Scotoma Research Articles

Reduced spatial attentional distribution in older adults.

Older adults show decline in visual search performance, but the underlying cause remains unclear. It has been suggested that older adults' altered performance may be related to reduced spatial attention to peripheral visual information compared with younger adults. In this study, 18 younger (M = 21.6 years) and 16 older (M = 69.1 years) participants performed pop-out and serial visual search tasks with variously sized gaze-contingent artificial central scotomas (3°, 5°, or 7° diameter). By occluding central vision, we measured how attention to the periphery was contributing to the search performance. We also tested the effect of target eccentricity on search times and eye movements. We hypothesized that, if attention is reduced primarily in the periphery in older adults, we would observe longer search times for more eccentric targets and with central occlusion. During the pop-out search, older adults showed a steeper decline in search performance with increasing eccentricity and central scotoma size compared with younger adults. In contrast, during the serial search, older adults had longer search times than younger adults overall, independent of target eccentricity and scotoma size. Longer search times were attributed to higher cost-per-item slopes, indicating increased difficulty in simultaneously processing complex symbols made up of separable features in aging, possibly stemming from challenges in spatially binding individual features. Altogether, our findings point to fewer attentional resources of simultaneous visual processing to distribute over space or separable features of objects, consistent with decreased dorsal visual stream functioning in aging.

Extensive Macular Atrophy with Pseudodrusen-like appearance: Progression Kinetics and Late-Stage Findings

To describe the clinical outcome and late-stage findings of extensive macular atrophy with pseudodrusen-like appearance (EMAP). Retrospective cohort study. Seventy-eight patients (156 eyes) affected by EMAP. We collected data on best-corrected visual acuity, kinetic perimetry, OCT, short-wavelength autofluorescence, and near-infrared autofluorescence findings. Genetic testing for the TIMP3 and C1QTNF5 genes was performed via Sanger sequencing for 58 patients, with no pathogenic variants identified. The primary outcomes were best-corrected visual acuity at the last examination, visual field at the last examination, and incidence rates and time-to-event curves for blindness with the United States Social Security Administration and World Health Organization (WHO) criteria, foveal involvement, and atrophy enlargement beyond the 30° and 55° field of view. Imaging findings at the last examination were secondary outcomes. At the most recent visit, mean age was 70.9 ± 5.2 years. Using United States criteria, 58.1% of the patients were blind, and 25.8% were blind according to WHO criteria. All eyes showed large central scotomas, which were associated with visual field constriction in 22.2% of eyes. We detected focal openings or large dehiscences of Bruch's membrane (BM) in 25.4% of eyes. Near-infrared autofluorescence showed increased visibility of the choroidal vessels beyond the atrophy in 87.2% of eyes. The incidence rates for blindness were 3.95 per 100 patient-years with United States criteria and 1.54 per 100 patient-years according to WHO criteria. The incidence rates were 22.8 per 100 eye-years for foveal involvement, 12.0 per 100 eye-years for atrophy enlargement beyond 30°, and 6.6 per 100 eye-years for atrophy enlargement beyond 55°. The estimates were not influenced by the age at onset. We identified characteristic imaging findings, including BM ruptures, in elder patients with EMAP and calculated incidence rates for different functional and anatomic outcomes. Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Correlation of Retinal Nerve Fibre Layer Thickness in Optical Coherence Tomography with Visual Outcome in Optic Neuritis

Abstract Purpose: In this study, the changes in optical coherence tomography (OCT) were correlated with the post-treatment visual outcome of optic neuritis, thereby determining the patients who are at risk of optic atrophy. Design: Prospective observational study. Methodology: All patients presenting to the outpatient department (OPD) with acute visual loss, pain on ocular movements and colour desaturation were subjected to comprehensive ophthalmic examination and OCT retinal nerve fibre layer (RNFL) analysis. All patients were treated with an Optic Neuritis Treatment Trial (ONTT) regimen and followed for one year. Results: Of 25 patients, 40% were 20 to 40 years of age, and females outnumbered males by 28%. The right eye had 48% unilateral affection and 12% bilateral affection; 56% had a central scotoma, and 24% had a centrocaecal scotoma. The most common aetiologies are as follows: idiopathic (84%), multiple sclerosis (MS) (12%) and Devic’s disease (4%). 60% presented with a visual acuity of 6/60–1/60 and 32% with a visual acuity of 6/18–6/36. Patients whose RNFL thickness was below 90 microns post-treatment had poorer visual outcome. Conclusion: In this study, optic neuritis was more common in females of 20 to 45 years with unilateral affection in the right eye. The most common field defect was central scotoma, and the most common cause of optic neuritis was idiopathic. It was also found that the visual acuity in optic neuritis can range from 6/18 to no PL. Of the 25 patients in the study, three patients had retrobulbar neuritis and six patients developed disc pallor during recovery after treatment.

Hemorrhagic unilateral retinopathy: a report of multimodal imaging in three cases.

To report three cases of hemorrhagic unilateral retinopathy, diagnosed by multimodal imaging. Case report of 3 patients, 2 women and one man, aged 51, 74, and 52, respectively. Symptoms were acute floaters, blurred vision, or central scotoma, unilateral in all cases. The best-corrected visual acuity was 20/20 in the affected eye in 2 patients with a paracentral scotoma, and 20/160 in the third patient. Funduscopic examination revealed multiple unilateral posterior hemorrhages located in the Henle fiber layer in the macula and beneath the internal limiting membrane around the optic disc on spectral-domain optical coherence tomography (SD-OCT). Fluorescein angiography and indocyanine green angiography (ICGA) did not show any vascular abnormalities. SD-OCT angiography (SD-OCT-A) did not show any capillary drop-out or choroidal abnormalities. In all patients, the visual symptoms completely disappeared within a few weeks, with spontaneous regression of the hemorrhages. Hemorrhagic unilateral retinopathy is a rarely reported and poorly understood disorder. ICGA and SD-OCT-A did not allow better understanding the condition. No etiology has been associated with this entity so far. The spontaneous resolution of the present cases confirmed the favorable visual prognosis of the condition.

Acute retinal pigment epitheliitis during treatment of hyperprolactinaemia

BackgroundAcute retinal pigment epitheliitis (ARPE) is a rare, idiopathic and self-limiting disease. The article aims to present ARPE in a patient using D2 dopamine receptor agonists for the treatment of hyperprolactinemia.Case presentationA 28-year-old female during hyperprolactinaemia treatment suffered from a dyschromatopsia and a central visual field defect in the left eye. She noticed a deterioration of vision and discontinued the cabergoline administration. The woman had not been diagnosed with other chronic conditions and exhibited no symptoms of infection. Upon admission, the patient was subjected to a test for COVID-19, which was negative.The ophthalmological examination revealed a decrease in visual acuity to distance in the left eye, which amounted to 18/20 on the Snellen chart. A central scotoma was noted on the Amsler chart and a loss of pigment epithelium was visible on the fundus of the left eye. Fluorescein angiography showed a discrete window defect in the left one, with no signs of leakage. Optical coherence tomography (OCT) scans of the maculae revealed a characteristic change in the photoreceptor layer and retinal pigment epithelium (RPE) in the fovea in the left eye. The electrophysiological tests revealed decreased function of cells in macular region.A magnetic resonance imaging (MRI) of the head and orbits demonstrated an asymmetric pituitary gland without chiasm compression and discrete signal enhancement from the left optic nerve.The patient underwent observation during hospitalisation. She reported improved colour vision and a decreased scotoma in the centre of her visual field.In regular outpatient follow-ups, successive improvements in visual acuity, as well as a decreased RPE damage and outer photoreceptor layer loss during an OCT test were observed.ConclusionsA case of ARPE is reported in a patient taking medications for hyperprolactinemia. The role of dopamine receptor antagonists in the photoreceptor function and causation of ARPE needs further evaluation.

Successful treatment of multiple sclerosis with refractory rheumatoid arthritis using ofatumumab: A case report

AbstractBackgroundOfatumumab is an anti‐CD20 human monoclonal antibody that is approved in several countries worldwide for the treatment of relapsing forms of multiple sclerosis (MS). Recent studies have shown promising results of ofatumumab therapy for rheumatoid arthritis (RA). We report a case with both MS and refractory RA that was successfully treated with ofatumumab.Case PresentationA 44‐year‐old woman with a history of RA since the age of 40, and prior treatment with methotrexate, prednisolone, and several disease‐modifying antirheumatic drugs (DMARDs), including salazosulfapyridine, iguratimod, tacrolimus, presented with a recent onset of visual acuity loss in the left eye. Ophthalmic examination revealed a decreased central flicker frequency (CFF) and central scotoma. Brain magnetic resonance imaging (MRI) revealed periventricular multiple lesions and contrast enhancement of the left optic nerve. Cerebrospinal fluid analysis revealed mild lymphocytic pleocytosis, elevation of protein, and oligoclonal bands. Serum anti‐aquaporin‐4 (AQP4) antibodies, anti‐myelin oligodendrocyte glycoprotein (MOG) antibodies, and other serological tests for optic neuritis were unremarkable. She was diagnosed with relapsing and remitting MS at 10 months after development of optic neuritis when she experienced a relapse, accompanied by new asymptomatic lesions detected on brain MRI. After ofatumumab administration, we discontinued all DMARDs and maintained remission over a 12‐month period.ConclusionThere is growing evidence of significant involvement of B‐cells in the pathogenesis of RA and the effectiveness of B‐cell depletion therapy in managing RA. Ofatumumab is an effective treatment for patients with MS and refractory RA.

Classic Choroidal Neovascularization in Young Man

Introduction : Classic choroidal neovascularization (CNV 2) represent a pathological growth of blood vessels and can result in loss of visual function. The most common cause of CNV 2 in elderly patients is age- related macular degeneration, meanwhile in young age it is frequently due to inflammation, high myopia, angioid disks and hereditary disorders. Hence, the presented case reports the characteristic findings as well the management of the disease.
 Case Illustration : A 37-year-old man had two weeks history of metamorphopsia on both eyes. The left eye (LE) had a worse visual acuity, macular fibrosis with pigment epithelial detachment in funduscopy and Optical Coherence Tomography (OCT). A submacular hemorrhage and exudative lesion were recognized on the right eye (RE) with type 2 choroidal neovascularization. Fundus Fluorescence Angiography (FFA) was also performed. Systemic conditions were unremarkable. Intravitreal anti-VEGF injections on RE were given for three consecutive months and showed significant improvement.
 Discussion : The etiology of CNV 2 includes high myopia, and inflammation. However, none was found in this patient. Nonetheless, CNV type 2 is still uncommon in young age without predisposing conditions. As most common lesions involve the macula, symptoms shown include metamorphopsia, central scotoma and floaters. FFA is still considered to be the gold standard to differentiate the types of choroidal vascularization. Neovascularization and submacular hemorrhage can be treated with intravitreal injection of anti-VEGF, as previous studies reported significant improvement.
 Conclusion : This was a case of bilateral CNV type 2 in a young man without any previous predisposing conditions. Three monthly injections of anti-VEGF showed significant improvement.

Interpretation of the Visual Field in Neuro-ophthalmic Disorders.

In this review, we will describe current methods for visual field testing in neuro-ophthalmic clinical practice and research, develop terminology that accurately describes patterns of field deficits, and discuss recent advances such as augmented or virtual reality-based perimetry and the use of artificial intelligence in visual field interpretation. New testing strategies that reduce testing times, improve patient comfort, and increase sensitivity for detecting small central or paracentral scotomas have been developed for static automated perimetry. Various forms of machine learning-based tools such as archetypal analysis are being tested to quantitatively depict and monitor visual field abnormalities in optic neuropathies. Studies show that the combined use of optical coherence tomography and standard automated perimetry to determine the structure-function relationship improves clinical care in neuro-ophthalmic disorders. Visual field assessment must be performed in all patients with neuro-ophthalmic disorders affecting the afferent visual pathway. Quantitative visual field analysis using standard automated perimetry is critical in initial diagnosis, monitoring disease progression, and guidance of therapeutic plans. Visual field defects can adversely impact activities of daily living such as reading, navigation, and driving and thus impact quality of life. Visual field testing can direct appropriate occupational low vision rehabilitation in affected individuals.

Optic Nerve Head Curvature Flattening Is Associated with Central Visual Field Scotoma.

This study aimed to develop a new index, the average curvature ratio (ACR), to represent the optic nerve head (ONH) tilting and investigate its clinical relevance. Myopic eyes were included and divided into two subgroups: flat ONH (ACR < 1.0) and convex ONH (ACR ≥ 1.0). The occurrences of central and peripheral visual field (VF) defects were compared between the two groups. A total of 375 myopic eyes were recruited, and 231 and 144 eyes were included in the flat and convex ONH groups, respectively. Central scotoma occurred more frequently in the flat ONH group. According to the Patella-Anderson criteria, the number of eyes with central scotoma was 103 (44.6%) in the flat and 44 (30.6%) in the convex ONH groups (p = 0.009). According to Kook's criteria, the number of eyes with central scotoma was 122 (52.8%) in the flat and 50 (34.7%) in the convex ONH groups (p < 0.001). Peripheral scotoma was not significantly different between the groups. In the correlation analysis, the ACR was positively correlated with spherical equivalence, but not with axial length or central corneal thickness. The ACR reflects the degree of the ONH tilt and is a good index for estimating central VF damage in myopic eyes.

Foveal-Sparing ILM Peeling with ILM Flap Transposition over the Macular Hole for Repair of Full-Thickness Macular Holes.

Full-thickness macular holes, defined as full-thickness defects of the fovea, lead to central scotoma and deterioration of vision. Apart from peeling of the internal limiting membrane (ILM), ILM flap techniques have been reported to have potential in improving results in macular hole surgery. In addition, foveal-sparing ILM peeling gives a high macular hole closure rate and improvement in postoperative visual acuity. The aim of this study was to examine outcomes in a cohort of patients with full-thickness macular holes that underwent vitrectomy with foveal-sparing ILM peeling and transposition of an ILM flap over the macular hole. This retrospective study included patients scheduled for pars plana vitrectomy with foveal-sparing ILM peeling, combined with ILM flap transposition over the macular hole, for macular hole repair. All patients received a gas tamponade with 20% sulphur hexafluoride and were encouraged to undergo postoperative face-down positioning for 48 hours after surgery. Optical coherence tomography (OCT) imaging of the macula and distance-corrected visual acuity (DCVA) were performed before and 3 months after surgery. In total, 42 eyes of 42 patients were included in this study. Leaving a broad area of residual ILM at the foveal rim led to a high risk of failure in macular hole closure, while leaving a narrow zone of residual ILM at the foveal rim resulted in high macular hole closure rates (97% type 1 closure and 3% type 2 closure), with a median improvement of DCVA of 4lines [interquartile range (IQR): 3 to 6] among pseudophakic and 3.5 lines (IQR: 2 to 5) among phakic patients. A combination of foveal-sparing ILM peeling with ILM flap techniques was shown to be a safe and effective surgical option for patients with full-thickness macular holes, resulting in a high macular hole closure rate and improvement in visual acuity in the majority of patients.

Unilateral Syphilitic Optic Neuritis without Uveitis

This report describes a case of case of unilateral syphilitic optic neuritis without uveitis. A 56-year-old man presented with complaints of four days history of decreasing vision in his left eye. At the initial visit, the patient’s best corrected visual acuity was 1.2 and 0.03 in the right and left eye, respectively. A relative afferent pupillary defect was observed in the left eye. Slit-lamp examination revealed no cells, flare or keratic precipitates in the anterior segment either eye. Fundoscopy revealed diffuse optic disc swelling with peripapillary hemorrhage in the left eye. Goldmann visual field testing identified central absolute scotoma and an enlarged blind spot in the left eye. On serological evaluation for syphilis, serum rapid plasma reagin (RPR) and treponema pallidum hemagglutination assay (TPHA) were positive (RPR 52.5, TPHA 740.0). In addition, IgM fluorescent Treponemal antibody absorbance (FTA-ABS) was positive. In contrast, cerebrospinal fluid showed negative RPR and positive TPHA (TPHA 11.8). Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis and increased protein content and glucose. Magnetic resonance imaging showed a swollen enhanced lesion in the left optic nerve. The patient was diagnosed with syphilitic optic neuritis and received the currently recommended treatment with intravenous penicillin G (PCG). Twenty-four million units per day of PCG were administered for 2 weeks. After 2 weeks of therapy, his left visual acuity improved to 0.3 in 2 months. However, the optic disc looked diffusely pale in the left eye. At 6-month visit, the patient was clinically stable. RPR turned out negative and there was a reduction in serological TPHA reactivity. We consider that it is essential to perform serological tests for syphilis in every patient with optic neuritis, which makes it possible to initially treat syphilitic optic neuritis with PCG.

Lamotrigine‐associated macular phototoxicity in Asperger syndrome

Purpose: Asperger's syndrome is a neurodevelopmental disorder that falls within the autism spectrum disorder family. Comorbid conditions such as depression and anxiety are commonly associated, and lamotrigine is often prescribed off‐label for treatment. It has the capacity to absorb ultraviolet (UV) light and generate free oxygen radicals, which can potentially cause cellular damage. In this case report, we present multimodal imaging findings in a patient diagnosed with solar maculopathy.Methods: A young patient was referred to our department for bilateral visual decline and central scotoma. The patient was examined with multimodal imaging including fundus photography, fundus autofluorescence (FAF), swept‐source optical coherence tomography (SS‐OCT) and multifocal electroretinogram.Results: A 25‐year‐old patient with Asperger's syndrome treated with lamotrigine was referred to our department for bilateral visual decline and central scotoma. Best corrected visual acuity (BCVA) was 0.40 in both eyes. Dilated fundus examination revealed a bilateral yellow foveal lesion. Fundus autofluorescence (FAF) imaging showed foveal hyperautofluorescence. SS‐OCT in the macular region of both eyes showed a disruption of the ellipsoid zone and the external limiting membrane. Upon further questioning, the patient disclosed history of repeatedly gazing at the sun. The diagnosis of solar maculopathy associated with lamotrigine was established.Conclusion: This is the second report of lamotrigine associated retinal phototoxicity. Lamotrigine, recognized as a photosensitizer, may induce a photochemical response, with retinal phototoxicity. Caution must be exercised to educate patients about the importance of not gazing directly at the sun, as it van cause irreversible retinal impairment.

LHON spectrum disorder: Phenotype and genes

Leber hereditary optic neuropathy (LHON) is an inherited optic neuropathy primarily affecting young male with subacute bilateral visual loss. Clinically it is characterized by central scotoma, discromatopsia, and usually permanent central visual loss.This disease has a typical optic disc appearance at fundus examination of pseudoedema and microangiopathy which characterize the asymptomatic and subacute phases of the disease. Optic disc atrophy follows in the chronic phase.When the disease manifests, the preferential involvement of papillomacular fibres and macular retinal ganglion cell (RGC) damage are evident. These clinical features combined with the ophthalmoscopic pattern create the framework that make the disease unique.Primary point mutations of mitochondrial DNA (mtDNA) have been described as the genetic cause of the disease in about 90% of cases. Recently, new autosomal recessive DNA mutations have been reported in patients affected by subacute visual loss associated with a clinical picture resembling LHON.We herein describe the phenotype of 6 patients in which whole‐exome sequencing identified variants in the MECR gene in 2 probands of the same pedigree, in the NDUFAF4 gene in one patient, and mutation in the nuclear gene DNAJC30 in 3 patients. The 3 male probands with DNAJC30 mutation presented the typical LHON clinical picture with subacute loss of vision at an adult age in 2 cases and at a childhood age in one case. The pattern of visual loss with central scotoma and discromatopsia was associated with pseudoedema and microangiopathy at fundus appearance. Optical coherence tomography (OCT) analysis showed in both acute and asymptomatic family members the swelling of fibres while in the dynamic and chronic stages a progressive thinning of fibres. Visual recovery was detected in the younger patient and, partially, in one of adult patient.The 3 other patients carrying the MECR and NDUFAF4 gene variants showed a particular course of the disease characterized by a childhood onset of visual loss followed by a recurrence of visual loss at an adult age. The childhood onset was insidious in one case and subacute in the other 2 cases (about 6 years of age).OCT analysis demonstrated in the insidious case the temporal thinning of fibres and the macular RGC atrophy followed by swelling of fibres during the recurrence.In the differential diagnosis of inherited optic neuropathies with clinical presentation resembling LHON we should take into consideration the recessive form with mutations in the DNAJC30 gene which appears to overlap the mtDNA LHON disease. Moreover, clinical patterns resembling LHON with recurrence of visual loss represent another form of LHON with unique clinical presentation associated with variants in the MECR and the NDUFAF4 genes. These new clinical and genetic patterns enlarge the spectrum of LHON disease.

Digoxin‐induced retinal toxicity after acute kidney injury in a patient with dilated cardiomyopathy: A case report

Aims/Purpose: We would like to report a case of digoxin‐induced retinal toxicity.Methods: Observational case report. Clinical follow‐up was performed using optical coherence tomography and electroretinography.Results: A 50‐year‐old female patient was referred to our clinic with symptoms of blurred vision and a whitening of the central visual field. She had no prior ophthalmic history but had underlying diseases including hypertension, hyperlipidaemia, dilated cardiomyopathy, systemic lupus erythematosus and chronic kidney disease. She had been admitted to the department of nephrology due to acute renal impairment, presenting with flank pain and oliguria. At the initial examination, her best‐corrected visual acuity was 0.5 in the right eye and 0.4 in the left eye. The Ishihara colour vision test revealed 3/17 and 1/17 in the right and left eye, respectively. Optical coherence tomography showed blurring and thickening of the ellipsoid zone in both eyes. Electroretinography demonstrated decreased cone cell function. She had been taking digoxin 0.125 mg for dilated cardiomyopathy for the past 2 years. Following the onset of acute renal impairment, her serum digoxin level was found to be 2.95 ng/mL, more than twice the normal range [0.6–1.2]. Suspecting digoxin intoxication, we consulted with her cardiologist and decided to discontinue digoxin. 3 weeks after discontinuing the medication, she reported improvement in the whitening of central visual field. Her best‐corrected visual acuity improved to 0.7 in the right eye and 0.6 in the left eye, and the Ishihara colour vision test showed 16/17 in both eyes. OCT demonstrated normalization of the ellipsoid zone thickness, and ERG showed ongoing recovery of cone cell function.Conclusions: Digoxin toxicity is known to result in visual disturbances—including decreased visual acuity, blurry vision and central scotoma with altered colour perception—caused by direct dysfunction of photoreceptors. Symptoms are gradually recovered within days to months after discontinuation of the medication. When facing visual problems in a patient with comorbidities, it is important to elicit a complete history, with all drug and detailed complaints, and to include a drug adverse effect. If a drug intoxication was suspected, medical consultations should be obtained.

When atypical becomes urgent: Unleashing the ophthalmologist's role in managing unconventional macroadenomas

Aims/Purpose: To present an atypical macroadenoma case and its management, in which the visual monitoring was key to indicate surgical intervention.Methods: Case report.Results: A 55‐year‐old male who was admitted to our emergency department with a main complaint of bilateral central and painless visual loss of 2 days' duration. He had DM type II. BCVA OU was 0.2, with no RAPD, but bilateral dyschromatopsia (OD: 4/16 OS: 10/16). Computerized visual field testing revealed a central scotoma (OD) and temporal hemifield loss (OS). An urgent CT scan revealed the presence of a 24 × 15 × 3 mm sellar‐suprasellar mass, with a locoregional mass effect and possible extension to cavernous sinuses and optic chiasm compression. 12 h after admission, deterioration of BCVA was observed (OD: 0.05 and OS: 0.1), along with worsening of dyschromatopsia (OD: 2/16 and OS: 7/16) and campimetric deficit progression (left homonymous hemianopic pattern). The MRI revealed the presence of a 21 × 21 × 36 mm sellar mass, without signs of recent intratumoral bleeding, with solid suprasellar extension, posterior cystic degeneration, compressing optic nerve, right optic tract, optic chiasm, and invading right cavernous sinus. Due to the worsening visual function, a consensus decision was made to proceed with same‐day endoscopic transsphenoidal resection. 24 h post‐surgery, the patient showed improvement in BCVA (1.0 OU) and dyschromatopsia (OD: 10/16 and OS: 12/16).Conclusions: In our case, despite unclear compressive effect on neuroimaging, careful monitoring of visual function allowed to timely indicate surgery, as the positive outcomes reflect. Managing this pathology in tertiary hospitals goes beyond the required experience of the neurosurgical team. The time for surgery indication is a big challenge due to the lack of clear definitions to indicate surgical intervention: “impending vision loss” or “clinically significant increase in size near the visual pathway.”

Long‐term response to idebenone treatment in a patient with advanced Leber hereditary optic neuropathy

Aims/Purpose: To present a case with advanced LHON and its response to 2‐year‐treatment with idebenone, which was prescribed 13 years after the onset of the disease.Methods: A 40‐year‐old Caucasian male patient with a history of bilateral painless visual loss since the age of 28 was examined at our Ophthalmology department. His brother had also suffered similar visual loss. At presentation ETDRS best corrected visual acuity (BCVA) was counting fingers (CF) in both eyes (BE). Fundoscopy revealed pale optic discs bilaterally. Visual fields (30‐2) showed central scotomas in BE. During OCT/OCTA imaging of the optic disc RNFL and GCC thickness were decreased bilaterally. In electrophysiological assessment with flash visual evoked potentials a reduction in amplitude was recorded in BE but with normal latency. Full‐field ERG was within normal limits in BE. At the genetic test, a mitochondrial point mutation m. 3460 G&gt;A at MT‐ND1 gene was detected, which was compatible with LHON. The patient was prescribed 300 mg idebenone per os three times/day for 24 months. Follow‐up visits were scheduled at 6, 12, 18 and 24 months.Results: At 6‐ and 12‐month follow‐up visits visual acuity, fundoscopic findings and OCT‐RNFL imaging remained unchanged. However, the patient reported subjective visual improvement bilaterally, with greater improvement in the visual field of the left eye. At 18 months following initiation of treatment an improvement of the visual field was documented in BE. No change of the GCC thickness was noted. At 2‐year follow‐up his condition remained improved.Conclusions: Delayed treatment with idebenone in advanced LHON can lead to subjective visual improvement. Moreover, an improvement in visual fields can also be documented even after 18 to 24 months of the initiation of the treatment.

Intraoperative Iridectomy in Femto-Laser Assisted Smaller-Incision New Generation Implantable Miniature Telescope.

In this study, we aimed to report the short-term (6 months) effects on visual functionality and safety of femto-laser assisted smaller-incision new-generation implantable miniature telescope (SING-IMT™) implanting, particularly related to postsurgical intraocular pressure increase, in patients suffering from end-stage age-related macular degeneration (AMD) and cataract. This device, designed for monocular use, aims to minimise the impact of the central scotoma by projecting the images onto a larger area of the photoreceptors surrounding the macula. In this prospective multicentric observational case series study, 6 eyes of 6 patients who underwent SING-IMT™ implantations were enrolled. At baseline and 6 months follow-up, best corrected distance visual acuity (BCDV) and best corrected near visual acuity (BCNVA), intraocular pressure (IOP), anterior chamber depth, endothelial cells count were assessed. In addition, IOP was also measured at 7, 15, 30, 45 days, and at 3 months follow-up. Finally, the incidence of complications was evaluated. At final follow-up, in the study eyes, mean BCDVA improved by +10.0 letters (6.25; 13.8) letters and mean BCNVA improved by -0.30 logMAR (-0.55; -0.20). At postoperative month 6, we reported a mean IOP decrease of 4.50 mmHg (-5.75; -0.25). Interestingly, 83.3% of patients had an increased IOP value in at least one of the first two postoperative follow-ups (7 days and 15 days). In patients in whom intraoperative mechanical iridotomy was not performed, it was necessary to perform a postoperative YAG laser iridotomy to improve IOP management. Compared to the baseline, ECD loss at 6 months follow-up was 12.6%. The SING IMT™ device was found to be effective in the distance and near vision improvement, without serious postoperative complications. We recommend intraoperative mechanical iridectomy in order to easily manage post-operative IOP and to avoid sudden IOP rise with its possible consequences. These good results can be a hope to partially improve the quality of life of patients suffering from severe end stage macular atrophy.

Correlation between Residual Sensitivity in the Central Inferior Nasal Visual Field and Visual Function in Chronic Leber Hereditary Optic Neuropathy Patients

Introduction: Leber hereditary optic neuropathy (LHON) is a maternally inherited, acute or subacute, optic neuropathy. The typical symptoms include reduced visual acuity and central scotoma. Despite the presence of deep central scotoma, some patients with LHON are able to perform daily activities. This study aimed to investigate the correlation between the residual visual field and visual acuity, critical flicker frequency, and fixation ellipse in patients with chronic LHON. Methods: Residual visual function (defined as sensitivity points where patients sensed the size V stimulus) of both eyes was evaluated in 10 patients with LHON carrying the m.11778 mitochondrial DNA mutation and with median age of onset and disease duration of 29 and 16.5 years, respectively. The central visual field was measured as static perimetry using the Humphrey visual field testing 30-2 program with the size III or V stimulus. Moreover, best-corrected visual acuity, critical flicker frequency, and the correlation between fixation ellipse and residual central visual fields were determined. The analysis was performed through a linear mixed-effects model. Results: The residual visual sensitivity in the inferior nasal visual field was significantly correlated with the logMAR (p < 0.05). The fixation ellipse fell within the residual visual field region with higher sensitivity. Conclusions: Patients with chronic LHON tended to retain the sensitivity detectable with the size V stimulus at the central inferior nasal visual field regions, where the fixation ellipse fell. Visual acuity, which influences daily activity, was spatially correlated with residual visual sensitivity.

Saccadic re-referencing training with gaze-contingent FRL-'fixation': Effects of scotoma type and size adaptation

Foveal vision loss makes the fovea as saccadic reference point maladaptive. Training programs have been proposed that shift the saccadic reference point from the fovea to an extrafoveal location, just outside the area of vision loss. We used a visual search task to train normal-sighted participants to fixate target items with a predetermined 'forced retinal location' (FRL) adjacent to a simulated central scotoma. We found that training was comparatively successful for scotomata that had either a sharp or blurry demarcation from the background. Completing the task with sharp-edged scotoma resulted in overall higher training gains. Training with blurry-edged scotoma, however, yielded overall better results when scotoma size was increased after training and participants needed to adapt to a more eccentric FRL, as may be necessary in patients with progressive degenerative eye diseases.

Caronia Glaucoma Card Versus Amsler Grid for Monitoring Patients With Advanced Glaucoma.

Caronia Glaucoma Card (CGC) design incorporates a vertical and horizontal line and seven concentric circles corresponding to 1 o arc at normal reading distance. The study showed that the CGC is preferred by study patients compared to Amsler Grid and can detect central scotomas. Evaluate patient acceptance and efficacy of the Caronia Glaucoma Card (CGC) in monitoring patients with severe glaucoma. A retrospective study of consecutive patients with severe glaucoma who agreed to monitor their visual function. Patients were given a two-sided sheet of paper. An Amsler grid was printed on one side, and a CGC was printed on the other side. The patient was given verbal and written instructions on how to perform each test. They were asked to use either one or both tests to monitor their visual function on a weekly basis. Upon return, they were asked which test they preferred and if they appreciated or noticed any change in their scotoma. Patient comments were recorded. Sixty-seven patients were given the test card whose reported past ocular history consisted solely of severe glaucoma. Seventeen patients were eventually excluded. Fourteen patients had 1 eye that fulfilled the inclusion criteria for the study, while the other eye was excluded. Eighty-six eyes of fifty patients satisfied the inclusion criteria and were enrolled in the study. Thirty of the patients were male (60%) and 20 were female (40%). The average age was 71.8 +/- 10.7 years. They all carried a diagnosis of severe glaucoma. When asked the question which test design they preferred to use, 30 patients preferred the CGC (60%), while 8 patients preferred the Amsler grid (16%). Twelve patients found no difference between the 2 test designs (24%). [χ 2 (2, 50)=16.480, ρ=0.000]. Five patients noticed a change in their scotoma while using the card (5.8%, 5 of 86 eyes). All were exclusively using the CGC. The CGC is a convenient and inexpensive tool that was able to detect a change in scotoma. It may be helpful to add to the armamentarium to monitor glaucoma and its possible progression in patients with advanced disease.