Search

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.

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.

Background: The extent of neurodegeneration in the earliest stages of central nervous system (CNS) demyelination is not known. Optical coherence tomography (OCT) is a powerful tool to study neurodegeneration in demyelinating disorders. Objectives: To study neuroaxonal loss in the retina of individuals with radiologically isolated syndrome (RIS) and investigate whether OCT measurements are associated with brain volumetrics and clinical conversion to multiple sclerosis (MS). Methods: Subjects fulfilling the Okuda criteria for RIS ( n = 15 patients, 30 eyes) and age- and sex-matched healthy controls (HC) underwent spectral-domain OCT and magnetic resonance imaging for volumetric measurement of brain structures. Results: Macular ganglion cell-inner plexiform layer (mGCIPL), macular retinal nerve fiber layer (mRNFL), and temporal peripapillary RNFL (pRNFL) thickness; normalized total brain volume (nTBV); and normalized thalamic volume (nTV) were reduced in RIS compared to HC. mGCIPL, mRNFL, and pRNFL measurements were associated with nTBV, nTV, and normalized gray and white matter volumes in the RIS group. pRNFL was thinner in individuals with RIS who converted to MS in 5 years. Conclusions: Retinal neurodegeneration can be detected in the papillomacular region in the earliest stages of CNS demyelination and reflects global disease processes in the brain. OCT can be potentially useful for predicting prognosis in RIS.

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

Background/purpose To assess EDI-OCT (enhanced depth imaging optical coherence tomography) of choroid for inflammatory signs in children with polyarteritis nodosa (PAN) and adenosine deaminase-2 deficiency (DADA-2). Methods In this cross-sectional study conducted between June 2017 and September 2018, we evaluated children diagnosed with PAN (n = 11) and DADA-2 (n = 4) and an age- and sex-matched control group (n = 15). Demographic and laboratory data were retrospectively analyzed from patient charts. Disease activity was assessed using the pediatric vasculitis activity score (PVAS). Choroidal images were obtained with spectral domain-OCT to measure choroidal thickness (ChT) at 5 points (750 and 1500 μm from the foveal center in the temporal and nasal quadrants and beneath the fovea), and to calculate the total subfoveal choroidal area (TCA), luminal area (LA), stromal area (SA), and the choroidal vascularity index (CVI). Results The median (min-max) age was 8 (4–16) years in PAN patients, 6 (5–16) years in DADA-2 patients and 8 (8–10) years in control group at the OCT visit (p = 0.214). The ChT at 3 points and the TCA, LA, and SA were higher in children with both PAN and DADA-2 patients compared to those of the control group (p p = 0.049, p = 0.007, p = 0.007, p = 0.006, p = 0.033, respectively). The CVI was similar in both groups. No association was observed between the OCT findings, PVAS, and the erythrocyte sedimentation rate, and serum leukocyte and C-reactive protein levels. Conclusion Similar CVI scores were obtained from PAN and DADA2 patients under treatment and from healthy controls. Increased subfoveal ChT without any other signs of ocular involvement may suggest choroidal thickening as a sign of mild subclinical inflammation.

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.

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.

Background/aim We aimed to assess the association between retinal vascular caliber (RVC) scores and disease activity in rheumatoid arthritis (RA) patients. Materials and methods Forty-seven RA patients, 32 systemic lupus erythematosus (SLE) patients, and 45 healthy people were enrolled. RA and SLE patients were subdivided into groups according to C-reactive protein (CRP) levels. RA patients were also grouped according to Disease Activity Score-28 (DAS-28). Fundus photography was performed for all patients. RVC was summarized as the central retinal artery and vein equivalents (CRAE and CRVE). Results Mean CRVE for RA patients was 213.3 ± 17.8 μm compared with 209.2 ± 14.1 μm for SLE and 217.5 ± 26.2 μm for the control group (P = 0.17). RVC scores did not differ between the CRP-high and CRP-low groups. As the RA disease activity increased, the widening of CRVE became more prominent and statistically significant. When the DAS-28 > 5.1 (CRVE, 220.4 (211.8–246.5) μm) group and DAS-28 ≤ 3.2 (CRVE, 214.4 (172.4–242.3) μm) group were compared, statistical significance was more pronounced (P = 0.03) than when comparing the DAS-28 > 3.2 and DAS-28 ≤ 3.2 groups (P = 0.05). Conclusions CRVE, which reflects systemic inflammation and possibly increased cardiovascular risk, was significantly increased in active RA patients. The association between retinal venular widening and disease activity, regardless of CRP, may be a sign that RA-related inflammation may have systemic vascular effects even with normal levels of CRP.

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.

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.

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.

Sarcoidosis, a multisystem disorder of unknown etiology that involves multiple organs, is rare in children. The true incidence and prevalence of childhood sarcoidosis is unknown. As in adults, many children with sarcoidosis may be asymptomatic; the disease may remain undiagnosed. A complete and systematic evaluation of the patient is essential for the sarcoidosis diagnosis in children. Here, we describe a case of 12-year-old female who presented with 2 years history of uveitis and hepatosplenomegaly. A chest computerized tomography revealed scattered peripheral pulmonary nodules and bilateral hiliar lymphadenopathy. Bone marrow aspiration and liver biopsy were not diagnostic. A lung biopsy showed non-necrotizing epithelioid cell granulomas. She was diagnosed with sarcoidosis according to demonstration of granulomatous

Sarcoidosis, a multisystem disorder of unknown etiology that involves multiple organs, is rare in children. The true incidence and prevalence of childhood sarcoidosis is unknown. As in adults, many children with sarcoidosis may be asymptomatic; the disease may remain undiagnosed. A complete and systematic evaluation of the patient is essential for the sarcoidosis diagnosis in children. Here, we describe a case of 12-year-old female who presented with 2 years history of uveitis and hepatosplenomegaly. A chest computerized tomography revealed scattered peripheral pulmonary nodules and bilateral hiliar lymphadenopathy. Bone marrow aspiration and liver biopsy were not diagnostic. A lung biopsy showed non-necrotizing epithelioid cell granulomas. She was diagnosed with sarcoidosis according to demonstration of granulomatous inflammation and the exclusion of confusable entitiesLa sarcoidosis, un trastorno multiorgánico de etiología desconocida que afecta varios órganos, es poco frecuente en los niños. Se desconocen la incidencia y la prevalencia reales de la sarcoidosis infantil. Al igual que en los adultos, muchos niños con sarcoidosis tal vez no presentan síntomas y la enfermedad cursa sin diagnosticarse. Es fundamental realizar una evaluación completa y sistemática del paciente para establecer el diagnóstico de sarcoidosis en los niños. Se describe el caso de una niña de 12 años con uveítis y hepatoesplenomegalia de dos años de evolución. Mediante una tomografía computarizada del tórax, se hallaron nódulos pulmonares periféricos dispersos y linfadenopatía hiliar bilateral. La aspiración de médula ósea y la biopsia de hígado no fueron diagnósticas. La biopsia de pulmón mostró granulomas de células epitelioides no necrosantes. A la paciente se le diagnosticó sarcoidosis en virtud del hallazgo de inflamación granulomatosa y de la exclusión de entidades confusoras.

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.

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 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.

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.

Galactosemia is a secondary glycosylation disorder characterized by galactose deficiency of glycoproteins and glycolipids. Abnormal glycosylation of coagulation factors and evidence of liver disease are associated with coagulopathy in galactosemic infants. We report a case of a neonate with galactosemia presenting with bilateral vitreous hemorrhage (VH). During the follow-up, hemorrhage in the right eye resolved; however, it persisted in the left eye. Vitrectomy was planned for the left eye. In addition to cataract, VH is another ophthalmic finding in galactosemia with serious sequelae such as amblyopia. Serious complications of coagulopathy in galactosemic infants can be prevented with early diagnosis and prompt treatment. Inclusion of galactosemia in the neonatal screening program offers an opportunity to prevent early severe symptoms.

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.

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 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.