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Purpose: To investigate the anatomical and visual results of intravitreal dexamethasone implant treatment for macular edema secondary to retinal vein occlusion. Materials and Methods: The study included a total of 25 patients undergoing dexamethasone implant for macular edema secondary to retinal vein occlusion. Retrospective evaluation included best corrected visual acuity (BCVA) measured with ETDRS charts and macular thickness using optical coherence tomography (OCT) at each visit. The contrast sensitivity levels of patients were evaluated with Pelli-Robson chart. Results: The mean age of the patients was 59.5±13.3 years (range, 34-84years). The mean follow-up time was14.6 ± 5.5 months (range, 6-27 months) and the mean number of implants was 2.6±1.5 (range,1-6). The increase in the visual acuity according to time was statistically significant (p<0.005). The decrease in macular edema was statistically significant (p<0.005). The initial mean contrast sensitivity before implant was 0.72±0.50 log, and the final mean contrast sensitivity was 1.08±0.46 log (p<0.005). No systemic adverse events were observed in any of the cases. When the ocular adverse effects were assessed, cataract progression was seen in 54.5% and cataract operations were performed on 4 patients (18.1%). The increase in intraocular pressure was statistically significant (p<0.005) and only one patient needed medical treatment. Conclusion: Dexamethasone implant had a positive effect on anatomic and visual results in retinal vein occlusion-associated macular edema but the ideal repeat interval for intravitreal dexamethasone treatment should be shorter than 6 months. [ABSTRACT FROM AUTHOR]

Purpose. To report the efficacy of hyperbaric oxygen (HBO) therapy in a case of branch retinal artery occlusion (BRAO) in a 15-year-old boy. Methods. We report a 15-year-old boy with sudden loss of vision due to BRAO. Examination included laboratory evaluation for systemic risk factors. Follow-up exams included visual acuity, fundus examination, fundus fluorescein angiography, and visual field testing. HBO therapy was employed for treatment. Results. Medical history was positive for isolated glucocorticoid deficiency. Laboratory evaluation disclosed hyperhomocysteinemia and methylenetetrahydrofolate reductase (MTHFR) mutation. The visual acuity 0.05 at presentation improved to 0.8 after 20 days of HBO therapy. There was no change on visual fields. Conclusion. In this pediatric case, HBO therapy was useful in the treatment of BRAO. [ABSTRACT FROM AUTHOR]

The article discusses the ocular manifestations of Behçet's disease (BD). The disease was first described in 1937 by Hulusi Behçet as a chronic multisystem disorder characterized by recurrent episodes of uveitis, oral aphthous lesions, genital ulcers and skin lesions, with underlying vasculitis. Ocular manifestations are usually reported in 70 to 95% of cases of the disease. Anterior uveitis is commonly observed in females while panuveitis is common among males.

Background: To evaluate the systemic and thrombophilic risk factors for retinal vein occlusion (RVO) and to determine whether the elevated level of soluble endothelial protein C receptor (sEPCR) is a risk factor for thrombosis. Methods: In this case–control study, 56 patients with central RVO (CRVO), 26 patients with branch RVO (BRVO) and 78 healthy sex- and age-matched subjects were enrolled. Following ophthalmological examination, venous blood was analysed for glucose, lipid profile, lipoprotein (a), homocysteine, activated partial thromboplastin time, fibrinogen, factor VIII, protein C activity, protein S activity, activated protein C resistance, antithrombin III activity, lupus anticoagulant, anti-cardiolipin antibody, anti-phospholipid antibody, sEPCR, factor V Leiden mutation and prothrombin G20210A mutation. Results: Apart from hypertension, glaucoma, lipoprotein (a), homocysteine and factor VIII, elevated levels of sEPCR were found to be a risk factor for CRVO (odds ratio, 1.02; 95% confidence interval, 1.007–1.028; P = 0.001). Patients with CRVO had significantly higher levels of sEPCR than those with BRVO and controls (respectively, 160.1 ± 83.8, 116.8 ± 65.2 and 111.3 ± 60.5; P = 0.005). Moreover, 39% of patients with CRVO had levels of sEPCR more than 200 ng/mL, and only 5% of controls and 11% of patients with BRVO had similar high levels. Conclusions: Besides known classical risk factors, elevated levels of sEPCR seem to be an important candidate risk factor for especially CRVO. [ABSTRACT FROM AUTHOR]

Purpose: To emphasize the importance of the association of optic disc drusen (ODD) with angioid streaks (AS). Methods: Three patients with ODD and AS underwent a thorough ophthalmic examination including perimetry and B-scan ultrasonography. Fundus fluorescein angiography was performed when indicated. Results: Bilateral ODD associated with AS were present in all of the cases. Pseudoxanthoma elasticum was the definite etiology of the AS in two cases, one of which was complicated by subretinal neovascularization. Laser photocoagulation resulted in closure of the membrane. Conclusions: The follow-up of patients with ODD with regard to the development of AS is essential as ODD may be the earliest clinical manifestation in patients with pseudoxanthoma elasticum, which has potential vision- and life-threatening complications. [ABSTRACT FROM AUTHOR]

• There are patients with early-onset LCHAD deficiency with poor metabolic control despite standard therapy. • The frequency and severity of rhabomyolysis was decreased dramatically with triheptanoin in the presented case. • The effect of triheptanoin on LCHADD-retinopathy needs to be monitored. Early-onset long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency is a fatty acid β-oxidation disorder with a poor prognosis. Triheptanoin, an anaplerotic oil with odd-chain fatty acids can improve the disease course. The female patient presented here was diagnosed at the age of 4 months, and treatment was started as fat restriction, frequent feeding, and standard medium-chain triglyceride supplementation. In follow-up, she had frequent rhabdomyolysis episodes (∼8 per year). At the age of six, she had 13 episodes in 6 months, and triheptanoin was started as part of a compassionate use program. Following unrelated hospital stays due to multisystem inflammatory syndrome in children and a bloodstream infection, she had only 3 rhabdomyolysis episodes, and hospitalized days decreased from 73 to 11 during her first year with triheptanoin. Triheptanoin drastically decreased the frequency and severity of rhabdomyolysis, but progression of retinopathy was not altered. [ABSTRACT FROM AUTHOR]

Cogan's syndrome (CS) is a rare inflammatory disease characterized by interstitial keratitis or uveitis, vestibular impairment, and progressive hearing loss, commonly bilateral. Although glucocorticoids are fundamental treatment options, in most cases, hearing loss gradually worsens. Herein we report 2 pediatric cases of CS who were treated with corticosteroids and methotrexate. One patient had a cochlear implant, and the hearing of the other patient improved with treatment. Also, a systematic literature review was conducted for articles including pediatric CS patients. In the literature, 34 articles describing 44 pediatric patients with CS were identified. Sudden hearing loss (95.3%) and ocular symptoms (92.5%) were the most common manifestations in these patients. Also, aortic involvement was present in 19.5% of patients in the literature. Otorhinolaryngologists, ophthalmologists, and pediatricians should collaborate to diagnose and manage CS to prevent progressive hearing loss and eye involvement. [ABSTRACT FROM AUTHOR]

Purpose: To develop an algorithm for the diagnosis of Behçet's disease (BD) uveitis based on ocular findings. Methods: Following an initial survey among uveitis experts, we collected multi-center retrospective data on 211 patients with BD uveitis and 207 patients with other uveitides, and identified ocular findings with a high diagnostic odds ratio (DOR). Subsequently, we collected multi-center prospective data on 127 patients with BD uveitis and 322 controls and developed a diagnostic algorithm using Classification and Regression Tree (CART) analysis and expert opinion. Results: We identified 10 items with DOR >5. The items that provided the highest accuracy in CART analysis included superficial retinal infiltrate, signs of occlusive retinal vasculitis, and diffuse retinal capillary leakage as well as the absence of granulomatous anterior uveitis or choroiditis in patients with vitritis. Conclusion: This study provides a diagnostic tree for BD uveitis that needs to be validated in future studies. [ABSTRACT FROM AUTHOR]

Aim: It is to review the clinical features, and surgical, and visual outcomes of rhegmatogenous retinal detachment in a pediatric population. Material and Methods: Medical records of 31 children aged 16 years old or younger with rhegmatogenous retinal detachment were retrospectively analyzed. Cases of acute retinopathy of prematurity were excluded. The data for etiologies, risk factors, prior intraocular surgery, therapeutic approach options, anatomic and functional results were evaluated. Results: Thirty-three eyes of 31 patients with RRD were evaluated. The mean patient age was 11.42±3.82 years (range 3-16). There were 26 (83.9%) males and 5 (16.1%) females. The most common etiology was trauma. At presentation 57.5% of affected eyes (19/33) had a visual acuity worse than 20/400. Preoperative retinal tear was found in 60.6% (20/33) of patients. At initial examination, proliferative vitreoretinopathy was detected in 36.3% (12/33) of patients. The most common primary operation was scleral buckle with subretinal fluid drainage (69.6%, 23/33). Anatomical reattachment was accomplished in 84.8% of cases (28/33). At the final visit, 78.78% of affected eyes (26/33) had a better visual acuity when compared to preoperative examination. In nine of affected eyes (27.27%), visual acuity was 20/200 or better. Mean follow-up time was 37.5 months (range 3-72). Conclusion: In our series, pediatric RRD occurred most commonly in association with trauma. Most eyes were anatomically reattached, and most eyes retained vision of 20/800 or better. Preserving vision in children with RRD is of great importance because of high risk of vision threatening events to the fellow eye. [ABSTRACT FROM AUTHOR]

Purpose To assess efficacy and safety of sarilumab, a human anti-interleukin-6 receptor antibody, for treatment of posterior segment noninfectious uveitis (NIU). Design Randomized, double-masked, placebo-controlled, phase 2 study. Participants Fifty-eight patients (eyes) with noninfectious intermediate, posterior, or panuveitis. Methods Eyes received treatment every 2 weeks for 16 weeks with subcutaneous sarilumab 200 mg or placebo. Main Outcome Measures The primary end point was the proportion of patients with ≥2-step reduction in vitreous haze (VH) on the Miami scale or with a reduction of systemic corticosteroids (prednisolone or equivalent) to a dose of <10 mg/day at week 16. Primary end point was based on VH evaluation by a central reading center. Investigator evaluation of VH was a prespecified, planned secondary analysis. Results At week 16, proportion of patients taking sarilumab or placebo with ≥2-step reduction in VH or corticosteroid dose <10 mg/day was 46.1% vs. 30.0% (P = 0.2354) based on central reading center assessment of VH and 64.0% vs. 35.0% (P = 0.0372) based on investigator assessment of VH, respectively. In the subgroup of eyes with VH grade ≥2 at baseline, the mean VH reduction from baseline to week 16 was significantly greater with sarilumab vs. placebo regardless of assessment by the central reading center (−2.1 [n = 11] vs. −1.7 [n = 3], respectively; P = 0.0255) or investigator (−2.5 [n = 19] vs. −1.2 [n = 11], respectively; P = 0.0170). The mean best-corrected visual acuity gain from baseline to week 16 was greater with sarilumab vs. placebo in the overall population (8.9 vs. 3.6 letters, respectively; P = 0.0333) and in the subgroup of eyes with central subfield thickness (CST) ≥300 μm at baseline (12.2 [n = 13] vs. 2.1 [n = 7] letters, respectively; P = 0.0517). Corresponding changes in CST were −46.8 vs. +2.6 μm (P = 0.0683) in the overall population and −112.5 [n = 13] vs. −1.8 [n = 6] μm (P = 0.1317) in the subgroup of eyes with CST ≥300 μm at baseline, respectively. The most common ocular adverse events were worsening of uveitis (0 [placebo] and 3 [sarilumab] patients) and retinal infiltrates (1 [placebo] and 2 [sarilumab] patients). Conclusions Subcutaneous sarilumab may provide clinical benefits in the management of NIU of the posterior segment, especially in eyes with uveitic macular edema. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the safety and efficacy of gevokizumab for the treatment of Behçet's disease uveitis in a prospective, open-label, randomized phase 2 trial.Methods: Behçet's disease patients with new acute ocular exacerbation or at risk of exacerbation received 30 or 60 mg gevokizumab every 4 weeks intravenously or subcutaneously, on top of a stable regimen of immunosuppressives and corticosteroids (≤20 mg/day equivalent prednisolone). Patients withdrew in cases of ocular exacerbation.Results: A total of 21 patients were included (17 acute and 4 at-risk; mean duration of uveitis 45.6 ± 37.4 months). There were no serious adverse events related to gevokizumab. Recorded adverse events were mostly associated with exacerbation of uveitis or its complications. Response was evaluated for 14 acute patients and all showed rapid control of acute ocular exacerbation, mostly within 1 week, without any increase in corticosteroid dosage.Conclusions: Gevokizumab was well tolerated and rapidly controlled acute ocular exacerbations of Behçet's disease uveitis without the need for high-dose corticosteroid. [ABSTRACT FROM AUTHOR]

Purpose: To compare visual outcomes, number of visits and ranibizumab injections in patients treated with a Wait & Extend (W&E) or Treat & Observe (T&O) regimen. Methods: This 12-month, randomized, multicentre, open-label study enrolled patients aged ≥50 years with choroidal neovascularization (CNV) secondary to AMD who had not received anti-VEGF agents. Patients received three monthly injections of ranibizumab before randomization (1:1): (i) T&O patients were examined monthly and retreated if needed, (ii) W&E patients had a follow-up visit 1 month later. If no lesions were active, the interval to the next visit was extended by 2 weeks to a maximum of 8 weeks. Active lesions were re-treated and the follow-up schedule restarted. Primary end-point was change in BCVA at Month 12. Results: Of the 104 screened patients, 99 were eligible and received ≥1 ranibizumab injection; 93 were randomized (T&O: 45, W&E: 48). The median (interquartile range [IQR]) change in BCVA (logMAR) from baseline at Month 12 was similar between groups (T&O: -0.12 [0.38]; W&E: -0.18 [0.32], p = 0.267). Median (IQR) number of visits at study end (including screening, baseline and control visit after 1st injection) was 15.0 (1.0) for T&O, and 12.0 (2.0) for W&E (p < 0.001). Injection numbers were similar between groups (median [IQR]: 6.0 [3.0] and 5.0 [4.0], respectively, p = 0.215). Adverse events were similar between groups. Conclusion: W&E regimen resulted in a similar efficacy and safety profile to the labelled T&O regimen in patients with CNV secondary to AMD, and may help reduce the burden of follow-up visits. [ABSTRACT FROM AUTHOR]

Subacute sclerosing panencephalitis is a rare disease of central nervous system caused by defective measles virus. Chorioretinitis with macular involvement is the mostly observed ocular finding in the disease. Other reported ocular findings in the disease are cortical blindness, hemianopsia, nystagmus, extraocular muscle paresis and optic atrophy. We present a rare case of subacute sclerosing panencephalitis with isolated bilateral optic neuritis as the only ocular finding without macular involvement. [ABSTRACT FROM AUTHOR]

Distinct eye abnormalities have been described in children with nephrotic syndrome, particularly in diffuse mesangial sclerosis (i.e. Pierson syndrome). The aim of the study was to investigate whether there were any associated ocular anomalies in children with steroid-resistant nephrotic syndrome (SRNS), all of whom had revealed primary focal segmental glomerulosclerosis in biopsy. Thirty-three SRNS patients (16 male, 17 female) with a median age of 10.5 years (range 3–25 years) were enrolled in the study. Twenty steroid-sensitive nephrotic syndrome (SSNS) patients (ten male, ten female) with a median age of 8 years (range 3–15 years) served as controls. All SRNS patients were examined by mutational analysis for mutations in the NPHS2, WT1, and LAMB2 genes. Nine out of 33 SRNS patients (27.2%) showed various eye abnormalities. However, no abnormal ocular findings were detected in any of the SSNS patients. Abnormal eye findings detected in SRNS patients were anisometropic amblyopia ( n = 4), Mittendorf’s dots ( n = 4), myopic astigmatism ( n = 3) and exotropia ( n = 1). Macular pigment changes ( n = 1), posterior subcapsular opacities ( n = 1) and cataract ( n = 1) were considered as steroid-induced side effects. In four patients, more than one eye abnormality was found. Mutational analysis for the NPHS2, WT1 and LAMB2 genes revealed disease-causing mutations in 24.2% of patients. Homozygous NPHS2 mutations were detected in five patients (15.1%), all of whom had parental consanguinity. In three patients (9%) from non-consanguineous parents, heterozygous de novo WT1 mutations were detected as disease-causing mutations. No LAMB2 mutation was detected in any patient. While four out of five (80%) patients with homozygous NPHS2 mutations showed at least one abnormal ocular finding (i.e. Mittendorf’s dot or anisometric amblyopia), none of the patients with a WT1 mutation had ocular involvement. In conclusion, ocular involvement may accompany SRNS caused by primary focal segmental glomerulosclerosis (FSGS). Ophthalmologic evaluation at the time of diagnosis might be beneficial to characterize further the spectrum of this possible association. [ABSTRACT FROM AUTHOR]