Journal of Neuro-Ophthalmology

Background Patients with severe traumatic optic neuropathy (TON) often show limited improvement in visual function despite therapy. Objective This study aims to investigate whether targeted shortwave diathermy (SWD) combined with perceptual training enhances visual function in patients with severe TON following endoscopic optic nerve decompression (EOND) surgery. Methods Forty patients with severe TON after EOND surgery were randomly assigned to either the rehabilitation (Reh) group ( n = 24) or the non-rehabilitation (Nreh) group ( n = 16). Subjects in the Reh group received targeted SWD therapy and perceptual training. Visual function, visual evoked potentials, and diffusion tensor imaging were evaluated. Results After 16 weeks of rehabilitation, the best-corrected visual acuity ( p = 0.006) and color vision scores ( p = 0.026) in the Reh group improved significantly more than those in the Nreh group. Two children who had completely lost their visual acuity 62 and 73 days after experiencing TON regained visual acuity scores of 1.0 logMAR or lower after rehabilitation. After 10 weeks of rehabilitation, the mean P100 amplitude ( p = 0.012) and fractional anisotropy (anterior segment, p = 0.032; middle segment, p = 0.014) of the affected nerves in the Reh group were significantly higher than those in the Nreh group. Conclusions Targeted SWD combined with perceptual training demonstrated beneficial effects in patients with severe TON after EOND surgery. Notably, this study provides the first evidence of visual function recovery occurring 73 days after complete vision loss in child with TON when combined rehabilitation was implemented.

Objective To evaluate changes in intraocular pressure (IOP) and mean ocular perfusion pressure (MOPP) following intravitreal anti-VEGF injections (IVI) and to assess influencing factors. Methods In a prospective study, 119 eyes received anti-VEGF IVI, with 60 eyes treated in the supine position and 59 eyes in the seated position. IOP was assessed using an iCare IC200 rebound tonometer before, immediately after, and at 5 and 30 min post-injection. MOPP was calculated at the same time points. Ocular biometric and demographic data were collected to analyze their impact on IOP changes. Results IOP increased from 15.76 ± 4.34 mmHg at baseline to 49.79 ± 15.73 immidiately post-IVI, then decreased to 29.96 ± 10.55 at 5 min and 21.80 ± 7.36 at 30 min. MOPP dropped from 54.02 ± 10.7 mmHg to 35.7 ± 19.7 mmHg immediately post-IVI, recovering to 40.68 ± 15.9 at 5 min and 51.08 ± 12.2 at 30 min. Supine patients exhibited higher IOP, but maintained higher MOPP compared to seated patients. Only 25% of glaucoma patients returned to baseline IOP within 30 min, compared to 48.9% of non-glaucomatous patients (p = 0.02). Phakic patients have greater IOP changes across time, with significant differences at 5 min ( p = 0.03). Conclusion IVI induced IOP increases and MOPP decreases are significant but normalized to baseline +2SD within 30 min in 67.77% and 97.52% of patients. Glaucomatous and phakic patients are at higher risk for prolonged IOP elevation, requiring careful management.

Purpose CLCN2 is a gene that encodes the voltage-gated chloride channel protein 2 in the human brain and eyes. While mutations in this gene have been associated with leukoencephalopathy as well as ocular manifestations including optic neuropathy and choroidopathy, here we report for the first time a case of severe proliferative retinopathy in a patient with CLCN2 mutation. Observations A 12-year-old girl with Familial Mediterranean Fever (FMF) was referred due to blurred vision in both eyes. Ophthalmoscopic examination revealed mild vitreous hemorrhage, large neovascularization at the disc (NVD), extensive neovascularization along the arcades (i.e., NVE; neovascularization elsewhere), retinal arteriolar narrowing, silver sheathing of the veins and preretinal hemorrhages. Fluorescein angiography (FA) demonstrated prominent leakage from multiple large NVEs. A thorough and multidisciplinary evaluation ruled out metabolic, thrombotic, infectious, inflammatory, or autoimmune etiologies. Notably, however, brain magnetic resonance imaging (MRI) revealed leukodystrophy yet poorly correlated with her clinical manifestation. Strikingly, whole exome sequencing uncovered a homozygous mutation in the CLCN2 gene. She was treated with intravitreal injections of the anti-vascular endothelial growth factor (VEGF) antibody Bevacizumab and laser pan-retinal photocoagulation (PRP) with partial response. Conclusions This case suggests that CLCN2 mutation may possess a key role in an aggressive form of proliferative retinopathy with partial response to anti-VEGF therapy. We hypothesize that the potential underlying mechanism involves astrocyte dysfunction and retinal blood barrier disruption. Noteworthy, a comprehensive approach is unequivocally significant in evaluating such cases of proliferative retinopathy with unclear etiology to establish a diagnosis and management strategy.

Introduction Myopia is a refractive error where distant objects are not clearly seen and appear blurred. Goal of this study was to evaluate long-term effectiveness of Repeated Low-Level Red Light (RLRL) therapy for children with myopia, which primarily focused on Axial length (AL) and Spherical Equivalent Refraction (SER) as primary endpoints. Methods The research compared outcomes between RLRL treatment with Single Vision Spectacles (SVS) for childhood myopia management. We performed a systematic literature search in the PubMed, Embase and Cochrane databases using “Myopia” and “ Repeated Low-Level Red Light “. Mean differences (MD) were estimated and the effects of therapies measured. Publication bias and heterogeneity analysis were carried out by Inverted Precision Effect Test-Precision Effect Estimate Standard Error (PET-PEESE) (and subsequent Search Sequential Analysis) and Tau test. Bayesian meta-analysis was performed using Jaffrey Amazing Statistical Package (JASP). Results This meta-analysis comprised 1,714 participants: 824 in RLRL and 890 in SVS group. Pooled effect size for AL reduction was 0.953 ± 0.294, (95% credible interval (CI) 0.775 to 0.980). Pooled effect size for SER reduction was 1.521 ± 0.662 (95% CI 0.102 to 2.736). PET-PEESE analysis revealed no significant publication bias ( p-value 0.407). Random effects models were employed for presence of significant heterogeneity (3.9 and 5.7 for AL; 5.7 for SER), alongside degree of variation (0.828 & 1.665) for Tau (τ), which estimates the between-study variance. Conclusion Long-term observations indicate that RLRL treatment significantly influences myopia management, leading to considerable reductions in both AL and SER. Additional research is essential to investigate potential long-term rebound effects.

Purpose To report a case highlighting the uncommon presentation of Frosted Branch Angiitis (FBA) in Behçet's Disease (BD), notably in a patient who received a COVID vaccine one month prior to the onset of symptoms. We explore the possible role of vaccination in unmasking BD. Methods A comprehensive evaluation of a 45-year-old patient with FBA post-COVID vaccination was undertaken. Results The patient exhibited characteristic features of FBA, with classic perivascular sheathing, optic disc edema, and retinal ischemia, which manifested one month after receiving the Pfizer/BioNTech COVID vaccine. Despite a history of recurrent oral aphthosis, systemic workup for autoimmune conditions was unremarkable. Diagnosis of presumptive Behçet's Disease was made based on clinical criteria. Treatment with intravenous methylprednisolone and infliximabn (5 mg/kg) led to complete resolution of ocular symptoms and retinal signs. Conclusion FBA is an uncommon presentation of BD. This case also serves as a stark reminder of the potential consequences of vaccination on immune-mediated diseases. However, the correlation between FBA onset, COVID vaccination, and Behçet's Disease diagnosis necessitates cautious interpretation. Successful use of methylprednisolone and infliximab in achieving resolution of visual symptoms highlights potential therapeutic considerations in similar cases.

Purpose To evaluate the appropriateness and readability of the responses generated by ChatGPT-4 and Bing Chat to frequently asked questions about glaucoma. Method Thirty-four questions were generated for this study. Each question was directed three times to a fresh ChatGPT-4 and Bing Chat interface. The obtained responses were categorised by two glaucoma specialists in terms of their appropriateness. Accuracy of the responses was evaluated using the Structure of the Observed Learning Outcome (SOLO) taxonomy. Readability of the responses was assessed using Flesch Reading Ease (FRE), Flesch Kincaid Grade Level (FKGL), Coleman-Liau Index (CLI), Simple Measure of Gobbledygook (SMOG), and Gunning- Fog Index (GFI). Results The percentage of appropriate responses was 88.2% (30/34) and 79.2% (27/34) in ChatGPT-4 and Bing Chat, respectively. Both the ChatGPT-4 and Bing Chat interfaces provided at least one inappropriate response to 1 of the 34 questions. The SOLO test results for ChatGPT-3.5 and Bing Chat were 3.86 ± 0.41 and 3.70 ± 0.52, respectively. No statistically significant difference in performance was observed between both LLMs ( p = 0.101). The mean count of words used when generating responses was 316.5 (± 85.1) and 61.6 (± 25.8) in ChatGPT-4 and Bing Chat, respectively ( p < 0.05). According to FRE scores, the generated responses were suitable for only 4.5% and 33% of U.S. adults in ChatGPT-4 and Bing Chat, respectively ( p < 0.05). Conclusions ChatGPT-4 and Bing Chat consistently provided appropriate responses to the questions. Both LLMs had low readability scores, but ChatGPT-4 provided more difficult responses in terms of readability.

Purpose Chronic myeloid leukemia (CML) is a rare malignant myeloproliferative disorder characterized by the presence of the Philadelphia chromosome, a reciprocal translocation between chromosomes 9 and 22. Ocular manifestations in CML are infrequent but can be the initial indicators of the disease. We report the case of a 28-year-old male patient with suspected hyperviscosity syndrome secondary to CML, based on fundus findings and supported by multimodal imaging. Methods Multimodal imaging was performed to assess the ocular manifestations, before and after treatment. Results The patient presented with posterior segment findings consistent with hyperviscosity syndrome, including retinal hemorrhages, vascular dilation, and tortuosity as demonstrated by multimodal imaging techniques. Further investigations confirmed the diagnosis of CML, and treatment with hydroxyurea, allopurinol, and imatinib led to a significant improvement in visual acuity and resolution of posterior segment findings. Conclusion Ocular manifestations in CML vary widely and include both anterior and posterior segment involvement. Regular ophthalmic examinations are essential for the early detection of relapses or complications. This case underscores the importance of interdisciplinary collaboration between ophthalmologists and hematologists for the timely diagnosis and management of ocular manifestations in CML.

Purpose To evaluate the differences between two distinct patient postoperative positions following rhegmatogenous macula-off retinal detachment surgery. Methods In this consecutive clinical study, 26 patients were placed in either the face-up or face-down postoperative position group. Postoperative follow-ups were conducted at 1, 3, and 6 months using type 1 M-CHARTS, best-corrected visual acuity (BCVA), intraocular pressure, and optical coherence tomography imaging to evaluate the functional and anatomical outcomes after surgery. Results The average vision recovery at 6 months was 0.35159 +/- 0.30945 logarithm of the minimum angle of resolution (logMAR) for the face-up group and 0.81521+/- 0.46662 logMAR for the face-down group. At 6 months, the average horizontal type 1 M-CHARTS for the face-up group was 0.1615 +/- 0.1710 and 0.2846 +/- 0.2828 for the face-down group; and the average vertical type 1 M-CHARTS was 0.1615 +/- 0.1557 for the face-up group and 0.2846 +/- 0.1725 for the face-down group. Conclusion The face-up position demonstrated significant improvement in both metamorphopsia and BCVA, suggesting that slow reattachment of photoreceptors on the retinal pigment epithelium layer can result in better quality of sight recovery. Further studies with larger sample sizes are warranted to confirm these results. The study adhered to the guidelines of the Declaration of Helsinki.

Introduction Variants in COL4A5 are responsible for X-linked Alport syndrome. It is characterized by kidney disease, sensorineural hearing loss and variable ocular abnormalities. In this case, a woman with corneal endothelial neovascularization, glaucoma, nuclear cataract, temporal retinal thinning, and renal defects was identified with a variant in COL4A5. Case presentation We described a 64-year-old woman who was referred to our Eye Clinic due to a progressive decline in vision in both eyes over the course of two years. Corneal endothelial neovascularization, elevated intraocular pressure (31.2 mmHg and 27.8 mmHg in the right and left eyes, respectively), lens opacity, an increased cup-to-disc (C/D) ratio (0.8 in both eyes), and thinner retinal nerve fiber layer were detected upon examination. Concurrently, microscopic hematuria and proteinuria were observed. Following phacoemulsification and the administration of Latanoprost, intraocular pressure returned to within the normal range. Whole exome sequencing revealed a novel hemizygous missense pathogenic variant in COL4A5 (c.3980G > T (p.G1327V)). Importantly, this particular variant was not identified in the healthy daughter of the proband, underscoring its potential relevance to the observed clinical manifestations. Conclusions Our study reports a novel phenotype observed in a 64-year-old woman, featuring corneal endothelial neovascularization and glaucoma, which has been linked to X-linked Alport syndrome caused by variants in COL4A5. This discovery contributes to the broadening of our understanding of the clinical heterogeneity associated with COL4A5 variants.

Purpose To evaluate anatomical and functional outcomes of Faricimab therapy on a case of polypoidal choroidal vasculopathy (PCV) refractory to previous treatments. Major findings A 56-year-old patient with PCV presenting with a large pigment epithelial detachment (PED) showed best-corrected visual acuity (BCVA) improvement, subretinal fluid (SRF) reduction and PED height reduction at 2 months follow up after a loading phase of 4 monthly Faricimab injections. The patient had previously undergone Aflibercept, Brolucizumab and photodynamic therapy (PDT) treatments with a suboptimal response. Conclusion Despite Faricimab proving itself as an efficient alternative in a case of PCV poorly responsive to previous treatments, definite evidence still needs to be confirmed in larger studies.

Purpose To compare Nepafenac effect on foveal thickness (FT), total macular volume (TMV), and BCVA after cataract surgery when added to the common regimen of topical steroids perioperatively. Methods PubMed, Scopus, EMBASE, the Cochrane Library, and ClinicalTrials.gov were searched systematically on April 28, 2022, for RCTs. Our primary outcome was the change in FT at final follow-up visits. We also considered FT at different follow-up durations, TMV, and visual outcome. We summarized our analyses by calculating the mean differences (MD) with associated 95% confidence intervals (CI) using restricted maximum likelihood in random effects models for continuous outcomes. The individual trials were evaluated for quality utilizing the Cochrane Collaboration's risk of bias assessment tool. Results Twenty-four RCTs totaling 4716 eyes were eligible. A significant difference was found between Nepafenac group and control group in the change in FT at final follow-up visit (MD, −11.622; 95% Cl [−17.475, −5.769]), at one week follow-up visit (MD, −6.409; 95% Cl [−13.148, 0.330]), at one month follow-up visit (MD, −9.090; 95% Cl [−13.260, −4.919]), at two months (MD, −5.769; 95% Cl (−22.477, −10.939]), and at three-month follow-up visit (MD, −12.913; 95% Cl [−22.254, −3.572]). However, there was no significant difference between the two groups regarding change in TMV at final follow-up visit. Nevertheless, a significant difference was found between the two groups regarding post-operative BCVA only at three-month follow-up visit. Conclusion Topical Nepafenac in addition to topical steroid is superior to the common regimen of topical steroid alone perioperatively in the prevention of macular swelling.

Introduction Torpedo maculopathy (TM) is a rare, typically benign and congenital anomaly of the retinal pigment epithelium (RPE) characterized by a torpedo-shaped lesion in the macula. DeSanto-Shinawi syndrome (DESS) is an equally rare genetic disorder caused by mutations in the WAC gene, presenting with intellectual disability, dysmorphic features, and growth retardation. Case description This report illustrates the case of monolateral TM in a patient with DESS. The genetic and phenotypic characteristic were described and correlated with retinal features, assessed by means of retinography, spectral domain OCT (SD-OCT) and fluorescein angiography (FA). Conclusion This case report discusses the unusual co-occurrence of TM in a patient diagnosed with DESS, contributing to the sparse literature on this association and expanding the phenotypic spectrum of both conditions.

Purpose To present successful visual rehabilitation of a patient who suffered from a severe chemical burn by performing a new alternative surgical approach termed dual lamellar keratoplasty combined with phacoemulsification and intraocular lens implantation. Methods A 45-year-old man with a history of caustic soda eye injury in his left eye leading to a limbal stem cell deficiency, persistent epithelial defect, corneal scarring, and progressive vision loss was admitted. No posterior segment alterations were noted. Once the ocular surface was stabilized, simple limbal epithelial transplantation was performed; however, deep corneal scarring, Descemet folds, and lens opacity were detected. Therefore, 6 months later, simultaneously Deep anterior lamellar keratoplasty (DALK) and Descemet stripping with automated endothelial keratoplasty (DSAEK), combined with cataract surgery was performed for visual rehabilitation. Results After 15 months of follow-up, anterior segment reconstruction was achieved, and visual acuity improved from hand motion to 0.5 LogMAR unit. No intraoperative or postoperative complications were noted. Conclusion This new modified procedure seems to be efficacy and safe for visual rehabilitation avoiding open-sky surgery-related risks by combining DALK, DSAEK and cataract surgery in a case of severe ocular burn.

Purpose To report a unique case of post-cataract surgery endophthalmitis caused by Ceftazidime-resistant Rhizobium radiobacter, successfully managed with timely vitrectomy and intravitreal Levofloxacin Case report A healthy 73-year-old man underwent phacoemulsification and posterior chamber intraocular lens implantation in his left eye. Postoperative ocular examination (OE) one day after the surgery revealed no specific findings. However, on postoperative day 6, he presented with a painful and red left eye. Diagnosed with postoperative endophthalmitis on day 8 at another hospital, he received IVI of Ceftazidime and Vancomycin before being urgently referred to our hospital on the same day. Early vitrectomy and anterior chamber irrigation were performed timely when he arrived on day 8, with cultures identifying Ceftazidime-resistant Rhizobium radiobacter, sensitive to Levofloxacin. Repeated IVI of Levofloxacin (500 mcg/0.1 ml) ensued on post-cataract operative days 13 and 18. Six months post-cataract surgery, the corrected distance visual acuity of the left eye stabilized at 20/33, and OE remained stable. Conclusion Early vitrectomy and vitreous culture prove effective in managing postoperative endophthalmitis in drug-resistant Rhizobium radiobacter. IVI of Levofloxacin, though rare, proved to be effective in treating Ceftazidime-resistant and Levofloxacin-sensitive pathogens in our case.