High diagnostic accuracy of T2FLAIR at 3 T in the detection of optic nerve head edema in acute optic neuritis.

OBJECTIVES: Optic nerve head edema (ONHE) detected by fundoscopy is observed in one-third of patients presenting optic neuritis (ON). While ONHE is an important semiological feature, the correlation between ONHE and optic nerve head MRI abnormalities (ONHMA), sometimes called "optic nerve head swelling," remains unknown. Our study aimed to assess the diagnostic accuracy of T2 fluid-attenuated inversion recovery (FLAIR) MRI sequence in detecting ONHE in patients with acute ON. METHODS: In the present single-center study, data were extracted from two prospective cohort studies that consecutively included adults with a first episode of acute ON treated between 2015 and 2020. Two experienced readers blinded to study data independently analyzed imaging. A senior neuroradiologist resolved any discrepancies. The primary judgment criterion of ONHMA was assessed as optic nerve head high signal intensity on gadolinium-enhanced T2FLAIR MRI sequence. Its diagnostic accuracy was evaluated with both the gold standard of ONHE on fundus photography (FP) and peripapillary retinal nerve fiber layer thickening on optic coherence tomography (OCT). RESULTS: A total of 102 patients were included, providing 110 affected and 94 unaffected optic nerves. Agreement was high between the different modalities: 92% between MRI and FP (k = 0.77, 95% CI: 0.67-0.88) and 93% between MRI and OCT (k = 0.77, 95% CI: 0.67-0.87). MRI sensitivity was 0.84 (95% CI: 0.70-0.93) and specificity was 0.94 (95% CI: 0.89-0.97) when compared with the FP. CONCLUSION: Optic nerve head high T2FLAIR signal intensity corresponds indeed to the optic nerve head edema diagnosed by the ophthalmologists. MRI is a sensitive tool for detecting ONHE in patients presenting acute ON. CLINICAL RELEVANCE STATEMENT: In patients with optic neuritis the high T2FLAIR (fluid-attenuated inversion recovery) signal intensity of the optic nerve head corresponds indeed to optic nerve head edema, which is a useful feature in optic neuritis etiological evaluation and treatment. KEY POINTS: Optic nerve head edema is a prominent clinical feature of acute optic neuritis and is usually diagnosed during dilated or non-dilated eye fundus examination. Agreement was high between magnetic resonance imaging, fundus photography, and optical coherence tomography. Optic nerve head high T2 fluid attenuation inversion recovery signal intensity is a promising detection tool for optic nerve head edema in patients presenting acute optic neuritis.

"En Face" Spectral-Domain Optical Coherence Tomography versus Multifocal Electroretinogram in Hydroxychloroquine Retinopathy Screening.

INTRODUCTION: The performance of "en face" optical coherence tomography (OCT) in screening for chloroquine (CQ) or hydroxychloroquine (HCQ) retinopathy has not been largely explored. The aim of this study was to determine the concordance of "en face" OCT with multifocal electroretinography (mfERG) in screening for CQ/HCQ retinopathy. METHODS: This is a prospective cohort study conducted at the Rothschild Foundation Hospital, Paris, between August 2016 and February 2021. Patients taking HCQ were followed up over 2 consecutive years and received an "en face" OCT and a mfERG on each visit. RESULTS: A total of 91 patients (182 eyes) were analyzed. mfERG and "en face" OCT were concordant in 147 eyes (86.3%). Cohen's kappa coefficient for concordance between mfERG and "en face" OCT was considered weak with a value 0.61 (95% CI: 0.50-0.72). The sensitivity and specificity of "en face" OCT were 70% (95% CI: 59-79%) and 91% (95% CI: 83-96%), respectively, relatively to mfERG. Proportion of abnormal R2/R5 and R3/R5 ratios did not differ between patients with normal and abnormal "en face" OCT (p = 0.2). DISCUSSION: "En face" OCT and mfERG have low concordance and cannot be used interchangeably as each investigation evaluates a different facet of CQ/HCQ retinopathy. "En face" OCT could be used as a complement in screening for CQ/HCQ retinal toxicity if the anomalies detected on "en face" OCT are confirmed by B-scan OCT sections.

Prospective longitudinal study on prognostic factors of visual recovery and structural change after a first episode of optic neuritis.

BACKGROUND AND PURPOSE: This study was undertaken to determine the role of optical coherence tomography (OCT) in predicting the final visual and structural outcome, and to evaluate the correlation between functional eye outcome and retinal changes, in patients with a first episode of optic neuritis (ON). METHODS: In this prospective study, consecutive adult patients with acute ON underwent ophthalmological evaluation at baseline and at 1 and 12 months, including OCT measurements of peripapillary retinal nerve fiber layer (pRNFL), macular ganglion cell and inner plexiform layer, and inner nuclear layer thicknesses; high- and low-contrast visual acuity; visual field assessment; and baseline brain magnetic resonance imaging. Univariate and multivariate linear regressions were used to assess predictive factors of outcome. Correlations between 12-month visual function and retinal structure were estimated by Spearman coefficients. Two groups of patients were analyzed, with or without multiple sclerosis (MS). RESULTS: Among 116 patients, 79 (68.1%) had MS, and 37 (31.9%) had ON not related to MS (including 19 idiopathic [i.e., isolated] ON, and 13 and five with myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies, respectively). We found no independent predictive factor of visual and retinal outcome. Analysis of the relationship between the visual field test (mean deviation) and pRNFL thickness demonstrated a threshold of 75.4 µm and 66.4 µm, below which the mean deviation was worse, for patients with MS (p = 0.007) and without MS (p < 0.001), respectively. CONCLUSIONS: We found that inner retinal layer measurements during the first month are not predictive of final outcome. The critical threshold of axonal integrity, below which visual function is damaged, is different between patients with and without MS.

Visual field loss and structure-function relationships in optic neuritis associated with myelin oligodendrocyte glycoprotein antibody.

BACKGROUND: A paradoxical discrepancy between severe peripapillary retinal nerve fiber layer (pRNFL) atrophy and good visual outcome had been reported in patients with myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG)-associated optic neuritis (ON). However, only visual acuity (VA) was assessed. OBJECTIVES: To study visual field (VF) outcomes of patients with MOG-IgG-associated ON and evaluate the correlation between functional eye outcome and retinal structural changes assessed by optical coherence tomography. METHODS: The records of 32 patients with MOG-IgG-associated ON who underwent ophthalmological examination at least 12 months after ON onset were reviewed. Degree of VF disability was determined by mean deviation (MD). RESULTS: At final assessment (median, 35 months), 4.2% of 48 affected eyes (AE) had VA ⩽ 0.1, 40% had abnormal MD, and among AE with final VA ⩾ 1.0, 31% had mild to moderate damage. Thinning of the inner retinal layers was significantly correlated with MD impairment. Analysis demonstrated a threshold of pRNFL thickness (50 µm), below which MD was significantly worse (mean, -2.27 dB vs -17.72 dB; p = 0.0003). ON relapse was significantly associated with poor visual outcome assessed by MD. CONCLUSION: Functional impairment measured with VF is not rare, and MD assessment better reflects actual structural damage.

Ten-year outcomes of anti-vascular endothelial growth factor treatment for neovascular age-related macular disease: A single-centre French study.

IMPORTANCE: Long-term data of intravitreal injections of vascular endothelial growth factor (VEGF) inhibitors are lacking. BACKGROUND: This study aims to assess visual and anatomic outcomes of eyes with neovascular age-related macular degeneration (nAMD) after 10 years of anti-VEGF therapy. DESIGN: Retrospective analysis of data from a prospectively designed database. PARTICIPANTS: One hundred and sixteen eyes with nAMD (94 participants) that started anti-VEGF therapy at least 10 years earlier. METHODS: Eyes were tracked by the Fight Retinal Blindness! registry. MAIN OUTCOME MEASURES: Mean change in visual acuity at 10 years vs baseline. Visual acuity was assessed by the number of letters read on a logarithm of the minimum angle of resolution chart. RESULTS: Eyes received a median of 27.5 injections over 10 years. Mean visual acuity was 57.5 letters (SD 17.5) at baseline. It increased slightly at 1 year, then dropped steadily by 18 letters (95% CI: 13.7; 22.3) at 10 years. Overall, 10% of eyes gained ≥10 letters, 64% lost ≥10 letters and 23% remained stable (±5 letters from baseline). Geographic atrophy and subretinal fibrosis were found in 93% and 71%, respectively, after 10 years, both mostly affecting the centre of the fovea. Pre-treated eyes (47.5%) had significantly worse visual acuity than treatment-naïve eyes at baseline and during follow-up and were significantly more likely to have atrophy and fibrosis. CONCLUSIONS AND RELEVANCE: Despite short-term stabilization, long-term visual outcomes of nAMD eyes under anti-VEGF therapy may be poor. Development of atrophy and fibrosis, resulting from the natural progression of the disease, may partly explain this evolution.

Comparison between multifocal ERG and C-Scan SD-OCT ("en face" OCT) in patients with a suspicion of antimalarial retinal toxicity: preliminary results.

PURPOSE: Pericentral visual field changes and disruption of the ellipsoid layer on spectral domain optical coherence tomography (SD-OCT) are the main features of antimalarial retinal toxicity. C-Scan OCT or "en face" enables a topographic frontal view of the changes observed within the different retinal layers in particular the ellipsoid layer. The aim of this prospective study was to compare multifocal ERG (mfERG) responses with the results of C-Scan OCT ("en face" OCT) in patients with abnormal visual field and to analyze relationships between the structural and functional abnormalities. METHODS: In 354 consecutive patients screened for antimalarial toxicity between January 1, 2014 and December 31, 2016, central visual field, mfERG recording, C-Scan OCT and short-wavelength fundus autofluorescent imaging were performed. RESULTS: Among the 17/354 patients with abnormal central visual field results, all presented with abnormalities on the mfERG at least in one eye. In 16/33 eyes, there was a good concordance between focal loss of the mfERG response and the disruption of the ellipsoid layer on C-Scan OCT. In one eye with characteristic changes in the ellipsoid layer on the C-Scan OCT, the mfERG was normal, whereas in three eyes the mfERG was abnormal in eyes with a normal C-Scan OCT. CONCLUSIONS: The contribution of the C-Scan OCT changes remains difficult to establish as there is no strict concordance with the local ERG responses. Although C-Scan OCT technology provides a new approach in analyzing focal abnormalities in the photoreceptor-retinal pigment epithelium interface, the sensitivity of this method compared with mfERG and other tests (central visual field, B-Scan OCT) needs to be evaluated. This study is still ongoing on a larger cohort.

Aflibercept Treatment in Polypoidal Choroidal Vasculopathy: Results of a Prospective Study in a Caucasian Population.

INTRODUCTION: Polypoidal choroidal vasculopathy (PCV) is a choroidal pathology characterized by frequent occurrences of subretinal hemorrhages and resistance to monotherapies such as ranibizumab or bevacizumab intravitreal injections (IVT). The purpose of this study is to evaluate both the anatomical and functional efficacy of aflibercept IVT as a monotherapy in PCV in a Caucasian population. METHODS: We conducted a prospective multicenter study in either treatment-naïve patients with PCV or PVC patients who had not been treated with anti-VEGF within the previous 3 months or with photodynamic therapy (PDT) within the previous 6 months. All patients had been treated with 3 initial monthly loading doses of aflibercept followed by a Q8 regimen for 28 weeks in total. All patients underwent a complete ophthalmic examination including the measurement of best-corrected visual acuity (BCVA) before each IVT and after 28 weeks as well as an optical coherent tomography (OCT) of the macula. At baseline and 28 weeks, the polypoidal dilations were analyzed with indocyanine green angiography. RESULTS: Thirty-four eyes of 34 patients were included in this study. All patients were followed for 28 weeks and received 5 aflibercept IVT. The mean baseline BCVA was 55 letters. After 28 weeks, significant +13 letters in BCVA and a regression of exudative signs on OCT in all patients were observed. In 62% of the cases, polyp disappearance was observed on indocyanine green angiography. DISCUSSION: In this study on a Caucasian population, we showed that aflibercept as a monotherapy provided both a significant visual gain and the regression of polypoidal dilations. Aflibercept use in monotherapy may contribute to reduce the hemorrhagic risk and atrophy linked to PDT.

"En face" optical coherence tomography imaging in type 2 idiopathic macular telangiectasia.

PURPOSE: To comprehensively evaluate the retinal and choroidal changes in eyes with Type 2 idiopathic macular telangiectasia using "en face" and B-scan spectral domain optical coherence tomography (OCT), and to compare their respective contributions to this evaluation. METHODS: Eyes with a diagnosis of proliferative or nonproliferative Type 2 macular telangiectasia were prospectively studied. All patients underwent an extensive ophthalmologic examination including biomicroscopic fundus examination, color photography, fundus autofluorescence, fluorescein angiography, B-scan and en face spectral domain OCT. RESULTS: Twenty eyes of 10 patients were included in this study. En face OCT C-scans and conventional B-scans were both able to show inner crystalline deposits (15%), retinal capillary anomalies (100%), intraretinal cysts (80%), hyperreflective spots in the outer nuclear layer (100%) and external limiting membrane (80%), hyperplastic pigment plaques (30%), intraretinal neovascularization (20%), photoreceptor loss (100%), and choroidal cavitations (30%). En face OCT C-scans provided more information than B-scans on intraretinal neovascularization, photoreceptor loss, and choroidal cavitations. Also, en face OCT C-scans provided better visualization of the retinal vessels and telangiectasia than fluorescein angiography. CONCLUSION: En face OCT is a noninvasive and reproducible technique that helps to better assess and follow up retinal and choroidal processes in Type 2 macular telangiectasia.

Efficacy and Safety of 0.19-mg Fluocinolone Acetonide Implant in Postoperative Cystoid Macular Edema after Pars Plana Vitrectomy: The ILUvien in Postoperative CYstoid Macular eDema study.

PURPOSE: To assess the efficacy and safety of 0.19-mg fluocinolone acetonide (FAc) intravitreal implant (Iluvien) in treating chronic postoperative cystoid macular edema (PCME) after pars plana vitrectomy. DESIGN: Retrospective multicentric case series in clinical settings. SUBJECTS: Patients with chronic PCME who underwent vitrectomy in tertiary care centers in France. METHODS: Review of charts and OCT scans. MAIN OUTCOME MEASURES: The primary end points were the best-corrected visual acuity (BCVA) and central retinal thickness (CRT). Secondary end points were the intraocular pressure (IOP); proportion of patients maintaining a BCVA ≥20/40; need for additional nonstudy treatment; differences between eyes that underwent a single and multiple surgeries; and OCT biomarkers of better BCVA. RESULTS: Forty-nine eyes of 49 patients with a mean follow-up of 24.5 ± 3.87 months were included. The mean BCVA increased from 0.40 ± 0.26 logarithm of the minimum angle of resolution (logMAR) at baseline to 0.32 ± 0.24 logMAR at month 24 (P = 0.0035). The mean CRT decreased from 409 ± 139 µm at baseline to 340 ± 92 µm at month 24 (P = 0.0001). The mean IOP was 14.0 ± 4 mmHg at baseline and remained stable at 14.03 ± 4.1 mmHg at month 24 (P = 0.99). During the follow-up, the IOP exceeded 21 mmHg in 9 eyes, with one eye requiring cyclophotocoagulation. The BCVA was ≥20/40 in 47% of eyes (95% confidence interval [CI], 34%-61%) at baseline and in 58% of eyes at month 24 (95% CI, 41%-73%). At month 18, the likelihood of achieving a BCVA ≥20/40 was higher in eyes with intact external limiting membrane and ellipsoid zone. Additional dexamethasone (DEX) implant was injected in 14 eyes (28.6%). The treatment burden of 2.45 ± 1.35 DEX implant/y was decreased to 0.57 ± 0.60 DEX implant/y after FAc implantation (P = 0.001). CONCLUSIONS: Fluocinolone acetonide implant improved the BCVA, reduced the CRT, and allowed reducing treatment burden in eyes with chronic PCME after vitrectomy. The safety profile was acceptable. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Retinal inner nuclear layer microcystic changes in optic nerve atrophy: a novel spectral-domain OCT finding.

PURPOSE: Optic atrophy constitutes the final stage in the evolution of optic neuropathy. The aim of this study was to describe the presence of macular microcystic changes or pseudocysts in patients with advanced optic atrophy. METHODS: The medical records of 24 patients who had retinal pseudocysts in association with optic atrophy have been analyzed. All patients underwent a complete neuro-ophthalmologic assessment; peripapillary retinal nerve fiber layer thickness and macular screening with spectral-domain optical coherence tomography and optical coherence tomography "en face" imaging analysis were also performed. RESULTS: A total of 36 eyes were included in the study. Patients' mean age was 37 years. The major cause of optic atrophy was glaucoma (12 cases). The retinal pseudocysts were observed as hyporeflective lesions in the internal nuclear layer. Infrared images revealed a hyporeflective circular or semilunar shape corresponding to the location of the pseudocysts in all cases. In eyes with pseudocysts, mean thickness of the peripapillary retinal nerve fiber layer was statistically significantly less than that of fellow eyes (P = 0.0003), whereas macular thickness was statistically significantly higher compared with fellow eyes (P < 0.005). CONCLUSION: The presence of pseudocystic lesions always associated with severe optic nerve fiber loss is reported. The reason why pseudocystic lesions develop within the retina is not well understood. They might constitute the translation of degeneration of Muller cells in severe optic nerve fiber loss. Recognizing these pseudocysts is crucial because they may be confused with cystoid macular edema. Their prognostic value and role in the therapeutic process need to be further evaluated with prospective studies and molecular experiments in vivo.

Atypical Foveal Hypoplasia in Best Disease.

PURPOSE: To determine the prevalence and characteristics of foveal hypoplasia (also called fovea plana) in patients with Best disease using spectral-domain (SD) optical coherence tomography (OCT) and OCT-angiography (OCT-A). DESIGN: A retrospective observational study including patients diagnosed with Best disease. SUBJECTS AND PARTICIPANTS: Fifty-nine eyes of thirty-two patients (fifteen females (46.9%) and seventeen males (53.1%), p = 0.9) diagnosed with Best disease were included. Patients' eyes were categorized into two groups: Eyes with a fovea plana appearance ('FP group') and eyes without fovea plana appearance ('no FP group'), based on the foveal appearance on B-scan SD-OCT. METHODS AND MAIN OUTCOME MEASURES: Cross-sectional OCT images were assessed for the persistence of inner retinal layers (IRL) and OCT-A was analyzed for the presence of a foveal avascular zone (FAZ), the size of which was determined when applicable. RESULTS: Overall, 16 eyes (27.1%) of 9 patients had a fovea plana appearance ('FP group') with the persistence of IRL, and 43 eyes (72.9%) of 23 patients did not have fovea plana appearance ('no FP group'). Among FP eyes, OCT-A performed in 13 eyes showed bridging vessels through the FAZ in 100% of eyes with OCT-A. Using Thomas classification, 14 out of the 16 eyes with fovea plana (87.5%) had atypical foveal hypoplasia, and the 2 others (12.5%) had a grade 1b fovea plana. CONCLUSION: In our series, foveal hypoplasia was present in 27.1% of patients with Best disease. OCT-A showed bridging vessels through the FAZ in all eyes. These findings highlight the microvascular changes associated with Best disease, which can be an early sign of the disease in patients with a family history.

Outcome of Patients Treated by Intravitreal Injections During the COVID-19 Lockdown: An Institutional Experience.

PURPOSE: To determine the loss of follow-up ratio and reasons during the COVID-19 lockdown in patients with retinal diseases treated by anti-vascular endothelial growth factor intravitreal injections and to report the visual outcome and rate of complications of these patients 1 year after the end of the lockdown. METHODS: This is a prospective descriptive cohort study (NCT04395859) conducted at the Rothschild Foundation Hospital - Paris between April 2020 and May 2021. Patients with retinal diseases treated by repeated intravitreal anti-VEGF injections (IVI) since before October 2019 were included. They filled-out a questionnaire and were followed up during a period of 1 year. RESULTS: During the COVID-19 lockdown 198 eyes (82.5%) of 157 patients (82.6%) received their injections in a timely manner (group 1) while 42 eyes (17.5%) of 33 patients (17.4%) had their injections delayed or missed (group 2). No statistically significant difference was found between group 1 and group 2 when comparing the change of mean best corrected distance visual acuity (BCVA) between month 12 and inclusion (p = 0.6) and the rate of ocular complications. The most frequent reasons for missing scheduled injections are appointments cancellation by the hospital (12 patients, 36%), fear of virus exposure during transportation (7 patients, 21%) or at the hospital (5 patients, 15%). Eighty-four percent (130/157 patients) of patients who attended their appointment were satisfied by the protective measures used in the hospital. CONCLUSION: COVID-19 lockdown did not seem to negatively affect the 1-year outcome of patients with retinal diseases treated by anti-VEGF IVIs who missed their scheduled injections. The BCVA and rate of complications at 1 year did not differ whether patients missed their scheduled injections or not. Maintaining IVIs during lockdown periods and educating patients about the risks of missing injections are pivotal in improving prognosis of retinal diseases.

Acute optic neuritis: What are the clues to the aetiological diagnosis in real life?

BACKGROUND: Previous cross-sectional studies have reported distinct clinical and radiological features among the different acute optic neuritis (ON) aetiologies. Nevertheless, these reports often included the same number of patients in each group, not taking into account the disparity in frequencies of ON aetiologies in a real-life setting and thus, it remains unclear what are the truly useful features for distinguishing the different ON causes. To determine whether clinical evaluation, ophthalmological assessment including the optical coherence tomography (OCT), CSF analysis, and MRI imaging may help to discriminate the different causes of acute ON in a real-life cohort. METHODS: In this prospective monocentric study, adult patients with recent acute ON (<1 month) underwent evaluation at baseline and 1 and 12 months, including, high- and low-contrast visual acuity, visual field assessment and OCT measurements, baseline CSF analysis and MRI. RESULTS: Among 108 patients, 71 (65.7%) had multiple sclerosis (MS), 19 (17.6%) had idiopathic ON, 13 (12.0%) and 5 (4.6%) had myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies, at last follow up respectively.At baseline, the distribution of bilateral ON, CSF-restricted oligoclonal bands, optic perineuritis, optic nerve length lesions and positive dissemination in space and dissemination in time criteria on MRI were significantly different between the four groups (p <0.001). No significant difference in visual acuity nor inner retinal layer thickness was found between the different ON aetiologies. CONCLUSIONS: In this large prospective study, bilateral visual involvement, CSF and MRI results are the most useful clues in distinguishing the different aetiologies of acute ON, whereas ophthalmological assessments including OCT measurements revealed no significant difference between the aetiologies.

One-year outcome of neovascular age-related macular degeneration patients followed-up using different optical coherence tomography modalities.

PURPOSE: The aim of this study is to compare the one year outcome of neovascular age-related macular degeneration (nAMD) patients treated by a PRN regimen of Anti-vascular endothelial growth factor (VEGF) intravitreal injections (IVTs), using optical coherence tomography B-scan (OCT-B) or OCT Angiography (OCT-A) imaging modalities during follow-up. METHODS: Patients older than 50 years with nAMD currently treated by PRN regimen of Anti-VEGF IVTs were recruited from Rothschild Foundation Hospital - Paris and Centre Ophtalmologique Maison Rouge - Strasbourg and followed-up for a year. Patients were randomized in two groups: one group was followed by OCT-B while the other was followed by OCT-A. RESULTS: Thirty-three patients were followed by OCT-A and 31 patients were followed by OCT-B. Twenty-nine patients in the OCT-A group and 27 patients in the OCT-B group attended the last visit. No statistically significant difference was found between the two groups at 1 year concerning: improvement or stabilization of the best corrected distance visual acuity (BCVA) (p > 0.9), exudative signs (p > 0.9), number of injections (p = 0.8) and the delay until the first reinjection was performed (p = 0.5). CONCLUSION: The use of OCT-A or OCT-B as imaging modalities in nAMD treated by a PRN regimen of Anti-VEGF IVTs seem to be comparable at one year.

Eye Involvement in Wilson's Disease: A Review of the Literature.

Wilson's disease (WD) is an autosomal recessive genetic disorder due to a mutation of the ATP7B gene, resulting in impaired hepatic copper excretion and accumulation in various tissues. Ocular findings are one of the hallmarks of the disease. Many ophthalmological manifestations have been described and new techniques are currently available to improve their diagnosis and to follow their evolution. We have performed a systematic PubMed search to summarize available data of the recent literature on the most frequent ophthalmological disorders associated with WD, and to discuss the newest techniques used for their detection and follow-up during treatment. In total, 49 articles were retained for this review. The most common ocular findings seen in WD patients are Kayser-Fleischer ring (KFR) and sunflower cataracts. Other ocular manifestations may involve retinal tissue, visual systems and eye mobility. Diagnosis and follow-up under decoppering treatment of these ocular findings are generally easily performed with slit-lamp examination (SLE). However, new techniques are available for the precocious detection of ocular findings due to WD and may be of great value for non-experimented ophthalmologists and non-ophthalmologists practitioners. Among those techniques, anterior segment optical coherence tomography (AS-OCT) and Scheimpflug imaging are discussed.

Visual Recovery with Iloprost Added to Corticosteroids in a Case of Giant Cell Arteritis.

INTRODUCTION: To date, corticosteroids remain the cornerstone treatment of ocular involvement of GCA, and no other treatment has proven to be effective in this setting. We herein report on a unique case of GCA with ocular involvement worsening despite high dose corticosteroids and recovering with intravenous iloprost. CASE REPORT: A 70-year-old man presented with acute vision loss in his left eye related to anterior ischemic optic neuropathy. The diagnosis of giant-cell arteritis was confirmed by a temporal artery biopsy. Despite intravenous pulse methylprednisolone for 3 days then oral prednisone at 60 mg/day, the patient developed from day 5 fluctuating vision loss in the right eye, related to ocular ischemia by occlusion of the ophthalmic artery, and responsive to hyperhydration. Iloprost, an analog of prostacyclin PGI2, was then administered intravenously for 5 days and resulted in a stable improvement in visual acuity in the right eye. CONCLUSION: This case highlights the potential role of vasodilatator agents in giant cell arteritis with ocular involvement.

Retinal and choroidal blood flow variations after an endurance exercise: A real-life pilot study at the Paris Marathon.

OBJECTIVES: To collect information about the retinal blood flow variations and other choroidal and retinal parameters during a prolonged effort such as marathon running. DESIGN: Non-randomized prospective cohort study. METHODS: Patients were recruited through an information campaign at the Rothschild Foundation Hospital (Paris, France). A first visit (V1) was planned in the month before the marathon. All participants underwent blood pressure measurement, fundus photography, spectral domain-optical coherence tomography (SD-OCT) and OCT-angiography (OCT-A). A second visit (V2) was scheduled within one hour of crossing the finish line. The same tests were repeated, using the same equipment. RESULTS: Of the 31 runners who were included, 29 finished the marathon and attended V2. At baseline, various ophthalmological abnormalities were found in 45.2% of the 58 eyes, among which almost a third concerned the optic nerve and a quarter the pachychoroid spectrum. A significant decrease in retinal vascular plexus density was found between V1 and V2 (p<0.01). While median macular and retinal nerve fiber layer (RNFL) thicknesses significantly increased after the marathon (p<0.01), median choroidal thickness significantly decreased (p<0.01). Both systolic and diastolic blood pressures significantly decreased (p<0.01 and p=0.021 respectively). CONCLUSIONS: Prolonged physical effort impacts the structure and vascularization of the retina and the choroid. Hypoxia and dehydration due to such an effort may induce a low ocular blood flow rate resulting in a choroidal thinning, contrasting with a transient subclinical ischemic edema of the inner retina and optic nerve head. CLINICAL TRIAL REGISTRATION NUMBER: NCT03864380.

Light-induced modifications of the outer retinal hyperreflective layers on spectral-domain optical coherence tomography in humans: an experimental study.

PURPOSE: Numerous small hyperreflective dots (HRDs) can be seen within the hyporeflective layer between the ellipsoid zone (EZ) and the interdigitation zone (IZ) on C-scan spectral-domain optical coherence tomography (SD-OCT) with a yet unknown variation under light conditions. The aim of this study was to explore light-induced SD-OCT changes in these HRDs. METHODS: The study subjects were randomly assigned to two groups: Group 1 experienced a dark adaptation protocol followed by intense retinal photobleaching, while Group 2, serving as the control group, was exposed to constant ambient light without any variation. The number of HRDs was automatically counted. RESULTS: Twenty healthy volunteers were prospectively included. The number of HRDs differed significantly over time (p = 0.0013). They decreased in Group 1 after dark adaptation and retinal photobleaching before returning to baseline levels 30 min later; conversely, they remained relatively constant in Group 2 throughout the study (p < 0.001). Light-skinned subjects had less HRD than dark-skinned subjects. CONCLUSION: We observed light-induced modifications in the space between the EZ and the IZ. We hypothesize that the HRDs visible in this zone correspond to melanosomes that are mobilized during the light stimulation protocol. Larger studies are recommended to further evaluate and confirm light-induced SD-OCT changes under physiological and pathological conditions.

Study of choroidal thickness variations after ingestion of a taurine and caffeine-containing energy drink.

BACKGROUND & AIMS: Choroidal thickness can undergo considerable variations in response to different substances. The aim of this study was to assess the change in choroidal thickness after the ingestion of taurine and caffeine contained in the Red Bull energy drink. METHODS: Enhanced Depth Imaging - Spectral Domain Optical Coherence Tomography was used to measure subfoveal choroidal thickness (SFCT) variations in healthy volunteers at several time points after drinking a Red Bull can (25 cl). RESULTS: Forty eyes of 20 volunteers were enrolled. A significant reduction in SFCT (-14 µm, p < 0.0001) occurred at 1 h after the Red Bull intake, and was even more important (-20.14 µm, p < 0.0001) in eyes with particularly thick choroids (≥ 395 µm). SFCT measurements at 4 h were comparable to baseline. CONCLUSIONS: A concomitant ingestion of caffeine and taurine can induce a transient choroidal thinning that is more marked in eyes with thick choroids. REGISTRATION NUMBER OF CLINICAL TRIAL: NCT02856256.

COVID-19 Associated Choroidopathy.

The aim of the study is to report on the indocyanine green angiography (ICGA) and OCT findings in patients hospitalized for severe COVID infection. In this observational prospective monocentric cohort study, we included patients hospitalized for severe COVID infection. The main outcomes were ICGA and OCT findings. A total of 14 patients with a mean age of 58.2 ± 11.4 years and a male predominance (9/14 patients; 64%) were included. The main ICGA findings included hypofluorescent spots in 19 eyes (68%), intervortex shunts in 10 eyes (36%), and characteristic "hemangioma-like" lesions in five eyes (18%). "Hemangioma-like" lesions were both unique and unilateral, and showed no washout on the late phase of the angiogram. The main OCT findings included focal choroidal thickening in seven eyes (25%), caverns in six eyes (21%) and paracentral acute middle maculopathy lesions in one eye (4%). All patients hospitalized for severe COVID infection had anomalies on ICGA and OCT. Lesions to both retinal and choroidal vasculature were found. These anomalies could be secondary to vascular involvement related directly or indirectly to the SARS-CoV2 virus.