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OBJECTIVES To analyse the entity of retinal vasculitis, including frosted branch angiitis (FBA), or retina vascular occlusion in patients with familial Mediterranean fever (FMF). METHODS Retrospective collaborative case series using invitation by email to uveitis specialists around the Mediterranean basin. This series was combined with a literature review. Exclusion criteria included infectious diseases, Behcet's disease or other autoimmune diseases. RESULTS A total of 16 patients (21 eyes) had FMF and retinal vasculitis (FBA 11 patients, mild retinal vasculitis 5 patients). The mean age at onset of vasculitis was 29.5 ± 13.4 (range 9-62) with a female to male ratio of 9 to 7. In 19 eyes treated with various forms of corticosteroid and/or immunosuppression, the mean initial spectacle-corrected visual acuity improved from 6/194 to 6/10.5 at the last mean follow-up of 29.0 ± 34.9 months (p

BACKGROUND/OBJECTIVES: To analyze the ophthalmic characteristics of congenital prepapillary vascular loop (PVL) and to propose a new morphologic classification dividing the loops into six types. SUBJECTS/METHODS: Collaborative multinational multicentre retrospective study of PVL cases. RESULTS: There was a total of 49 cases (61 eyes), 37 unilateral (75.5%) and 12 bilateral (24.5%), 32 arterial type (65.3%) and 18 venous type (36.7%) (one patient had either kind in each eye). The mean number of loops per eye was 2.7 (range, 1-7). The loops were asymptomatic in 42 cases (85.7%). Other findings included: the presence of cilioretinal artery (14 cases), retinal vascular tortuosity (26 cases), amaurosis fugax (1 case), branch retinal artery occlusion (1 case) and vitreous haemorrhage (3 cases). Six morphologic loop types could be discerned based on elevation (flat vs. elevated), shape (figure of 8 or corkscrew with hyaline sheath), number (multiple or single), location (central or peripheral), lumen size (arterial vs. arteriolar) and presence of vascular tortuosity or vitreous traction. CONCLUSIONS: PVL are usually asymptomatic and can be divided into six morphologic types with different pathogenesis during early embryogenesis.

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.

We aimed to evaluate the retina and the choroid in children with juvenile idiopathic arthritis (JIA) employing optical coherence tomography (OCT). This cross-sectional study, carried out between June 2017–December 2019, included JIA patients with (JIAU; n = 28) and without (JIAN; n = 65) uveitis and age-matched healthy controls (HC) (n = 102). Laboratory and demographic information of the children were obtained from hospital records. Activity of the disease was evaluated by the Juvenile Arthritis Disease Activity Score-71 (JADAS-71). Choroidal scans were obtained with spectral domain-OCT in enhanced-depth imaging (EDI)-OCT mode to assess choroidal thickness (ChT) at five locations (under the fovea, at 750 and 1500 μm nasal and temporal sections), luminal area (LA), stromal area (SA), total subfoveal choroidal area (TCA) and CVI (choroidal vascularity index). Central foveal thickness (CFT) and 1-mm diameter foveal thickness (FT) were calculated automatically through macular volume scan analysis. The choroid was significantly thicker in JIAU and JIAN patients than in HC at the subfoveal and at the 750N, 750T, 1500T points (p

Objective: To compare the peripapillary retinal nerve fiber layer (pRNFL) thickness and macular volume in patients with multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD), and healthy controls. Materials and Methods: This study used spectral domain-optical coherence tomography to measure pRNFL and the volume of retinal layers at the macula. Forty patients with MS or NMOSD and 19 healthy controls were enrolled. Results: After at least one clinical episode of optic neuritis, the average pRNFL for NMOSD [60 µm (34-105)] and patients with MS [85 µm (41-109)] were lower than in healthy controls [99 µm (88-109)], and patients with NMOSD had significantly lower pRNFL compared with patients with MS (p=0.002). Foveal volume did not differ between optic neuritis eyes of patients with NMOSD and MS (NMOSD 1.18±0.3 mm3, MS 1.82±2.07 mm3). The mean pRNFL values in seronegative NMOSD eyes [58 µm (range, 47-111)] tended to be thinner compared with seropositive NMOSD eyes [76 µm (range, 42-105)]. This finding was not considered to be statistically significant. Conclusion: Our study revealed that both foveal (area in a 1.5 mm ETDRS ring) and total macular volume (area in a 6 mm ETDRS ring) were lower in both patients with MS and NMOSD compared with healthy controls. Optic neuritis of NMOSD is associated with a thinner average RNFL compared with MS, suggesting earlier axonal injury in the affected optic nerves.

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 Results At week 16, proportion of patients taking sarilumab or placebo with ≥2-step reduction in VH or corticosteroid dose Conclusions Subcutaneous sarilumab may provide clinical benefits in the management of NIU of the posterior segment, especially in eyes with uveitic macular edema.

Predominantly hemorrhage represents one of the possible manifestations of choroidal neovascularisation (CNV) in eyes with age-related macular degeneration (AMD). The purpose of this study is to evaluate the effecte of ranibizumab treatment in patients with predominantly hemorrhagic CNV secondary to AMD.Twenty-five patients with predominantly hemorrhagic choroidal neovascularization due to AMD with at least three ranibizumab injections and followed up for at least 12 months were included in the study. The months of follow-up were recorded (baseline, 3The mean age of the patients was 68.1±5.7 (range: 63-82) years, the mean follow-up was 19.9±14.5 (range: 12-67) months, and the mean number of injections was 4.0±1.4 (range: 3-15). The initial VA was 39.3±17.9 (range: 1-65) letters, CMT was 272.7±104 (range: 164-587) μm, and the initial lesion width was 11.4±10.5 (range: 1.3-45.7) mmAlthough the final visual outcome is limited by the progression of the disease, hemorrhagic lesions treated with ranibizumab have stable anatomical outcome.

PurposeTo ascertain the anatomic factors that help achieve non-surgical sealing in full thickness macular hole (FTMH).MethodsRetrospective collaborative study of FTMH that closed without surgical intervention.ResultsA total of 78 patients (mean age 57.9 years) included 18 patients with blunt ocular trauma, 18 patients that received topical or intravitreal therapies and 42 patients with idiopathic FTMH. Mean±SD of the initial corrected visual acuity (VA) in logMAR improved from 0.65±0.54 to 0.34±0.45 (pConclusionOur data suggest an observation period in new onset FTMH for non-surgical closure, in the setting of trauma, treatment of CME and size

ObjectiveThe aim of this study was to conduct a detailed ophthalmological examination in children with systemic lupus erythematosus (jSLE), including choroidal thickness (ChT), choroidal vascularity index (CVI) and peripapillary retinal nerve fiber layer (RNFL).MethodsThe study included all jSLE patients ( n = 21) diagnosed according to the Systemic Lupus International Collaborating Clinics classification criteria between January 2017 and April 2017, and an age- and gender-matched control group ( n = 21). The Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was used to assess disease activity. After routine eye examinations, ChT at five points (750 µ and 1500 µ from the center of the fovea both in the temporal and nasal quadrants and under the fovea), total subfoveal choroidal area (TCA), luminal area (LA), stromal area (SA), CVI and RNFL thickness at the optic disc were evaluated.ResultsOne patient had active ocular involvement in the form of episcleritis. Another patient had corticosteroid-induced cataract. The median age of the patients was 16 years (6-19 years). ChT at five points, TCA, LA and SA were found to be higher in patients with jSLE, whereas RNFL thickness and CVI were similar to those of the healthy control individuals. No correlation was determined between optical coherence tomography findings, SLEDAI and the immunological parameters (antinuclear antibodies, anti-double-stranded DNA, complements 3 and 4, extracted nuclear antigen antibody, antiphospholipid antibody). Intraretinal and subretinal fluid was not present in any of the patients.ConclusionThe choroid was thicker in patients with jSLE than in the control group. The study results suggest that jSLE may affect the choroid. Ophthalmological evaluation is important in SLE patients, even in the absence of relevant complaints.

To determine the prevalence of cerebrotendinous xanthomatosis (CTX) in patients with idiopathic bilateral juvenile cataract in Turkey.In this multicenter, epidemiologic observational study, patients with idiopathic bilateral juvenile cataract (aged ≥1 year at study entry) were identified from the records of 31 ophthalmology clinics. The Mignarri suspicion index determined in all potential CTX patients; blood samples were collected, and genetic testing for CYP27A1 gene mutation was performed. Cholestanol levels of the patients were measured, and those with a level of ≥3.75 μg/mL (threshold value) underwent genetic testing for mutations in the CYP27A1 gene.CTX was diagnosed in 7 of 452 patients (1.55%) with bilateral juvenile cataract. There was no significant difference between patients with a Mignarri score of100 (n = 8) and those with a score of ≥100 (n = 19) with respect to cholestanol levels and genetic test results. Genetic testing was undertaken in 27 patients based on elevated cholestanol levels. Of these 27, 7 (26%) had CYP27A1 gene mutations. Of these 7 patients with genetically confirmed CTX, 5 (71%) were in the group with higher Mignarri score (≥100).CTX is a treatable condition, and early detection is crucial for avoiding irreversible neurological manifestations. Screening using the Mignarri suspicion index and cholestanol blood levels can be helpful in identifying suspected cases of CTX.

Objective: To compare the visual and anatomical outcomes of intravitreal ranibizumab (group 1), bevacizumab (group 2), and triamcinolone (group 3) for center-involving diabetic macular edema. Methods: We retrospectively enrolled 275 eyes of 208 consecutive patients. Visual acuity (VA) in Early Treatment Diabetic Retinopathy Study (ETDRS) letters and central macular thickness (CMT) values on optical coherence tomography were extracted. Reported side effects were noted. Results: At 6 months, the mean changes in VA in group 1, group 2, and group 3 were +4.9, +4.3, and +4.6 letters, respectively (p = 0.911). Improvement of CMT at 6 and 24 months was significantly better in group 3 compared to groups 1 and 2 (p = 0.012 and p = 0.001, respectively). At 24 months, the only independent variable affecting the change in VA was initial VA (p = 0.020). Cataract and glaucoma prevalences were higher in group 3 (p = 0.000 and p = 0.001, respectively). Conclusions: Three treatment methods had similar effects with regard to improvement in VA; however, intravitreal triamcinolone provided additional anatomical improvement.

Objective: Evaluation of Complement Factor I (CFI) rs10033900 and rs2285714 polymorphism frequencies in patients with age-related macular degeneration (AMD) and healthy controls in a Turkish population. Methods: A total of 111 eyes of 111 AMD patients and 96 eyes of 96 healthy controls, only one eye of individuals, were included in the study; however, 2 patients’ and 4 controls’ samples were excluded as analyses could not be performed for rs10033900 polymorphism. The AMD patients and control group (>50 years) lacked corneal, lenticular, vitreal opacity. However, these patients did not have any retinal diseases apart from AMD. Venous blood samples of patients were collected. Central macular thickness, subfoveal choroidal thickness (SCT), presence of reticular drusen, epiretinal membrane, and pigment epithelial detachment were investigated using Spectral-Domain Optical Coherence Tomography, and the largest diameter of atrophic areas measured. Drusen properties were documented from fundus photographs. The lesion width was calculated by using fundus fluorescein angiography. Results: There was no difference between patient and control groups and polymorphism distributions. The frequency of the CT allele was higher in patients with dry-type AMD with retinal pigment epithelial abnormality (p = 0.041). SCT was significantly thinner in TT allele carriers with rs2285714 polymorphism (p = 0.030). No significant relationship was found between the other parameters and polymorphism distributions. Con­clusion: CFI rs10033900 and rs2285714 polymorphisms in a Turkish population were not associated with AMD.

Background Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis affecting small or medium arteries with a negative ANCA serology and no evidence of glomerulonephritis. Objectives The aim of this study was to evaluate the choroid with optical coherence tomography (OCT) in children with polyarteritis nodosa (PAN) and adenosine deaminase-2 deficiency (DADA-2). Methods The study included all PAN and DADA-2 patients (n=15), examined between June 2017 and September 2018, and an age and gender-matched control group (n=15). After ocular examination, choroidal images taken with sd-OCT (Heidelberg Spectralis) were evaluated with regard to choroidal thickness (ChT) at five points (750 and 1500 microns from the center of the fovea both in the temporal, nasal quadrant and under the fovea), total subfoveal choroidal area (TCA), luminal area (LA), stromal area (SA) and choroidal vascularity index (CVI). Results None of the patients had active ocular complaints or findings. The mean (±SD) age was 8.4 ± 3.69 years. ChT at 3 points, TCA, LA, and SA were found to be higher in patients with PAN and DADA-2. The CVI values were similar in both groups. No correlations were found between the OCT findings, activity score index (PVAS) and the biochemical parameters (Erythrocyte sedimentation rate, leukocyte, C-Reactive Protein). Conclusion The results of this study showed that the choroid was thicker in patients with PAN and DADA-2 than in the control group, suggesting that PAN and DADA-2 may affect the choroid. Ophthalmologic evaluation is important in PAN and DADA-2 patients, even in the absence of relevant complaints. References [1] Ozen S, Anton J, Arisoy N, et al. Juvenile polyarteritis: results of a multicenter survey of 110 children. J Pediatr. 2004;145(4):517-522. [2] Kur J, Newman EA, Chan-Ling T. Cellular and physiological mechanisms underlying blood flow regulation in the retina and choroid in health and disease. Prog Retin Eye Res. 2012;31(5):377-406. Acknowledgement None Disclosure of Interests Hafize Emine Sonmez: None declared, Abdullah Agin: None declared, Sibel Kadayifcilar: None declared, Ata Baytaroglu: None declared, Ozge Deliktas: None declared, Selcan Demir: None declared, Yelda Bilginer: None declared, Bora Eldem: None declared, Seza Ozen Consultant for: Seza Ozen is receiving consultancy fees from Novartis, Speakers bureau: Roche

A woman with Bietti’s crystalline dystrophy (BCD) was first examined when she was 27 years of age and has been followed for 10 more years. The disease course was monitored initially with spectral domain-optical coherence tomography and then with swept-source optical coherence tomography angiography (OCTA). OCTA analysis showed that choroidal vessels could be visualized at the outer retinal layer segmentation due to retinal pigment epithelial atrophy and blood flow was reduced at the level of choroidal segmentation. OCTA can play a major role in the follow-up of BCD patients by analyzing changes in choroidal flow.

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.

Topic: An international, expert-led consensus initiative to develop systematic, evidence-based recommendations for the treatment of noninfectious uveitis in the era of biologics. Clinical relevance: The availability of biologic agents for the treatment of human eye disease has altered practice patterns for the management of noninfectious uveitis. Current guidelines are insufficient to assure optimal use of noncorticosteroid systemic immunomodulatory agents. Methods: An international expert steering committee comprising 9 uveitis specialists (including both ophthalmologists and rheumatologists) identified clinical questions and, together with 6 bibliographic fellows trained in uveitis, conducted a Preferred Reporting Items for Systematic Reviews and Meta-Analyses protocol systematic review of the literature (English language studies from January 1996 through June 2016 ; Medline [OVID], the Central Cochrane library, EMBASE, CINAHL, SCOPUS, BIOSIS, and Web of Science). Publications included randomized controlled trials, prospective and retrospective studies with sufficient follow-up, case series with 15 cases or more, peer-reviewed articles, and hand-searched conference abstracts from key conferences. The proposed statements were circulated among 130 international uveitis experts for review. A total of 44 globally representative group members met in late 2016 to refine these guidelines using a modified Delphi technique and assigned Oxford levels of evidence. Results: In total, 10 questions were addressed resulting in 21 evidence-based guidance statements covering the following topics: when to start noncorticosteroid immunomodulatory therapy, including both biologic and nonbiologic agents ; what data to collect before treatment ; when to modify or withdraw treatment ; how to select agents based on individual efficacy and safety profiles ; and evidence in specific uveitic conditions. Shared decision-making, communication among providers and safety monitoring also were addressed as part of the recommendations. Pharmacoeconomic considerations were not addressed. Conclusions: Consensus guidelines were developed based on published literature, expert opinion, and practical experience to bridge the gap between clinical needs and medical evidence to support the treatment of patients with noninfectious uveitis with noncorticosteroid immunomodulatory agents.

AIM: To investigate the long-term visual and anatomical outcomes of patients who underwent intravitreal ranibizumab monotherapy to treat neovascular age-related macular degeneration (AMD) and followed-up for at least 2y. METHODS: A total of 74 eyes of 74 patients who underwent ranibizumab monotherapy for neovascular AMD were included in this retrospective study. RESULTS: The average patient age was 72.1±6.5 (range, 57-85)y, the average follow-up time 46.2±13.1 (range, 24-75)mo, and the average number of visits 24.1±9.5 (range, 8-48). The mean number of injections in year 1 was 4.5, 1.6 in year 2, 0.9 in year 3, 0.4 on year 4, and 0.1 in the following years. Within the entire follow-up period, the mean number of injections was 7.6±4.4 (range, 2-21). The mean visual acuity was 48.1±15 (range, 15-76) letters at baseline and 45.7±19 (range, 7-75) at year 5. The mean central macular thickness was 303±78 (range, 178-552) µm at baseline and 251±51 (range, 138-359) µm at year 5. Scars developed in 47 (63.5%) eyes at the end of the follow-up period, and atrophy was evident in 6 (8.1%) eyes. CONCLUSION: Ranibizumab monotherapy can stabilize visual acuity for a mean period of 4y in patients with neovascular AMD.

Purpose To evaluate the efficacy of 0.7 mg intravitreal dexamethasone implant in the treatment of radiation maculopathy after stereotactic radiotherapy for posterior uveal melanoma. Methods Retrospective chart review of seven eyes of seven consecutive patients was performed. Extracted data included age, sex, initial and follow-up visual acuities and central macular thickness values, intraocular pressure, follow-up time, number of implants, and time elapsed from radiotherapy to implantation. Main outcome measures were visual acuity and central macular thickness. Glaucoma, cataract formation, or systemic side effects, if any, were recorded. Results Female to male ratio was 4:3. Mean age was 49.9 ± 17.0 (range: 27-73). Initial mean visual acuity was 20.4 ± 12.5 Early Treatment Diabetic Retinopathy Study letters and initial central macular thickness measured 514.1 ± 135.1 μm on spectral domain optical coherence tomography. All patients except one showed improvement in visual acuity and a mean improvement of 7.4 ± 6.2 letters was observed in the whole group (range: 0-16). The mean reduction in central macular thickness was 226.7 ± 157.0 μm after a mean 9.1 ± 3.4 months of follow-up. On average, implantation of intravitreal dexamethasone was performed 35.2 ± 16.5 months after radiotherapy. Four patients were treatment naive and three had previous intravitreal bevacizumab injections with limited response. Ozurdex reimplantations were performed in four patients and the mean number of injections was 1.7 ± 0.8. Mean time to reimplantation was 5.0 ± 2.12 months. Only one patient developed posterior subcapsular cataract and all patients had intraocular pressures within normal limits. No systemic side effects were observed. Conclusion In our experience, intravitreal implantation of 0.7 mg dexamethasone is an anatomically, and to a lesser extent functionally effective procedure for radiation maculopathy after stereotactic radiotherapy for posterior uveal melanoma.

To compare visual outcomes, number of visits and ranibizumab injections in patients treated with a WaitExtend (WE) or TreatObserve (TO) regimen.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) TO patients were examined monthly and retreated if needed, (ii) WE 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.Of the 104 screened patients, 99 were eligible and received ≥ 1 ranibizumab injection; 93 were randomized (TO: 45, WE: 48). The median (interquartile range [IQR]) change in BCVA (logMAR) from baseline at Month 12 was similar between groups (TO: -0.12 [0.38]; WE: -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 TO, and 12.0 (2.0) for WE (p0.001). 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.WE regimen resulted in a similar efficacy and safety profile to the labelled TO regimen in patients with CNV secondary to AMD, and may help reduce the burden of follow-up visits.

The purpose of this study was to assess retinal vascular characteristics of patients with Laron syndrome (LS) as a genetic model of IGF-I deficiency before and after rhIGF1/IGFBP3 treatment and to compare them with healthy controls.A total of 28 subjects (11 LS, and 17 controls) were enrolled. Patients with LS received combined rhIGF1/rhIGFBP3 1-2 mg/kg/d in a single dose and digital fundus imaging was performed. The number of branching points and tortuosity of retinal vessels were studied. Pre- and post-treatment findings were compared with each other and with controls.The number of branching points was significantly lower in patients with LS in comparison to controls (12.73 ± 3.41, and 17.47 ± 5.82 respectively, p = 0.012). This difference persisted after treatment (12.09 ± 2.66 post-treatment LS versus controls, p = 0.017). Tortuosity indices of nasal arteries (NA) were significantly less in LS than that of controls (upper NA 1.07 ± 0.04 and 1.12 ± 0.06 respectively p = 0.022; lower NA 1.07 ± 0.03 and 1.13 ± 0.07 respectively, p = 0.004). This difference also persisted following treatment (p 0.05). Remaining vessels did not differ in tortuosity index. There was no significant difference of tortuosity index and number of branching points before and after treatment in patients with LS.Retinal vascular development may be adversely affected in the setting of severe IGF-I deficiency confirming a major role for GH/IGF-I axis during retinal vascular development in humans antenatally. Resolution of IGF-I deficiency following birth using rhIGF1, however, may not reverse these changes, suggesting that IGF-I may be necessary but insufficient by itself for postnatal angiogenesis.

We report the autofluorescence pattern and optical coherence tomography (OCT) characteristics in a 6-year-old boy with glutathione synthetase deficiency. The patient underwent complete ophthalmological examination, including full-field electroretinography, visual evoked potentials, fundus autofluorescence, and OCT imaging. Electrophysiological studies showed time-delay and subnormal responses. Fundus autofluorescence imaging revealed increased parafoveal autofluorescence compared to normal, and OCT showed alteration of photoreceptor and retinal pigment epithelium layers in the parafoveal region.

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.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.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.Gevokizumab was well tolerated and rapidly controlled acute ocular exacerbations of Behçet's disease uveitis without the need for high-dose corticosteroid.

PURPOSE To evaluate the changes in choroidal thickness (ChT) and intraocular pressure (IOP) during the water drinking test (WDT) in patients with exfoliation syndrome (XFS). MATERIALS AND METHODS In this prospective study, 20 eyes of 20 patients with XFS and 20 eyes of 20 healthy subjects underwent measurements of subfoveal ChT, IOP, systolic and diastolic blood pressure before and 30 and 60 minutes after the WDT. WDT involved ingestion of 1 L of water within 5 minutes. χ test and analysis of variance were used for the statistical analysis. RESULTS There were no significant differences between the 2 groups with respect to age (P = 0.238), sex (P = 1.000), central corneal thickness (P = 0.714), axial length (P = 1.000), diastolic blood pressure (P = 0.350), systolic blood pressure (P = 0.064), and IOP (P = 0.234) before water ingestion. Baseline ChT was lower in the XFS group (285.1 ± 69.1 μm) as compared with that of controls (339.4 ± 81.2 μm) (P = 0.028). ChT was found to be significantly increased at 30 and 60 minutes after water ingestion in the XFS group (P < 0.001), whereas in the control group an increase was found only at the 60-minute time-point (P = 0.028). Independent of baseline ChT, at 60-minute endpoint, the magnitude of choroidal expansion was higher in XFS eyes (13.2 ± 14.3%) compared with control eyes (6.2 ± 3.5%) (P = 0.028). The mean IOP was higher at all time-points with respect to their respective baseline values in both XFS (P = 0.002, P < 0.001) and control (P = 0.038, P = 0.002) eyes. CONCLUSIONS Choroidal expansion during WDT does not seem to be an important mechanism for IOP elevation in healthy eyes or those with XFS. Furthermore, the similarity of IOP increase to controls suggests that trabecular outflow facility is not impaired in eyes with XFS.

We conducted this study to elucidate the possible role of interleukin-6 (IL-6) in the pathogenesis of proliferative diabetic retinopathy (PDR).Together with pertinent clinical and laboratory data, intravitreal and serum concentrations of IL-6 were determined in 8 patients with PDR by means of enzyme-linked immunosorbent assay (ELISA). The results were compared with data from 8 nondiabetic control subjects undergoing vitrectomy.Significantly higher intravitreal IL-6 concentrations were found in patients with PDR (mean [SD], 755 [177] pg/mL) compared with control subjects (93 [151] pg/mL) (p = 0.001). The serum IL-6 levels in the PDR group were lower than the measurable threshold of the ELISA kit (0.16 pg/mL). Diabetic patients with macular edema had a higher mean (SD) level of intravitreal IL-6 (896 [73] pg/mL) compared with patients without macular edema (613 [119] pg/mL) (Mann-Whitney U test, p = 0.03). Correlation analysis did not reveal any significant association between intravitreal IL-6 levels and patient age, duration of either diabetes mellitus or vitreous hemorrhage, panretinal photocoagulation, type of current medical therapy, hyperglycemia, or the biochemical indicators of renal function.IL-6, a proinflammatory cytokine, may have a role in PDR. Intraocular production of IL-6 appears to be responsible for the elevated intravitreal levels observed.

A case of a paraneoplastic vitelliform retinopathy is described in the fellow eye of a 40-year-old woman 4 years after enucleation of her left eye for a ciliochoroidal melanoma. There were multiple yellow subretinal vitelliform-like lesions at the posterior pole, which were hyperautofluorescent and hypofluorescent on fluorescein and indocyanine green angiographies. Spectral-domain optical coherence tomography showed hyperreflective material between the retinal pigment epithelium and the photoreceptor layer. Paraneoplastic vitelliform retinopathy may appear concomitantly with the development of distant metastases years after treatment of a ciliochoroidal melanoma and may present with symptoms resembling melanoma-associated retinopathy and electroretinogram findings. However, unlike with melanoma-associated retinopathy, the retinal lesions are located between the retinal pigment epithelium and the photoreceptor layer. [ Ophthalmic Surg Lasers Imaging Retina. 2013;44:290–292.]

Purpose: To report cerebral venous thrombosis as a complication of intravenous corticosteroid treatment in a patient with multiple sclerosis. Method: A case report. A 44-year-old woman with a previous diagnosis of multiple sclerosis presented with panuveitis and retinal perivasculitis. Intravenous pulse corticosteroid therapy was given for three days. Results: The panuveitis and retinal periphlebitis began to resolve within one week; however, ten days after the last corticosteroid dose, the patient was hospitalized with the diagnosis of cerebral venous thrombosis. Conclusion: Although intravenous corticosteroid treatment for uveitis associated with multiple sclerosis can be very helpful, the patient should be monitored closely for systemic side effects.

Purpose: To evaluate the outcomes and complications of cataract surgery in patients with Behcet's disease. Setting: Department of Ophthalmology, Hacettepe University School of Medicine, Ankara, Turkey. Methods: Thirty-three eyes of 26 patients with Behcet's disease that had extracapsular cataract surgery between January 1993 and July 1999 were analyzed retrospectively. The mean age of the patients was 38.9 years (range 20 to 54 years). The mean postoperative follow-up was 22.9 months (range 6 to 66 months). Results: Extracapsular cataract extraction (ECCE) was performed in 22 eyes with and in 6 eyes without intraocular lens (IOL) implantation. Phacoemulsification with IOL implantation was performed in 5 eyes. Postoperatively, the visual acuity was better in 29 eyes (87.8%) and was 0.5 or better in 14 eyes (42.4%). Posterior segment complications of Behcet's disease, mainly optic atrophy and macular alterations from preoperative inflammatory episodes, restricted final acuity. No significant difference was detected in postoperative inflammation among the types of surgery; that is, ECCE, ECCE with IOL implantation, and phacoemulsification with IOL implantation. A neodymium:YAG laser posterior capsulotomy was performed in 3 cases. Conclusions: In patients with Behcet's disease, inflammation after extracapsular surgery was mild when surgery was performed after at least 3 months of no inflammatory signs. The results show that the outcomes of extracapsular cataract surgery mainly depend on the degree of preoperative posterior segment involvement.

AIM—To determine whether a newly identified thrombophilia factor, activated factor VII (FVIIa), is associated with retinal vein occlusion (RVO). METHODS—54 consecutive cases with RVO seen between March and September 1999 were included in the study. 22 cases had central retinal vein occlusion (CRVO) and 32 had branch retinal vein occlusion (BRVO). Ophthalmoscopic examination with detailed medical history was followed by blood analyses for liver and renal functions, cholesterol, triglycerides, complete blood count, and coagulation factors including protein C activity, free protein S, antithrombin III, fibrinogen, and factor VIIa (FVIIa). Data were compared with those of the control group, composed of 19 cases under ophthalmological follow up for refractive errors, presbyopia, or senile cataract. RESULTS—Hypertension was highly prevalent in cases with BRVO. Complete blood count, and liver and kidney function tests were within normal limits in the study group. Two cases had low protein C activity, and one had low free protein S. FVIIa levels were significantly higher in the RVO group than in the control group (p=0.0004). There was no significant difference in FVIIa levels between the CRVO and BRVO groups (p=0.51). CONCLUSION—No haematological parameter except FVIIa differed significantly from that of the control group. Elevation of FVIIa level may play a part in the pathophysiology of both CRVO and BRVO.

Purpose : The goal of the study was to investigate the prevalence of photopsias and formed visual hallucinations (Charles Bonnet syndrome) in patients suffering from low vision. Methods : Eighty patients with low vision due to different ophthalmic pathologies were included in this study. Low vision rehabilitation examination was performed at the Low Vision Unit of our Retina Service. After examination, the patients were asked in a nonthreatening and noninvasive manner whether they had experienced any event of seeing something that really had not existed there at that time. Results : Visual hallucinations and/or photopsias were experienced by 31.25% of the patients. Conclusion : Charles Bonnet syndrome is common in patients with low vision and is not age-dependent.

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.

Purpose In the etiology of pterygium abnormalities in tear functions have also been emphasized. In this study, tear function tests are evaluated in patients with pterygium. Methods Schirmer's test 1, tear film break-up time and mucus fern patterns were evaluated in 70 eyes with pterygium and in 70 eyes of the age matched control group. Marginal tear films were also assessed. Results Tear film break-up time was significantly reduced in the pterygium group. Mucus fern patterns and marginal tear films were found to be markedly abnormal in the eyes with pterygium, however, there was no significant difference in Schirmer's test 1. Conclusion Tear function tests disclosed disrupted tear film stability which is more likely to be due to the altered mucin. This change may either be the primary factor inducing pterygium formation or reflect an existing pathology in the cells lining the ocular surface.

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.

We report a case of bilateral choroidal detachment that occurred shortly after uneventful bilateral lensectomy and anterior vitrectomy. B-scan ultrasonography showed severe choroidal detachment in the right eye accompanied by limited detachment of the overlying retina and mild choroidal effusion in the left eye. Two bilateral peribulbar injections of corticosteroid 5 days apart resulted in complete resolution of the choroidal effusion in each eye.

Objective: To describe a series of patients with bilateral acute iris transillumination, pigment dispersion, and sphincter paralysis. Methods: We reviewed the medical records and clinical photographs of 26 patients seen at 5 centers in Turkey and Belgium between March 16, 2006, and July 6, 2010. Observation procedures included clinical examination, anterior segment color photography, gonioscopy, laser flare photometry, and pupillometry. Results: All 26 patients (20 women and 6 men; mean [SD] age, 43.2 [10.5] years) had bilateral involvement. Twenty-three patients (88%) had acute-onset disease with severe photophobia and red eyes. Nineteen patients (73%) had a preceding flulike illness and used systemic antibiotics, including moxifloxacin. Diagnostic laboratory workup was unremarkable. There was pigment discharge into the anterior chamber, and flare was elevated in the absence of inflammatory cells. Most patients had severe diffuse transillumination of the iris and mydriatic distorted pupils. Pupillometry revealed a compromised reaction to light. The most serious complication was an intractable early rise in intraocular pressure. Gonioscopy revealed heavy pigment deposition in the trabecular meshwork. Although symptoms were relieved promptly by application of topical corticosteroid, the median duration of pigment dispersion was 5.25 months. Conclusions: Bilateral acute iris transillumination with pigment dispersion and persistent mydriasis is a new clinical entity that is not an ocular adverse effect of oral moxifloxacin treatment, as previously suggested. The etiopathogenesis of this entity remains to be elucidated.

Purpose: To describe a case with retinitis pigmentosa associated with sea fan type retinal neovascularization. Methods: Complete ocular examination including fluorescein angiography was performed in a 9-year-old girl. Results: Ophthalmoscopically, in addition to arteriolar narrowing and bone corpuscular pigmentation of both retinae, a vascular lesion with surrounding intraretinal exudation was noted in the upper equatorial region of the right eye. On fluorescein angiography, the lesion stained in the form of a sea fan neovascularization. Conclusion: Sea fan type of neovascularization can be seen in association with retinitis pigmentosa. Fluorescein angiography is important in identifying the exact nature of such a lesion.

To evaluate visual outcomes in patients with neovascular age-related macular degeneration (NV-AMD) who were treated with pegaptanib sodium in European clinical ophthalmology practices.Thirteen centres in eight European countries participated in this retrospective study. Medical records for patients with any angiographic subtype of subfoveal choroidal neovascularisation secondary to NV-AMD with visual acuities (study eye) of 20/40-20/320 treated with 0.3 mg pegaptanib as first-line treatment and with at least 24 weeks of follow-up were identified. Anonymised data reflecting at least 24 and up to 54 weeks of follow-up were recorded. Primary end points were visual acuity outcomes at weeks 24 and 54 compared with those reported at week 54 in the vascular endothelial growth factor (VEGF) Inhibition Study in Ocular Neovascularisation (VISION) trial.In all, 253 patients were followed for at least 24 weeks; 62 patients completed 54 weeks of follow-up. A mean of 4.4 (SD, 1.8) pegaptanib injections were administered through 24 weeks. Compared with the VISION trial, the European experience showed that90% of patients in the current cohort lost15 letters from baseline at both time points compared with 70% in the VISION trial at 54 weeks. Pegaptanib was well tolerated with no reported cases of endophthalmitis, traumatic cataract, or iatrogenic retinal detachment.Pegaptanib was found to stabilise vision in a greater percentage of patients and produced greater overall visual improvement in this group of treatment-naive patients with NV-AMD compared with outcomes reported in the VISION trial; however, interpretation of these results should be tempered given the differences in design between this retrospective study and the prospective controlled trial.

To identify the morphologic appearance of keratic precipitates (KPs) with in vivo confocal microscopy (IVCM) in uveitic syndromes.A total of 75 eyes of 72 patients with a mean age of 38.6+/-15.1 years who had active intraocular inflammation and whose corneas had KP on slit-lamp examination were included in this study. IVCM (Confoscan 3.0, Vigonza, Italy) was used to image the part of the corneal endothelium in which KP were most densely deposited. KP were classified into five groups: type I (small, round), type II (stippled), type III (dendritiform), type IV (large, smooth-rounded), and type V (globular). When more than one type of KP was observed with IVCM, a distinction between the predominant and the less frequent KP was made as 'primary' and 'secondary' KP.In 50 (66.7%) eyes more than one type of KP was imaged. The size of the KP ranged between 5 and 150 microm. The most frequently observed primary KP type in Behçet's disease was type I (100%), in ankylosing spondylitis type II (57.1%), in Fuchs' uveitis syndrome type III (85.7%), in granulomatous uveitis type V (42.9%), in infectious uveitis type III (66.7%), and in juvenile idiopathic arthritis associated uveitis type I (66.7%). The KP types showed a statistically significant difference between different uveitic syndromes (Fisher's exact test, P0.001).Certain KP types appear to be characteristic of various uveitic syndromes. IVCM may have a potential role in the diagnostic work-up of uveitic patients.

Purpose: To determine whether elevated levels of thrombin activatable fibrinolysis inhibitor (TAFI) may contribute to thrombotic risk for patients with retinal vein occlusion (RVO) and to investigate the possible correlations between TAFI activity level and other conventional risk factors. Methods: Ninety patients with RVO (cases), except those receiving medication affecting the study parameters, those undergoing a surgical procedure within the last week, and those with kidney and/or liver failure, were enrolled in the study. The control group included similar patients matched for age and sex. After written informed consent was obtained, parameters including TAFI activity levels, conventional risk factors, results of routine hematological examination, and factor V Leiden and prothrombin G20210A mutations were evaluated by analysis of blood samples obtained after an 8-hour fast. Results: Although TAFI activity levels were slightly elevated in cases (190.5 ± 43.8) compared with controls (183.9 ± 41.8), the difference was not statistically significant (P = 0.36). According to evaluation of TAFI activity in subgroups (>200%, 150–200%, and 0–150%), 36.7% with central RVO, 40.0% with branch RVO, and 30% of controls were found to have TAFI activity of >200% (P = 0.83). TAFI activity levels did not correlate with age, sex, demographics, clinical status, and hematological variables. Finally, in stepwise regression analysis, TAFIa (carboxypeptidase U) activity was not found to be an important risk factor for RVO. Conclusion: On the basis of these data, TAFI activity was not found to be a new risk factor for either type of RVO. However, further larger studies may better identify the exact role of TAFI in the pathogenesis of RVO.

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.

The purpose of this study was to investigate the association of age-related macular degeneration (AMD) with plasma homocysteine, vitamin B12, and folate levels.Sixty patients diagnosed with AMD at our clinic between March 2004 and September 2004 were assessed in a prospective cross-sectional study. Plasma homocysteine, vitamin B12, and folate levels taken after 8 h of fasting from 30 patients with exudative AMD and 30 patients with dry AMD were compared with the results of 30 age- and sex-matched healthy participants.Patients with both exudative and dry types of AMD had significantly higher plasma homocysteine levels (mean 14.19+/-3.11 micromol/l; 13.07+/-2.90 micromol/l respectively) compared with the controls (mean 10.79+/-2.56 micromol/l; (p=0.000 and p=0.008 respectively). Homocysteine levels were higher in the exudative AMD group compared with the dry AMD group, but the difference was not statistically significant (p=0.290). Plasma vitamin B12 levels were found to be significantly lower in the exudative AMD group (289.14+/-113.44 pg/l) compared with the controls (436.17+/-204.12 pg/l) and dry AMD group (443.47+/-190.83 pg/l; (p=0.000). Plasma folate levels were comparable among groups (p=0.106).This study suggests an association between elevated plasma homocysteine and AMD regardless of the subtype. Further controlled prospective studies are needed to investigate the possible role of homocysteine in AMD and the effect of vitamin B12 and folate supplementation in this process.

Purpose: To review the etiologic factors and complications of uveitis in patients younger than 16 years. Patients and Methods: Between January 1989 and December 1999 in the Department of Ophthalmology of Hacettepe University School of Medicine, 219 patients were diagnosed or observed as having pediatric uveitis. After complete ocular and physical examinations, routine and specific laboratory and radiologic investigations were performed. Medical or surgical treatment was employed when necessary. Results: Of the 219 patients, 112 were girls, with a mean age of 7.4 ± 4.2 years, and 107 were boys, with a mean age of 8.3 ± 3.4 years. In 24.2% of the cases, no etiologic factor could be ascertained; these cases comprised the idiopathic group. Among the remaining cases, the most common etiologies were toxoplasmosis, juvenile rheumatoid arthritis ORA), pars planitis, Behçet's disease, and Fuchs' heterochromic iridocyclitis. Anatomically, anterior uveitis was the most common form. The mean follow-up time was 37 ± 6.2 months. Complications for which surgical treatment was employed were identified in 71 eyes (20.9%), most of which were due to JRA, pars planitis, or Behçet's disease. Conclusion: Uveitis in childhood may be idiopathic or most commonly due to toxoplasmosis, JRA, and pars planitis. Due to inflammation itself or to prolonged therapy especially with corticosteroids, pediatric uveitis entities (mostly JRA, pars planitis, or Behçet's disease) may result in complications necessitating a surgical approach. J Pediatr Ophthalmol Strabismus 2003;40:335-340.

Purpose To describe a case of sympathetic ophthalmia (SO) associated with ocular and cerebral vasculitis. Methods The authors report a 38-year-old woman who presented with bilateral uveitis 7 years after a penetrating trauma to the right eye. Ocular examination included fundus fluorescein and indocyanine green angiography. Medical history disclosed an episode of dysarthria and right-sided weakness. Results Anterior uveitis was present in the previously injured pseudophakic right eye, which harbored a piece of glass stuck in the lower temporal iris. In the left eye, vitreal inflammation with retinal sheathing and subretinal lesions was predominant. Besides retinal vasculitis, fundus angiography showed choroidal vasculitis. Neurologic studies were compatible with cerebral vasculitis. Conclusions In SO, choroidal vasculitis that is invisible clinically can be demonstrated angiographically. Although rare, inflammation can also affect the nervous system.

Clinical findings of a 2.5-year-old girl presenting with barely detectable horizontal nystagmus and high hypermetropia are described. Despite the normal appearing anterior segments, the child had posterior microphthalmus and bilateral papillomacular retinal folds, conforming to a recently described, rare congenital disease. The patient also had significant posterior pole excyclorotation and avascular zones at the extreme temporal periphery without ridge formation or neovascularization. These findings were not reported previously. Other remarkable features include mildly depressed photopic and scotopic electroretinogram amplitudes and a short axial length of the vitreous cavity compared to age-matched normals, measured by ultrasonography. The present case adds new elements to this relatively rare ocular developmental abnormality.