prognosis and treatment.

Benefit of Stimulus Size V Perimetry for Patients With a Dense Central Scotoma From Leber's Hereditary Optic Neuropathy.

PurposeLeber's hereditary optic neuropathy (LHON) is the most common mtDNA optic neuropathy. It most frequently causes dense bilateral central scotomas that are often characterized in clinical studies by Humphrey visual field testing (HVF) using a stimulus size III. This provides numerical quantification of the visual field defect using the mean deviation. However, this size III testing strategy has limitations. We used stimulus size V to monitor these patients and evaluated intertest variability and dynamic range to determine the testing reliability and reproducibility.MethodsThis was a longitudinal retrospective cohort study comparing Stimulus III and Stimulus V HVF of 62 LHON patients who had reached the plateau stage of the disease. The intertest variability and mean defect were calculated for both stimulus sizes for 38 patients. The mean defect for stimulus size V was calculated using an algorithm developed by the University of Iowa Visual Field Reading Center.ResultsStimulus size V HVFs had lower inter-test variability as measured by mean defect standard deviation (Z = 169, P < 0.01). The floor effect seen with Stimulus III HVF in LHON, was less pronounced with Stimulus V HVF. The correlation of stimulus size III and V mean defect was strong (r = 0.90, P < 0.01), and a mathematical model was constructed to calculate the Stimulus size V mean defect from the Stimulus size III results (MDstimV = 0.988 x MDStimIII + 1.35, R2 = 0.82 P < 0.01).ConclusionsStimulus size V HVF had lower intertest variability and a better dynamic range than Stimulus size III HVF in LHON patients. This makes the stimulus V HVF a more reliable metric to follow LHON patients especially in clinical trials. The mathematical model presented can be used to generate a Stimulus V equivalent mean defect from Stimulus III HVFs.Translational RelevanceUsing Stimulus V HVF in LHON patients increases its ability to detect and quantify a response to treatment, making it a useful metric for future LHON clinical trials and the clinical setting.

Nonarteritic Anterior Ischemic Optic Neuropathy Simultaneously Associated with Hashimoto’s Thyroiditis

Purpose To report one case of nonarteritic anterior ischemic optic neuropathy (NAION) simultaneously associated with Hashimoto’s thyroiditis. Case summary A-51-year-old man with no underlying disease and painless blurring of vision in his left eye that lasted for a week visited our office. His best-corrected visual acuity was 20/20 in the right eye and 20/1,000 in the left eye. Ophthalmological examination showed a relative afferent pupillary defect in the left eye. Fundoscopic examination revealed swelling of the left optic disc; however, orbital magnetic resonance imaging was normal. Visual field testing by automated perimetry showed a dense central scotoma defect in the left eye. The possibility of optic neuritis was considered; thus, intravenous methylprednisolone (1 g/day) was initiated for 3 consecutive days. Three days after therapy, his visual field showed an inferior altitudinal defect; thus, anterior ischemic optic neuropathy was diagnosed in the left eye. Before the therapy, the anti-thyroid peroxidase antibody level was high, and Hashimoto thyroiditis was detected by thyroid ultrasonography. Conclusions This is the first reported case of NAION associated with concurrent Hashimoto’s thyroiditis. Notably, the patient had none of the risk factors associated with NAION. Keywords: Autoimmune diseases; Hashimoto’s thyroiditis; Nonarteritic anterior ischemic optic neuropathy; Optic disc swelling

THUR 149 ANCA/MPO vasculitis presenting with bilateral ischaemic optic neuropathy

An 80 year old gentleman presented with bilateral, sequential ischaemic optic neuropathy. He initially developed progressive loss of vision in left eye with loss of colour vision and subsequently developed similar symptoms in the right eye with headaches, weight loss, malaise and lethargy. His visual acuity dropped to 6/12 on the right and counting fingers on the left. He had a dense central scotoma in left eye with left-sided RAPD but no other focal neurological deficits.Blood tests revealed an ESR of 107 with an MPO ANCA titre of 19. MRI brain with contrast showed prominent meningeal enhancement and infiltration with ischaemic changes in the brain. CSF analysis revealed WCC of 24 (95% lymphocytes), RCC 22 and protein 0.4 g/L with negative bacterial culture. Temporal artery biopsy was normal.He was treated initially with IV methylprednisolone and 6 cycles of IV cyclophosphamide and subsequently put on methotrexate. His systemic symptoms have resolved completely and his visual acuity continues to gradually improve.MPO-ANCA vasculitis can mimic temporal arteritis and should be considered in patients presenting with an ischaemic optic neuropathy. It is also a treatable cause of meningeal disease.

The Photopic Negative Response: An Objective Measure of Retinal Ganglion Cell Function in Patients With Leber's Hereditary Optic Neuropathy.

The photopic negative response (PhNR) is a slow negative component of a flash photopic full-field ERG that has been shown to be specific for retinal ganglion cell (RGC) activity. Direct evaluation of RGC function is desirable in patients with Leber's hereditary optic neuropathy (LHON) in which the loss of central acuity can make it difficult to monitor patients with standard metrics. The purpose of this study was to evaluate the use of PhNR as an objective noninvasive clinical metric in LHON. Full-field photopic ERG recordings were collected in subjects with the mt.11778G>A/ND4 LHON mutation using a red on blue stimulus. The PhNR was identified using a computer-based automated detection system, and data were manually examined to remove movement artifacts. The PhNR amplitude was compared between controls (n = 13), carriers (n = 17), and affected (n = 6). Mean PhNR amplitude decreased significantly across groups (P < 0.0001). Post hoc Tukey's test revealed a significant decrease in PhNR amplitude between carriers and controls (P < 0.05) and between carriers and affected (P < 0.01). We are able to demonstrate that the PhNR amplitude is significantly decreased in patients affected by LHON compared to carriers in a well-described pedigree. Surprisingly, there was also a decrease in PhNR in carriers, suggesting potential subclinical RGC dysfunction in some carriers. This is important in patients affected with LHON who typically have a dense central scotoma. The PhNR may be a useful objective outcome measure for future clinical trials.

The Pattern of Visual Fixation Eccentricity and Instability in Optic Neuropathy and Its Spatial Relationship to Retinal Ganglion Cell Layer Thickness.

PurposeThe purpose of this study was to assess whether clinically useful measures of fixation instability and eccentricity can be derived from retinal tracking data obtained during optical coherence tomography (OCT) in patients with optic neuropathy (ON) and to develop a method for relating fixation to the retinal ganglion cell complex (GCC) thickness.MethodsTwenty-nine patients with ON underwent macular volume OCT with 30 seconds of confocal scanning laser ophthalmoscope (cSLO)-based eye tracking during fixation. Kernel density estimation quantified fixation instability and fixation eccentricity from the distribution of fixation points on the retina. Preferred ganglion cell layer loci (PGCL) and their relationship to the GCC thickness map were derived, accounting for radial displacement of retinal ganglion cell soma from their corresponding cones.ResultsFixation instability was increased in ON eyes (0.21 deg2) compared with normal eyes (0.06982 deg2; P < 0.001), and fixation eccentricity was increased in ON eyes (0.48°) compared with normal eyes (0.24°; P = 0.03). Fixation instability and eccentricity each correlated moderately with logMAR acuity and were highly predictive of central visual field loss. Twenty-six of 35 ON eyes had PGCL skewed toward local maxima of the GCC thickness map. Patients with bilateral dense central scotomas had PGCL in homonymous retinal locations with respect to the fovea.ConclusionsFixation instability and eccentricity measures obtained during cSLO-OCT assess the function of perifoveal retinal elements and predict central visual field loss in patients with ON. A model relating fixation to the GCC thickness map offers a method to assess the structure–function relationship between fixation and areas of preserved GCC in patients with ON.

Analysis of Mp‐1 audio‐biofeedback impact on fixation in low vision patient with maculopathy

PurposeTo study the impact of a low vision rehabilitation strategy based on Nidek MP‐1 auditory biofeedback (BFB).MethodsRetrospective study of 39 eyes/26 consecutive patients referred for low vision rehabilitation. Patients received 8 monocular training sessions of BFB, each of 10 minutes duration, 7–14 days apart. Microperimetry, ETDRS VA and Pelli‐Robson was performed at the beginning and at the end of the sequence. BFB employs a sound to train the patient to keep a specific gaze position.ResultsAge median was 70. Pre‐BFB logMAR VA was 1, logCS 0.60. Post‐BFB logMAR VA was 1, logCS 0.75. Dense central scotomas and eccentric fixation locations were detected in 35 eyes/24 patients, and relative scotomas with central fixations in 4 eyes/2 patients. Mean sensitivity was 7.60 dB pre‐BFB, and 8 db post‐BFB. Pre‐BFB fixation stability was 24% within 2°, and 67% within 4°; post‐BFB, it was 25% within 2°, and 64% within 4°. BCEA analysis showed pre‐BFB values for 68.2% BCEA to be 3.6, and 4.46 post‐BFB. After BFB, no significant difference was found for any of the above‐mentioned parameters in the sample as a whole. Nevertheless, grouping cases by change in pre‐vs post‐BFB BCEA (group A: decrease in BCEA, n = 21; group B: increase in BCEA, n = 18) showed a significant difference in terms of BCEA itself, and in the related fixation stability (p &lt; 0.05).ConclusionsAlthough no significant difference in VA, CS, mean sensitivity, fixation stability at 2° and 4°, and BCEA was found after BFB in the sample as a whole, still there was an improvement in 21 eyes (53.8%) in terms of BCEA and fixation stability. Grouping analysis demonstrated that, while other tested outcome measures were not affected, some cases did respond significantly to BFB improving their fixation stability.

Comparing fixation location and stability in patients with neovascular age-related macular degeneration treated with or without Ranibizumab

To compare fixation location and stability in patients with neovascular age-related macular degeneration (AMD) treated with or without ranibizumab. Patients were recruited from the Macular Clinic of the King's College Hospital in London. Two groups of patients with neovascular AMD with at least 12 months of follow-up were included in the study. The treated group was treated with ranibizumab while the untreated group did not have any treatment. Best corrected visual acuity (BCVA) with modified ETDRS chart, fixation location and stability as measured with Nidek MP1, central retinal thickness as measured by Zeiss Cirrus SD-optical coherent tomography (OCT), and lesion size as measured by Topcon TRC-50IX camera were analysed and correlated. In total, 102 eyes were included in the study with 76 in the ranibizumab-treated group and 26 in the untreated group. There were no significantly demographic differences between the two groups. However, as expected, the treated group has significantly better vision (48.5 vs 15.5 letters, P < 0.0001) and smaller lesions (10.8 vs 18.3 mm(2), P = 0.004), the central macular thickness as measured by OCT also showed a trend of normalised macular thickness (252 vs 282 microns, P = 0.07). The location of fixation was significantly more central in the ranibizumab-treated group (χ(2) 17.9, P < 0.0001) with over 50% of eyes with predominantly central fixation. Majority (84.6%) of the patients in the untreated group had predominantly eccentric fixation. Fixation stability was significantly better in the ranibizumab-treated group as compared with the untreated group, using both the software provided by the MP1 machine (χ(2) 21.8, P < 0.0001) and the mean log bivariate contour ellipse area calculated from the raw data obtained from the machine (3.64 vs 4.39 in treated and untreated group respectively, P < 0.0001). Low vision rehabilitation strategy for this group of patients in the ranibizumab era will be very different from those used in untreated patients with dense central scotoma. Further studies on the visual rehabilitation in the ranibizumab-treated patients should consider fixation characteristics of the patients.

Unexplained vision loss following removal of epiretinal membrane

PurposeTo report unexplained severe central vision loss accompanied by a dense central scotoma as an uncommon complication following epiretinal membrane removal.MethodsRetrospective, multicentred, case series.ResultsSix patients underwent uncomplicated vitrectomy surgery between...

Leber Hereditary Optic Neuropathy Associated with Malabsorption Syndrome After Bariatric Surgery

We report a patient who developed bilateral visual loss from Leber hereditary optic neuropathy (LHON) in conjunction with chronic malabsorption consequent to bariatric surgery. A 31-year-old man reported visual loss in his left eye and, 2 weeks later, in his right eye. His vision gradually worsened down to finger counting at a distance of 1 m, and he was admitted to the hospital for study. He had undergone vertical banded gastroplasty for morbid obesity 3 years earlier. He smoked 10 cigarettes/day and drank a few alcoholic drinks on weekends. Past ocular history was negative. Family history was remarkable for a maternal cousin with a history of unexplained poor vision since childhood. On our examination, visual acuity was 20/40 in the right eye and 20/200 in the left eye. There was a dense central scotoma in the visual field of the left eye and a pale optic disc in that eye. There was an afferent pupil defect on the left. No other ocular findings were detected, and results of the neurologic examination were unremarkable. Results of extensive hematologic, serologic, and cerebrospinal fluid studies were all normal or negative except for decreased serum levels of vitamin E at 3.4 μg/mL (normal range 5-20 μg/mL), borderline serum levels of vitamin B12 and folate (253 pg/mL and 3.6 ng/mL, respectively), and increased mean corpuscular volume at 101 fL. Results of CT and MRI of the brain and orbits were normal. Pattern-reversal visual evoked potentials revealed marked bilateral delay with P100 latencies of 130.8 ms from the right eye and 156.3 ms from the left eye. The patient received treatment with 1,000 mg/day methylprednisolone intravenously for 5 days, as well as oral vitamin B complex and vitamin E. There was no improvement in vision during his hospitalization. Genetic testing, performed at Public Foundation of Genomic Medicine (Santiago de Compostela, Spain), showed a homoplasmic mitochondrial DNA (mtDNA) mutation at position 11778A, confirming the clinical diagnosis of LHON. On a 10-month follow-up, clinical examinations showed no substantial changes. We have described a young man with a LHON mtDNA mutation and a history of moderate alcohol and tobacco abuse who developed subacute bilateral optic neuropathy in the setting of chronic malabsorption (multiple vitamin deficiency) induced by bariatric surgery. Epigenetic factors are reported to contribute to the clinical expression of LHON mtDNA mutations, including tobacco smoking, alcohol consumption, uncontrolled diabetes, ethambutol use, and antiretroviral therapy (1). There are similarities between the optic neuropathies of LHON, vitamin B1 deficiency, and vitamin B12 deficiency, suggesting that abnormalities of adenosine triphosphate levels might be the common underlying pathogenetic mechanism (2). In our patient, tobacco smoking and alcohol consumption may have contributed. Visual loss began after bariatric surgery, perhaps in the context of malabsorption. Asymptomatic 11778 mtDNA LHON carriers frequently show manifestations of optic nerve impairments in relation to environmental factors (3), just as mutations in the 12S rRNA gene of the mitochondrial genome increase susceptibility to the ototoxicity of aminoglycoside antibiotics (4). Three cases of LHON in subjects who also had low serum vitamin B12 levels at the time of symptom onset have recently been reported (5). Taken together, these observations suggest that optic neuropathy in patients carrying a primary LHON mtDNA mutation may be precipitated by vitamin deficiency. Patients who have undergone bariatric surgery show multiple biochemical abnormalities consistent with malabsorption, including low serum levels of vitamin B12, vitamin D, vitamin E, calcium, copper, or zinc. Some patients develop encephalopathy, optic neuropathy, posterolateral myelopathy, acute polyradiculoneuropathy, and polyneuropathy. Perhaps a positive family history of visual loss should prompt mtDNA investigations before bariatric surgery is considered. Diego Santos-García, MD, PhD Javier Abella, MD Section of Neurology Hospital A. Marcide Ferrol, Spain Begoña De Domingo, MD Service of Ophthalmology Hospital A. Marcide Ferrol, Spain Raúl de la Fuente-Fernández, MD Section of Neurology Hospital A. Marcide Ferrol, Spain [email protected]

Increased Role of Peripheral Vision in Self-Induced Motion in Patients with Age-Related Macular Degeneration

The contribution of peripheral vision in inducing self-motion (vection) was investigated in people with bilateral age-related macular degeneration (AMD). Eleven patients with bilateral AMD and dense central scotomas with no islands of functional central retina and 12 age-matched control subjects were exposed to random-dot patterns projected on a large screen. The dots either moved from left to right, inducing linear vection, or rotated about the roll axis, inducing roll vection. Latency, total vection time, and objective and subjective measures of tilt were recorded. The patients with AMD experienced shorter latencies than did the age-matched control participants, but the total vection time in both conditions and tilt during roll vection were the same in both groups. There was a positive correlation between objective tilt and subjective measures of tilt in the AMD, but not in the age-matched control group. There was a negative relationship between absolute scotoma size and latency. Two main conclusions were drawn. First, the role of peripheral vision in inducing vection is enhanced in people with bilateral central vision loss. Second, people with bilateral AMD adapt successfully to a moving environment (they do not experience vection longer, nor do they tilt more) and are more aware of their postural position than are age-matched control subjects.

Long-Term Evaluation of Eccentric Viewing Spectacles in Patients With Bilateral Central Scotomas

Yoked prism spectacles (eccentric viewing spectacles [EVS]) facilitate eccentric viewing in patients with bilateral central scotomas. This study was conducted to evaluate the long-term success and patient satisfaction of this type of low-vision aid. METHODS. In this retrospective cross-sectional study, we reviewed the low-vision rehabilitation records of all patients who received EVS since 1993. With an 18-item survey, 191 patients were questioned about the wearing characteristics, advantages, and disadvantages of the EVS. Descriptive and nonparametric statistics were applied to compare regular users with those persons who no longer use the EVS. The response rate was 83.2% and the average follow-up time was 4.5 years. Forty percent of all patients still used the EVS. The main disadvantages of these spectacles, mentioned mainly by the nonusers, included the heavy weight (41%), perception of curved lines (46%), dizziness during walking (46%), and poor cosmetic appearance (25%). Many successful long-term users considered the EVS suitable for home activities (86%) and walking in the street (70%) and reported that this device reduced eccentric viewing (77%), created better vision in the center of their visual field (67%), and helped with recognition of objects and faces (64%). The prescription of EVS aids eccentric viewing in patients with dense central scotomas. Although associated with a number of disadvantages and side effects, 40% of the patients continued to use EVS. The results of this study indicate that patients who experience difficulties with eccentric fixation are most likely to benefit from these low-vision aids. A thorough explanation of the advantages and disadvantages of the EVS is important to prevent unnecessary disappointments.

Reversible Optic Neuropathy Associated With Low-Dose Methotrexate Therapy

A 66-year-old woman had progressive bilateral optic neuropathy with dense central scotomas and dyschromatopsia. She had been taking oral methotrexate 2.5 mg three times per week for rheumatoid arthritis for the previous 10 months (total intake 322.5 mg) without folic acid supplementation. She had never smoked or abused alcohol and her diet was healthy. Serum folate was reduced at 1.6 ng/mL (normal >4 ng/mL) and vitamin B12 levels were normal. After stopping methotrexate and after administration of oral folic acid, she experienced complete recovery of vision. Serum folate levels returned to normal during folic acid treatment but decreased to below normal once folic treatment was stopped. The persistently low folate level remains unexplained and may reflect a genetic defect in folate metabolism. Methotrexate can cause toxic side effects resulting from folate inhibition but has not been shown definitively to cause a reversible optic neuropathy associated with low serum folate.

Fixation pattern and macular sensitivity in eyes with subfoveal choroidal neovascularization secondary to age-related macular degeneration. A microperimetry study

Purpose. To investigate the effects of subfoveal choroidal neovascularizzation (CNV) secondary to age-related macular degeneration (AMD) on macular functional parameters quantified with an automatic fundus perimeter. Methods. 118 eyes of 98 consecutive patients with subfoveal CNV secondary to AMD were evaluated. Best corrected visual acuity (ETDRS charts), fundus photography, and fluorescein angiography were performed. Microperimetry (fundus-related perimetry) was used to quantify macular sensitivity and fixation pattern (location and stability). Results. Of 118 eyes: 26 (21.9%) had central, 18 (15.1%) poor central and 74 (63.0%) eccentric fixation; 31 (26.0%) had stable, 42 (35.6%) relatively unstable and 45 (38.4%) unstable fixation. In 75 eyes (63.4%) a dense central scotoma was found. Angiographic classification of subfoveal CNV (occult versus classic) was not significantly related to fixation pattern (location: P = 0.274; stability: P = 0.385), and presence of dense scotoma (P = 0.41). Conclusion. Microperimetric quantification of macular sensitivity and fixation pattern in eyes with subfoveal CNV secondary to AMD offers new data about the impact of visual impairment in these eyes. Moreover, microperimetry improves the functional evaluation of subfoveal CNV in AMD.

Detection of localized retinal dysfunction in a choroideremia carrier

Purpose To investigate severe unilateral vision loss in a choroideremia carrier. Design Case report. Methods Ocular examination, genetic testing, Humphrey visual fields, full-field and multifocal (mf) electroretinogram (ERG) tests were used to study a family with choroideremia. Results In a carrier with unilateral central vision loss, mfERG showed severely reduced amplitudes which correlated with a band of retinal pigment epithelial and choroidal atrophy in the macula, a dense central scotoma on Humphrey visual fields testing, and decreased ERG amplitudes. Conclusions Multifocal ERG may be a sensitive tool to measure functional abnormalities in choroideremia carriers. Mosaic inactivation of the normal gene may cause expression of the mutation with severe vision loss in choroideremia carriers.

Characteristics of visual loss by scanning laser ophthalmoscope microperimetry in eyes with subfoveal choroidal neovascularization secondary to age-related macular degeneration

Purpose To evaluate the effects of subfoveal choroidal neovascularization secondary to age-related macular degeneration on functional parameters obtained by scanning laser ophthalmoscope microperimetry. Design Retrospective observational case series and cross-sectional study. Methods At the Doheny Retina Institute and Wilmer Eye Institute a consecutive series of 179 eyes of 175 patients with subfoveal choroidal neovascularization secondary to age-related macular degeneration was studied. The onset of visual symptoms, best-corrected visual acuity, fluorescein angiography, evaluation of fundus microperimetry and fixation pattern using the Rodenstock scanning laser ophthalmoscope were obtained for each patient. The main outcome measures were central retinal sensitivity and fixation pattern (fixation location and fixation stability) in eyes with subfoveal choroidal neovascularization and their relationship to the length of disease, type and characteristics of choroidal neovascularization, and visual acuity. Results Of 179 eyes, 135 (75%) had central fixation, 27 (15%) had poor central fixation, and 17 (9%) had predominantly eccentric fixation. Seventy-six eyes (42%) had stable fixation, 70 eyes (39%) had relatively unstable fixation, and 33 eyes (18%) had unstable fixation. In 50 eyes (28%) a dense central scotoma was noted. Eighty-nine of 100 eyes (89%) with length of symptoms of less than 3 months had predominantly central fixation and 58 (58%) had stable fixation; 14 of 34 eyes (41%) with length of symptoms of more than 6 months had predominantly central fixation, and 5 eyes (15%) had stable fixation. In 15 eyes of patients who elected not to receive treatment, successive scanning laser ophthalmoscope microperimetry were obtained over time (follow-up of 18 months after onset of symptoms). Three months or less after the onset of symptoms, 13 eyes (87.7%) had predominantly central fixation and 9 eyes (60%) had stable fixation. More than 3 months and 6 months or less after the onset of symptoms, 10 eyes (66.7%) had predominantly central fixation and 7 eyes (46.7%) had stable fixation. This trend was further demonstrated in eyes more than 6 months after the onset of symptoms. Conclusions We conclude that the sequence of events leading to visual function deterioration appears to involve an initial mild decrease in central retinal sensitivity and visual acuity followed by progressive fixation instability and, ultimately, development of an absolute central scotoma with totally eccentric fixation. Increased length of disease is associated with worse fixation pattern and retinal sensitivity deterioration as assessed by scanning laser ophthalmoscope microperimetry. A better understanding of the characteristics of visual loss assessed by fixation pattern evaluation and microperimetry in age-related macular degeneration may help optimize timing, patient selection, and treatment options in eyes with this condition.

Patient selection for macular translocation surgery using the scanning laser ophthalmoscope

Objectives To evaluate the use of the scanning laser ophthalmoscope (SLO) as a predictor for potential visual improvement in eyes with subfoveal choroidal neovascularization (CNV) secondary to age-related macular degeneration (AMD) undergoing limited macular translocation. Design Retrospective noncomparative case series. Participants Consecutive series of 71 eyes of 70 patients with subfoveal CNV secondary to AMD that underwent effective and uncomplicated limited macular translocation at the Wilmer Ophthalmological Institute. Methods Evaluation of the fundus microperimetry and fixation pattern was conducted on all patients using the Rodenstock scanning laser ophthalmoscope (G. Rodenstock Instrument GmbH, Munich, Germany) within 72 hours before surgery. Main outcome measures Sensitivity and specificity in addition to positive and negative predictive values of SLO findings for visual outcome after macular translocation. Results Stable fixation presented the highest positive predictive value for visual acuity equal to or better than 20/100 (30 of 33 = 0.91), and predominantly eccentric fixation had the highest negative predictive value for visual acuity worse than 20/100 (7 of 9 = 0.78). Sensitivity was higher for the presence of predominantly central fixation (42 of 48 = 0.87), and specificity was higher for relatively unstable/unstable fixation (20 of 23 = 0.87). Conclusions Eyes with stable and central fixation (without dense central scotoma) good preoperative visual acuity, and short length of symptoms are those with the greatest chance to achieve good vision after macular translocation. The knowledge of the fixation and microperimetry pattern enables better understanding of the macular function in eyes with AMD and may be useful for evaluation of baseline retinal cell viability. Incorporation of these testing modalities may help to optimize patient selection for macular translocation or other future techniques aimed at rescuing photoreceptors.

Scotoma and fixation patterns using scanning laser ophthalmoscope microperimetry in patients with macular dystrophy

PURPOSE: We used scanning laser ophthalmoscope microperimetry to evaluate the retinal scotoma and the fixation points in the patients with macular dystrophy. METHODS: We studied 10 eyes of five patients with macular dystrophy (three patients with cone dystrophy and two patients with Stargardt disease). The mean patient age was 37 years (range, 13 to 64 years). An estimation of scotoma and fixation points on the retina was performed using scanning laser ophthalmoscope microperimetry. RESULTS: All 10 eyes (100%) had one of two types of dense scotoma: type one was a dense ring scotoma (five eyes, 50%), and type two was a dense central scotoma (five eyes, 50%) that included the center of the fovea. In all eyes with a dense ring scotoma, the fixation points were stable and did not shift. In all eyes with a dense central scotoma, the fixation shifted. The logarithm of minimal angle of resolution of the visual acuity in the eyes with the dense central scotoma was significantly worse than that of eyes with the dense ring scotoma type ( P = .005). CONCLUSIONS: Scanning laser ophthalmoscope microperimetry findings demonstrate two types of dense scotoma (dense ring scotoma and dense central scotoma) in the patients with macular dystrophy. The two types of dense scotoma affect the shifting of the fixation points and the stability of fixation and may result in the difference in visual acuity in the patients with macular dystrophy.

Detection of fenestrated central scotoma by scanning laser ophthalmoscope microperimetry in a patient with Leber’s hereditary optic neuropathy after visual recovery

Landmark-driven fundus microperimetry using a scanning laser ophthalmoscope (SLO) was performed to assess the local sensitivities of the central retina and to determine the fixation point in a patient with Leber’s hereditary optic neuropathy (LHON) after visual recovery. A 16-year-old Japanese boy was diagnosed as having LHON with an 11778 mutation of mitochondrial DNA. Fourteen months after starting idebenone and vitamin therapy, his vision began to recover and reached 1.0 in both eyes. His corrected visual acuity, Humphrey 10-2 threshold test, SLO microperimetry, critical flicker frequency (CFF), and visual evoked potentials (VEPs) were tested after visual recovery. At an early stage of visual recovery, the Humphrey 10-2 threshold test revealed a fenestrated scotoma in the right eye and a dense central scotoma in the left eye. SLO microperimetry of the left eye, however, detected a few spots that were sensitive to a stimulus intensity of 0 dB on the temporal side of the fovea. A fixation point was detected on the nasal side of the fovea and it was relatively stable in both eyes. CCF and VEPs deteriorated despite the recovery of vision in both eyes. The ability to detect small islands of vision and to identify fixation points are two advantages of SLO microperimetry over Humphrey perimetry.