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Nasser G Alsaedi,1,2 Ruba M Alselaimy,3 Abdulaziz A Alshamrani,1 Muhammed AlAjmi,4 Rajiv Khandekar,1 Hassan Al-Dhibi,1 Abdulelah A Al-Abdullah1 1King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia; 2King Abdullah Medical City, Makkah, Saudi Arabia; 3College of Medicine, King Saud University, Riyadh, Saudi Arabia; 4Albahar Eye Centre, Kuwait City, KuwaitCorrespondence: Abdulelah A Al-AbdullahVitreoretinal and Uveitis consultant, Vitreoretinal division, King Khaled Eye Specialist Hospital, P. O. Box 7191, Riyadh, 11462, Saudi ArabiaTel +966114821234 extension 2655Email hasadoc@gmail.comPurpose: To compare the visual and anatomic outcomes of aflibercept versus ranibizumab as a second line treatment for persistent diabetic macular edema (DME) after initial bevacizumab injections.Methods: In this retrospective cohort study, patients with center-involved DME of ≥ 300 μm thickness after bevacizumab intravitreal injections in 2015– 2019 were included. Those treated with ranibizumab (R) and aflibercept (A) were grouped as group R and group A, respectively. The change in central macular thickness (CMT) measured by optical coherence tomography (OCT) and the best corrected distance visual acuity (BCVA) before and after three-monthly anti-VEGF injections (anti-VEGF) in group R and group A were compared and reviewed.Results: There were 80 eyes of 75 patients in group R and 80 eyes of 72 patients in group A. The initial bevacizumab injections in group R and group A varied significantly (p = 0.01). The median change of the CMT after the three injections was not significantly different in group R (80 μm) and group A (81.5μm) (p = 0.7). The improvement of BCVA in group R and group A was not significant (p = 0.5). Dry macula was noted in 1 vs 14 eyes in group R vs group A.Conclusion: After treating refractory DME with initial bevacizumab injections, 3 injections of either aflibercept or ranibizumab had similar anatomic and functional outcomes. Aflibercept achieved dry macula in more eyes with refractory DME compared to ranibizumab.Keywords: aflibercept, bevacizumab, persistent DME, ranibizumab, VEGF switch, vascular endothelial growth factor

Moustafa S Magliyah,1 Abdulrahman H Badawi,2 Abdulaziz A Alshamrani,2 Rizwan Malik,3 Hassan Al-Dhibi2 1Ophthalmology Department, Prince Mohammed Medical City, AlJouf, Saudi Arabia; 2Vitreoretinal Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia; 3Glaucoma Division, King Khaled Eye Specialist Hospital, Riyadh, Kingdom of Saudi ArabiaCorrespondence: Moustafa S MagliyahVitreoretinal Division, King Khaled Eye Specialist Hospital, Al- Oruba Street, PO Box 7191, Riyadh, 11462, Kingdom of Saudi ArabiaTel +966 11 482 1234 ext. 2655Fax +966 11 4821234 ext. 3773Email mussam8@yahoo.comPurpose: To study the effects of perioperative uveitis control (PUC) on postoperative intraocular pressure (IOP) and uveitis activity in uveitic glaucoma (UG) patients who required glaucoma surgeries.Patients and Methods: A retrospective chart review of 109 patients (120 eyes) which had glaucoma surgery for UG. A total of 66 eyes which had PUC were compared to 54 eyes which did not have. Measurements of IOP and uveitis activity were recorded preoperatively and over 2 years postoperatively. Average number of antiglaucoma medications and frequency of surgical failure were obtained in both groups.Results: Over 2 years postoperatively, average IOP was lower in eyes which had PUC. Significant differences in IOP were found at 3 months (P = 0.004), 6 months (P = 0.001), 1 year (P < 0.001), and 2 years (P < 0.001). Lower grades of anterior chamber (AC) inflammation were found in eyes which had PUC. Significant differences were found at 1 month (P < 0.001), 3 months (P < 0.001) and 6 months (P = 0.001). Mean number of antiglaucoma medications at last visit was 0.7 ± 1.1 for eyes which had PUC and 2.6 ± 1.5 for eyes which did not have PUC (P < 0.001). Among eyes which had PUC, only two eyes required second glaucoma surgeries, while 16 eyes with no PUC required further glaucoma surgeries after 27.7 ± 12.5 months (P < 0.001).Conclusion: Proper PUC in patients going for UG surgeries results in lower IOP levels and less AC inflammation over 2 years postoperatively. A comprehensive PUC regimen is needed for uveitic glaucoma patients going for surgeries.Keywords: perioperative uveitis control, uveitic glaucoma, glaucoma surgeries, immunosuppressive therapy

Muneera A Abunajma,1 Hassan Al-Dhibi,1 Emad B Abboud,1 Yahya Al Zahrani,1 Essam Alharthi,2 Abdullah Alkharashi,3 Nicola G Ghazi1,4 1Vitreoretinal Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia; 2Al Hokama Eye Center, Riyadh, Saudi Arabia; 3Department of Ophthalmology, College of Medicine, King Saud University Riyadh, Saudi Arabia; 4Department of Ophthalmology, University of Virginia, Charlottesville, VA, USA Purpose: To investigate the outcomes of pars plana vitrectomy (PPV) for chronic diabetic traction macular detachment (CTMD).Methods: Ninety-six eyes that underwent PPV for CTMD of at least 6 months duration were retrospectively analyzed. Retinal reattachment rate, final vision, and prognostic factors for poor visual outcome were the main outcome measures.Results: All eyes had long-standing TMD (median 12, range: 6–70 months). The median postoperative follow-up was 15 (range: 3–65) months. Eighty-seven eyes (90.6%) had their retina and macula reattached after one PPV. At final examination, 84 eyes (87.5%) had stable vision or at least one line improvement, and three had no light perception. Seventeen (17.7%) and 41 (43%) eyes had preoperative visual acuity of ≥20/200 and ≥5/200 as compared to 40 (41.6%; P=0.0005) and 64 (66.7%; P=0.0014) eyes at final follow-up, respectively. Age >50 years (Odds ratio [OR] =5.84, 95% confidence interval [CI] =1.53–22.19, P=0.01), preoperative vision

Objective: To investigate the clinical features, prevalence, role of surgical intervention and the visual prognosis of macular holes (MH) in patients with Behcet's disease (BD).Materials and Methods: Retrospective study of patients with BD and MH from January 1998 to November 2008.Results: Out of 159 patients, 21 eyes of 17 patients were identified with MH. The mean age was 38.59 (range 23-61) years and the mean follow-up period was 5.1 years (range 13-164 months). The prevalence of MH was 7%. Visual acuity (VA) at the time of presentation ranged from 20/70 to hand-motion. Optical coherence tomography (OCT) findings revealed intraretinal cysts at the edge of the MH. The mean size of MH was 983.6 um; 52% had elevated edges, 43% had flat edges and only one eye (5%) was closed postoperatively. Fluorescein angiography (FA) was consistent with macular ischemia in 76% of the cases. Human leukocyte antigen (HLA) B51 association was found in 14 of the 15 patients investigated. Six patients (out of 17) underwent pars plana vitrectomy. The final VA on their last follow-up ranged from 20/70 to 2/200. Surgical intervention for MH did not result in any visual improvement as compared to non-operated eyes. One patient lost vision completely due to elevated intraocular pressure post vitrectomy and silicon oil tamponade.Conclusions: MH in patients with BD may lead to significant visual disability. Surgical intervention does not seem to have any potential beneficial effect on the VA, probably due to significant macular ischemia and sequelae from the ocular inflammation. [ABSTRACT FROM AUTHOR]

Retinal periphlebitis is a subtype of retinal vasculitis affecting the retinal veins. We report a case of bilateral branch retinal vein occlusion (BRVO) associated with idiopathic retinal periphlebitis and complicated by subfoveal hemorrhage (SFH). An 18-year-old woman presented with best-corrected visual acuity of 20/20 in the right eye and 20/30 in the left eye. Examination revealed bilateral retinal vascular sheathing predominantly involving the retinal veins and bilateral BRVO. Fundus fluorescein angiography revealed localized vascular leakage in the right eye and diffuse vascular leakage in the left eye. Spectral-domain optical coherence tomography showed mild nasal thickening with subfoveal fluid in the left eye. Oral steroids were started on a tapering dosage as well as oral methotrexate. A year later, she presented with regressed vascular sheathing in both eyes with 5/200 vision and SFH in the left eye. Pars plana vitrectomy, subretinal tissue plasminogen activator, intravitreal ranibizumab, laser photocoagulation, and gas injection were performed. The SFH resolved and the visual acuity improved to 20/100. Good vision was preserved in both eyes with no active inflammation. Timely management of SFH in idiopathic retinal periphlebitis can achieve a favorable visual outcome.

Diabetes mellitus (DM) and its complications are major public health burdens in Saudi Arabia. The prevalence of diabetic retinopathy (DR) is 19.7% and the prevalence of diabetic macular edema (DME) is 5.7% in Saudi Arabia. Diabetic macular edema is a vision-threatening complication of DR and a major cause of vision loss worldwide. Ocular treatments include retinal laser photocoagulation, anti-vascular endothelial growth factor (anti-VEGF) agents, intravitreal corticosteroids, and vitreoretinal surgery when necessary. The present consensus was developed as a part of the Saudi Retina Group's efforts to generate Saudi guidelines and consensus for the management of DME, including recommendations for its diagnosis, treatment, and best practice. The experts' panel stipulates that the treatment algorithm should be categorized according to the presence of central macula involvement. In patients with no central macular involvement, laser photocoagulation is recommended as the first-line option. Patients with central macular involvement and no recent history of cardiovascular (CVS) or cerebrovascular disorders can be offered anti-VEGF agents as the first-line option. In the case of non-responders (defined as an improvement of <20% in optical coherence tomography or a gain of fewer than 5 letters in vision), switching to another anti-VEGF agent or steroids should be considered after 3 injections. Within the class of steroids, dexamethasone implants are recommended as the first choice. In patients with a recent history of CVS events, the use of anti-VEGF agents is not recommended, regardless of their lens status. The experts' panel recommends that a future study be conducted to provide a cut-off point for early switching to steroid implants in pseudo-phakic eyes., (Copyright: © Saudi Medical Journal.)

Purpose: There has been a recent increase in accidental retinal injuries due to the careless use of handheld laser devices. The scenarios under which these incidents occur suggest a lack of awareness of the sight-threatening hazards of some handheld lasers. This study aimed to assess knowledge of the population to plan awareness programs and establish protective health policies., Methods: This observational cross-sectional study used a newly-developed, validated, and pilot-tested questionnaire to survey attendees of football matches in Saudi Arabia between September to October 2018., Results: Five-hundred and sixty-nine attendees were surveyed, and 76% of respondents knew laser pointers were harmful and could damage the eye. However, 73% of respondents did not think that the blue laser was dangerous, indicating poor awareness regarding this particular laser. Only 38% knew which ocular structure is most commonly damaged, and 15.6% did not realize that brief exposure can cause vision loss. Females had better knowledge compared to males. Respondents between 25 and 40 years old, females, and married individuals had the highest levels of a positive attitude. There was no statistically significant difference in the mean knowledge score between those in the medical field and other fields ( P = 0.5)., Conclusion: There is an inadequate knowledge of protective measures against handheld lasers. However, there's an overall positive attitude towards spreading awareness about this issue. Yet, targeted awareness campaigns are still needed in addition to more strict government laws to prevent importing or using high-powered handheld laser pointers., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Saudi Journal of Ophthalmology.)

Purpose: Tubercular intermediate uveitis (TIU) and panuveitis (TBP) are difficult to manage because of limitations in diagnostic tools and lack of evidence-based treatment guidelines. The Collaborative Ocular Tuberculosis Study (COTS) analyzed treatment regimens and therapeutic outcomes in patients with TIU and TBP. Methods: Multicentre retrospective analysis. Results A total of 138 TIU and 309 TBP patients were included. A total of 382 subjects received antitubercular therapy (ATT) (n = 382/447; 85.4%) and 382 received corticosteroids (n = 382/447; 85.4%). Treatment failure was observed in 78 individuals (n = 78/447; 17.4%), occurring less frequently in patients receiving ATT (n = 66/382; 17.2%) compared to those who did not (n = 12/65; 18.5%). The study did not show any statistically significant therapeutic effect of ATT in patients with TIU and TBP. Conclusion Taking into account the limitations of the retrospective, non-randomized study design, resultant reliance on reported data records, and unequal size of the samples, the current study cannot provide conclusive evidence on the therapeutic benefit of ATT in TIU and TBP.

Purpose : The Collaborative Ocular Tuberculosis Study (COTS) Group sought to address the diagnostic uncertainty through retrospective cohort analysis of treatment regimens and therapeutic outcomes for patients with tubercular Anterior Uveitis (TAU) across international centers. Methods : Multicentre retrospective analysis of patients diagnosed with TAU between January 2004 to December 2014 that had a minimum follow-up of 1 year. Results : One hundred and sixty-five patients were included. One hundred and seven subjects received antitubercular therapy (ATT) (n = 107/165; 64.9%) with all the patients receiving topical steroid therapy. Treatment failure was noted in 17 patients (n = 17/165; 10.3%), more frequently described in patients that received ATT (n = 13/107, 12.2%), than those that did not receive ATT (n = 4/58, 6.9%). Conclusion : In this retrospective study, addition of ATT did not have any statistically significant impact on outcome in patients with TAU.

Purpose: To examine disease profile of tubercular uveitis (TBU) in Paediatric population. Methods: Among 945 patients of the retrospective multinational study by the Collaborative Ocular Tuberculosis Study (COTS)-1, 29 Paediatric patients diagnosed with TBU were analyzed. Results: Mean age of disease presentation was 12.8 (range 4-18 years), with predominance of males (n = 14/20; 70.0%) and Asian ethnicity (n = 25/29; 86.2%). Posterior uveitis (n = 14/28; 50%) was the most frequent uveitis phenotype, with choroidal involvement occurring in 64.7% (n = 11/17). Incidence of optic disc edema and macular edema was higher in children (n = 8/18; 44.4% and n = 5/18; 27.8%, respectively) than in adults (n = 160/942; 16.9% and n = 135/942; 14.3%, respectively). Comparison of optic disc edema between subgroups showed a significant difference ( P =.006 ). All patients received oral corticosteroids, most of them with antitubercular therapy. Treatment failure developed in 4.8% (n = 1/21). Conclusions: Children have a more severe inflammatory response to the disease, and an intensive anti-inflammatory therapeutic regimen is required to achieve a positive treatment outcome.

Purpose : To report the clinical findings, anatomical features, and treatment outcomes in subjects with ocular tuberculosis (OTB) at 24 months in the Collaborative Ocular Tuberculosis Study (COTS)-1. Methods : Of the 945 subjects included in COTS-1, those who completed a 24-month follow-up after completion of treatment were included. The main outcome measure was a number of patients with treatment failure (TF). Results : 228 subjects (120 males; mean age of 42.82 ± 14.73 years) were included. Most common phenotype of uveitis was posterior ( n = 81; 35.53%), and panuveitis ( n = 76; 33.33%). Fifty-two patients (22.81%) had TF. On univariable analysis, odds of high TF was observed with bilaterality (OR: 3.46, p = .003), vitreous haze (OR: 2.14, p = .018), and use of immunosuppressive therapies (OR: 5.45, p = .003). However, only bilaterality was significant in the multiple regression model (OR: 2.84; p = .02). Conclusions : Majority of subjects (>75%) achieved cure in the COTS-1 at 24-month follow-up. The concept of "cure" may be a valuable clinical endpoint in trials for OTB.

Objective: Aim of the study was to examine extent, natural history, and clinical features associated with visual impairment (VI) in patients diagnosed with ocular tuberculosis (OTB) by the Collaborative Ocular Tuberculosis Study (COTS)-1. Methods: Multi-center retrospective cohort study. Main outcomes were VI. Results: A total of 302 patients were included in the study, including 175 patients whose data related to BCVA were available throughout the 2 years of follow up. Mean BCVA grossly improved at 12, 18, and 24 months of follow-up ( p < .001). Mean BCVA was worse at 12-18th month follow-up for patients treated with ATT versus patients who were not treated with ATT, but patients treated with ATT had a statistically significant improvement in BCVA at the 24-month endpoint. Conclusions: OTB is associated with significant visual morbidity, future well-designed prospective studies are warranted to establish the causal association between OTB and visual loss.

Purpose : To contribute a global description of the spectrum of choroidal involvement in tubercular uveitis (TBU). Methods : Retrospective cohort study of TBU patients with choroidal involvement from 25 centers between January 2004 and December 2014. Medical records of patients with a minimum follow-up of 1 year were reviewed. Results : 245 patients were included. The phenotypic variations included serpiginous-like choroiditis (SLC) (46%), tuberculoma (13.5%), multifocal choroiditis (MFC) (9.4%), ampiginous choroiditis (9%), among others. 219 patients were treated with anti-tubercular therapy (ATT) ( n  = 219/245, 89.38%), 229 patients with steroids ( n  = 229/245, 93.47%) and 28 patients with immunosuppressive agents ( n  = 28/245, 11.42%). Treatment failure was noted in 38 patients ( n  = 38/245, 15.5%). Patients with SLC and ampiginous choroiditis appeared to have superior outcomes on survival analysis ( p  = 0.06). Conclusion : This study provides a comprehensive description of choroidal involvement in TBU. Patients with SLC and ampiginous choroiditis may have better clinical outcomes.

Background: Tuberculosis (TB) is a major infection that can affect the eye as first and sole presentation without features of systemic disease. Controversy exists regarding diagnosis and management of tubercular uveitis (TBU), further compounded by regional variations in disease expression. Purpose: Collaborative Ocular Tuberculosis Study (COTS)-1 aims to address knowledge deficits through collaboration amongst uveitis specialists across the globe by sharing the data of patients with TBU presented at participating centers from January 2004 to December 2014. Methods: Data collection was facilitated by a novel method of real-time encrypted web-based data entry allowing regular updates as new data and recommendations become available. Results: Information on clinical features, investigation findings, management, and treatment outcomes were reviewed to get an idea about real world scenario. Conclusion: The current review aims to focus on methodology and briefing of published reports from COTS group in COTS-1 study to highlight key messages from this large data.

An international, expert led consensus initiative was set up by the Collaborative Ocular Tuberculosis Study (COTS) group to develop systematic, evidence, and experience-based recommendations for the treatment of ocular TB using a modified Delphi technique process. In the first round of Delphi, the group identified clinical scenarios pertinent to ocular TB based on five clinical phenotypes (anterior uveitis, intermediate uveitis, choroiditis, retinal vasculitis, and panuveitis). Using an interactive online questionnaires, guided by background knowledge from published literature, 486 consensus statements for initiating ATT were generated and deliberated amongst 81 global uveitis experts. The median score of five was considered reaching consensus for initiating ATT. The median score of four was tabled for deliberation through Delphi round 2 in a face-to-face meeting. This report describes the methodology adopted and followed through the consensus process, which help elucidate the guidelines for initiating ATT in patients with choroidal TB.

Purpose: To report a favorable effect of intravitreal dexamethasone implantation in Familial Retinal Arterial Macroaneurysms (FRAM)., Methods: Retrospective Case Report., Results: A 32-year-old male who presented with bilateral retinal macroaneurysms. Whole Exome Sequencing (WES) revealed a homozygous c.830-1 G > A mutation in Insulin Growth Factor Binding Protein 7 (IGFBP7) gene, confirming the diagnosis FRAM. The left eye was lost in the course of the disease, whereas the right eye developed a persistent macular edema due to multiple leaking retinal arterial macroaneurysms and responded poorly to intravitreal ranibizumab and only partially to intravitreal aflibercept. Intravitreal dexamethasone implantation in the right eye, on the other hand, resulted in marked visual and structural improvement., Conclusion: Intravitreal dexamethasone injections have beneficial anatomical and visual outcomes in FRAM patients with persistent macular edema poorly responsive to intravitreal injections.

Purpose: To assess the one-year effectiveness and safety of ranibizumab 0.5 mg in treatment- naïve patients with diabetic macular edema (DME) enrolled in the real-world LUMINOUS study., Patients and Methods: A 5-year, prospective, observational, open-label, global study which recruited 30,138 patients across all approved indications. Consenting patients (≥18 years) who were treatment-naïve or previously treated with ranibizumab or other ocular treatments were treated as per the local ranibizumab label. Here, we present the change in visual acuity (VA) (Early Treatment Diabetic Retinopathy Study letter score; primary treated eye) at Year 1, as well as the change in VA based on injection frequencies (≤4 and ≥5), treatment exposure, and the overall adverse events (AEs) and serious AEs (SAEs) in treatment-naïve DME patients., Results: Of the 4,710 DME patients enrolled in the study, 1,063 were treatment-naïve. At baseline, mean age was 64.5 years, 54.7% were male, and 69.2% were white. At 1 year, mean VA letter score improved by +3.5 (n = 502) from a baseline of 57.7 with a mean of 4.5 injections. Presented by injection frequencies ≤4 and ≥5, VA letter score gains were 0.5 (n = 264) and 6.9 (n = 238) from baseline letter scores of 56.6 and 59.0, respectively. Over 5 years, the incidence of ocular/non-ocular AEs and SAEs was 7.2%/10.1% and 0.3%/5.8%, respectively. No endophthalmitis cases were reported., Conclusions: The LUMINOUS study included patients with DME with more diverse baseline characteristics than those in randomized clinical trials. The 1-year data showed improvement in VA with low number of injections in treatment- naïve patients with DME. Greater VA gains were observed in patients who received ≥5 injections. No new safety findings were identified. LUMINOUS confirms the effectiveness and safety of ranibizumab for the treatment of patients with DME in a real-world clinical practice., Competing Interests: Financial disclosures: Paul Mitchell: Consultant − Abbott, Allergan, Bayer, Genentech, Novartis, Roche. Tom G. Sheidow: Research support – Novartis, Pfizer; Advisory board – Novartis, Alcon, Bayer Michel E. Farah: Consultant: Allergan, Bayer, Novartis, Alcon, Zeiss; Advisor: Cristália, Latinofarma; Research grant: Fapesp, Capes, CNPq Sajjad Mahmood: Research Grant – Bayer, and Novartis; Personal Fees – Alcon, Allergan, Bayer, and Novartis. Angelo M. Minnella: Consultant – Theà Laboratoire; Travel and meeting grant- Allergan, Bayer, Novartis, Theà. Nicole Eter: Consultant – Allergan, Alimera, Bausch & Lomb, Bayer, Heidelberg Engineering, Novartis, Roche. Bora Eldem: Consultant – Alcon Laboratories, Bayer Healthcare, and Novartis. Hassan Al-Dhibi: Consultant –Bayer, AbbVie and Novartis. Wayne Macfadden: Employee of Novartis Pharma AG, Basel, Switzerland at the time of development of the manuscript. Soumil Parikh: Employee of Novartis Pharma AG, Basel, Switzerland. Cornelia Dunger-Baldauf: Employee of Novartis Pharma AG, Basel, Switzerland. Mohamed M. Mahgoub: Nothing to declare. Ursula Schmidt-Erfurth: Consultant – Alcon, Bayer, Boeringher-Ingelheim GmbH, and Novartis. We confirm that this does not alter our adherence to PLOS ONE policies on sharing data and materials.

Peripheral exudative hemorrhagic chorioretinopathy (PEHCR) is a rare retinal vasculopathy that might cause subretinal and/or vitreous hemorrhages. Although the primary etiology is still unknown, choroidal neovascularization is mainly involved in the pathogenesis. The main risk factors are age and systemic hypertension. Ancillary testing such as fluorescein angiography, indocyanine green angiography and ultrasonography can be of great value for diagnosing this entity and distinguishing PEHCR from other lesions as choroidal melanoma and retinal vasoproliferative tumor. Various treatments have been reported including photocoagulation, cryotherapy, intravitreal injection of anti-vascular endothelial growth factor (Anti-VEGF) and surgical intervention as pars plana vitrectomy. This review handles an up-to-date perspective regarding PEHCR., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Middle East African Journal of Ophthalmology.)

Purpose: To provide a focused review of sickle cell retinopathy in the light of recent advances in the pathogenesis, multimodal retinal imaging, management of the condition, and migration trends, which may lead to increased prevalence of the condition in the Western world., Methods: Non-systematic focused literature review., Results: Sickle retinopathy results from aggregation of abnormal hemoglobin in the red blood cells in the retinal microcirculation, leading to reduced deformability of the red blood cells, stagnant blood flow in the retinal precapillary arterioles, thrombosis, and ischemia. This may be precipitated by hypoxia, acidosis, and hyperosmolarity. Sickle retinopathy may result in sight threatening complications, such as paracentral middle maculopathy or sequelae of proliferative retinopathy, such as vitreous hemorrhage and retinal detachment. New imaging modalities, such as wide-field imaging and optical coherence tomography angiography, have revealed the microstructural features of sickle retinopathy, enabling earlier diagnosis. The vascular growth factor ANGPTL-4 has recently been identified as a potential mediator of progression to proliferative retinopathy and may represent a possible therapeutic target. Laser therapy should be considered for proliferative retinopathy in order to prevent visual loss; however, the evidence is not very strong. With recent development of wide-field imaging, targeted laser to ischemic retina may prove to be beneficial. Exact control of intraoperative intraocular pressure, including valved trocar vitrectomy systems, may improve the outcomes of vitreoretinal surgery for complications, such as vitreous hemorrhage and retinal detachment. Stem cell transplantation and gene therapy are potentially curative treatments, which may prevent retinopathy., Conclusions: There is lack of evidence regarding the optimal management of sickle retinopathy. Further study is needed to determine if recent progress in the understanding of the pathophysiology and diagnosis of sickle retinopathy may translate into improved management and outcome.

Purpose : To analyze the role of polymerase chain reaction (PCR) of ocular fluids in management of tubercular (TB) anterior, intermediate, posterior, and panuveitis. Methods: In Collaborative Ocular Tuberculosis Study (COTS)-1 (25 centers, n  = 962), patients with TB-related uveitis were included. 59 patients undergoing PCR of intraocular fluids (18 females; 53 Asian Indians) were included. Results: 59 (6.13%) of COTS-1 underwent PCR analysis. PCR was positive for Mycobacterium TB in 33 patients (23 males; all Asian Indians). 26 patients were PCR negative (18 males). Eight patients with negative PCR had systemic TB. Anti-TB therapy was given in 18 negative and 31 PCR cases. At 1-year follow-up, five patients with positive PCR (15.15%) and three with negative PCR (11.54%) had persistence/worsening of inflammation. Conclusions : Data from COTS-1 suggest that PCR is not commonly done for diagnosing intraocular TB and positive/negative results may not influence management or treatment outcomes in the real world scenario.

Retinopathy of Prematurity (ROP) is one of the leading causes of bilateral blindness in childhood. Early detection and effective treatment can prevent blindness. Efficient and timely screening examination of the retina by an experienced ophthalmologist who deals with preterm neonates with ROP is the mainstay in the management of this disease. All neonatologists and pediatricians who care for these at-risk preterm neonates should also be aware of this timing. This practical guideline intends to provide guidance to ophthalmologists, neonatologists and allied health care professionals in Saudi Arabia on current indications for screening and management of retinopathy of prematurity to prevent or minimize subsequent complications. This practical guideline was led by the National Eye Health Program (NEHP) and Neonatology Services Improvement Program at Ministry of Health (MOH), furthermore it has been solicited and endorsed from both Saudi Ophthalmological Society (SOS) and Saudi Neonatology Society (SNS).

Purpose: We studied the validity, usefulness, and relative cost to detect diabetic retinopathy (DR) and sight-threatening DR (STDR) by using a hand-held electrophysiologic tool compared to digital fundus photography., Method: Patients with diabetes attending the screening unit of King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia were evaluated by "RET eval ", Amsler grid, and digital dilated fundus photography. Fundus images were evaluated by a retina specialist to determine grade of DR. The sensitivity and specificity of STDR and DR screening compared to photography were calculated, as well as "RET eval " combined with Amsler grid testing. The area under the curve (AUC) of "RET eval " screening outcome was calculated., Result: We analyzed data of 400 diabetic patients. The prevalence of DR of any grade was 48.8% (95% confidence interval [CI], 43.9-53.7) while the prevalence of STDR was 27% (95% CI, 22.6-31.4). The outcome of RET eval test was "fail" (based on 20 μV or more amplitude of electrophysiologic spikes) in 351 (87.8%; 95% CI, 84.5-91.0) eyes. The sensitivity of the device was 95.4% and the specificity was 17.5%. Thus, the sensitivity of sequential testing with RET eval and Amsler grid test was 30.1% and the specificity was 80.1%. The AUCs for STDR and DR in general were 76.6% and 50.6%, respectively., Conclusions: "RET eval " is a rapid screening device with excellent sensitivity for detecting STDR. It has potential as a first level screening tool to detect patients who require further evaluation., Translational Relevance: Retinal function, such as electrophysiology, can be used as a new concept for screening for DR.

MERTK is an essential component of the signaling network that controls phagocytosis in retinal pigment epithelium (RPE), the loss of which results in photoreceptor degeneration. Previous proof-of-concept studies have demonstrated the efficacy of gene therapy using human MERTK (hMERTK) packaged into adeno-associated virus (AAV2) in treating RCS rats and mice with MERTK deficiency. The purpose of this study was to assess the safety of gene transfer via subretinal administration of rAAV2-VMD2-hMERTK in subjects with MERTK-associated retinitis pigmentosa (RP). After a preclinical phase confirming the safety of the study vector in monkeys, six patients (aged 14 to 54, mean 33.3 years) with MERTK-related RP and baseline visual acuity (VA) ranging from 20/50 to <20/6400 were entered in a phase I open-label, dose-escalation trial. One eye of each patient (the worse-seeing eye in five subjects) received a submacular injection of the viral vector, first at a dose of 150 µl (5.96 × 10(10)vg; 2 patients) and then 450 µl (17.88 × 10(10)vg; 4 patients). Patients were followed daily for 10 days at 30, 60, 90, 180, 270, 365, 540, and 730 days post-injection. Collected data included (1) full ophthalmologic examination including best-corrected VA, intraocular pressure, color fundus photographs, macular spectral domain optical coherence tomography and full-field stimulus threshold test (FST) in both the study and fellow eyes; (2) systemic safety data including CBC, liver and kidney function tests, coagulation profiles, urine analysis, AAV antibody titers, peripheral blood PCR and ASR measurement; and (3) listing of ophthalmological or systemic adverse effects. All patients completed the 2-year follow-up. Subretinal injection of rAAV2-VMD2-hMERTK was associated with acceptable ocular and systemic safety profiles based on 2-year follow-up. None of the patients developed complications that could be attributed to the gene vector with certainty. Postoperatively, one patient developed filamentary keratitis, and two patients developed progressive cataract. Of these two patients, one also developed transient subfoveal fluid after the injection as well as monocular oscillopsia. Two patients developed a rise in AAV antibodies, but neither patient was positive for rAAV vector genomes via PCR. Three patients also displayed measurable improved visual acuity in the treated eye following surgery, although the improvement was lost by 2 years in two of these patients. Gene therapy for MERTK-related RP using careful subretinal injection of rAAV2-VMD2-hMERTK is not associated with major side effects and may result in clinical improvement in a subset of patients.

Background: To estimate the magnitude and determinants of diabetic retinopathy (DR) among persons with diabetes registered at the employee health department of King Khaled Eye Specialist Hospital (KKESH)., Methods: A retrospective review of medical records was conducted in 2013-14 at KKESH. The case record review extracted demographic, profile of diabetes, diabetic complications, and different blood indices to determine the status of potential risk factors. Ocular profile, especially DR was also noted., Results: Our cohort had 94 staff with diabetes. Eye examination was carried out in 51 (54.8%) of them. The rate of DR was 52% (95% confidence interval (CI) 28-66). Sight-threatening diabetic retinopathy (STDR) (proliferative DR and/or diabetic macular edema) was present in 40% of those examined. Good glycemic control was noted in 42% of participants. Duration of diabetes was associated with DR (P = 0.04). Good glycemic control was negatively associated to DR (odds ratio = 0.2 [95% CI 0.04-0.6]). The coverage of eye screening was 55% only. Laser treatment was given to 80% of STDR cases. The lens opacity and glaucoma rate was 15% and 8.3%, respectively., Conclusions: Low coverage for eye screening and laser treatment to diabetics among the staff of an eye hospital is a matter of concern. The underlying causes of low coverage of screening, digital fundus photography as a screening tool and management should be addressed.

Background: Sympathetic ophthalmia is a rare form of bilateral uveitis that develops following an ocular penetrating injury to one eye. Several cases of sympathetic ophthalmia have been reported after non-penetrating cyclodestructive procedures including Nd:YAG and diode laser cyclophotocoagulation (CPC) but information on inciting factors and outcome is limited. In this case series we investigated the inciting factors, clinical features, course and outcome of six patients who developed sympathetic ophthalmia after diode CPC., Methods: Six patients who developed sympathetic ophthalmia after diode CPC were identified from the King Khaled Eye Specialist Hospital uveitis database between 2008 and 2013. The medical records of these patients were reviewed for demographic information, ocular history, diode laser parameters, clinical presentation, treatment and outcome., Results: Six patients were diagnosed with sympathetic ophthalmia following treatment with diode laser CPC, an incidence of 0.001% during the study period. Two patients had a history of accidental trauma and all patients had undergone at least one ocular surgery that included a variety of procedures. At presentation, two patients had predominantly posterior segment findings in the sympathising eye, one patient had only anterior segment findings and the rest had panuveitis. All patients were treated with topical and systemic corticosteroids and/or immunosupressive therapy that resulted in complete resolution of inflammation and return to baseline vision in the sympathising eye within 2-48 months of treatment., Conclusions: Sympathetic ophthalmia after diode CPC is rare and is seen in patients with a history of intraocular surgery with or without previous trauma. Early recognition of sympathetic ophthalmia and aggressive management with immunosuppressive therapy results in good outcomes for these patients., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Background/Aims. To assess the effect of intravitreal bevacizumab injection (IVBI) for the treatment of macular edema due to infectious and noninfectious uveitides. Design. Retrospective interventional case series. Methods. A chart review was performed on all the patients who were diagnosed with uveitic macular edema (UME) and received 1.25 mg of IVBI at two referral centers in Riyadh, Saudi Arabia. All included patients had their visual acuity and macular thickness analyzed at baseline and at 1 and 3 months following IVBI and any sign of reactivation was noted. Results. The mean age of patients was 41 ± 16 years with a mean followup of 4 ± 1 months. Ten patients had idiopathic intermediate uveitis, 9 patients had Behcet's disease, 10 had idiopathic panuveitis, and twelve patients had presumed ocular tuberculosis uveitis. Following IVBI, the mean LogMAR visual acuity improved from 0.8 ± 0.8 at baseline to 0.4 ± 0.5 at 1 month and 0.3 ± 0.5 at 3 months (P < 0.002, at 3 months). The mean macular thickness was 430 ± 132 μm at baseline. Following IVBI macular thickness improved to 286 ± 93 μm at 1 month and to 265 ± 88 μm at 3 months of followup (P < 0.001, at 3 months). Conclusion. Bevacizumab was effective in the management of UME associated with both infectious and noninfectious uveitides. Intravitreal bevacizumab induced remission of UME with infectious uveitis and had no immunosuppressive effect against infectious agents.

The ocular involvement of tuberculosis is not uncommon but diverse. Rarely, patients initially present with ocular signs that simulate intraocular malignancy. We report 3 cases of isolated presumed choroidal tuberculoma masquerading as intraocular tumor. Two patients had no systemic evidence of tuberculosis with strongly positive purified protein derivative skin test and one patient had evidence of inactive old pulmonary tuberculosis demonstrated by chest computed tomography. Antituberculous regimen including isoniazid 5 mg/kg/day, rifampicin 600 mg/day, ethambutol 15 mg/kg/day, and pyrazinamide 30 mg/kg/day were employed in all cases for 9 months. In addition, oral prednisone 1 mg/kg/day has been given until clinical response was seen, then slowly tapered over 4 months until discontinued. All cases responded well to treatment without complications. Ocular tuberculosis may show challenging clinical presentations, and proper diagnosis and treatment can save the patient's eye and even life.

Objective: Medulloepithelioma is a rare intraocular embryonal neuroepithelial tumour. The study aims at identifying the causes for the delay in diagnosis and treatment., Design: A retrospective study of all cases with histopathologic diagnosis of medulloepithelioma over a period of 25 years., Participants: Six patients with intraocular tumour in 6 eyes., Methods: A data collection sheet is used for clinical and radiologic information based on charts review. The histologic sections are reviewed by a single pathologist., Results: All cases presented initially in childhood with equal sex distribution. There was a delay of up to 5 years until final diagnosis and management. The most common clinical findings were high intraocular pressure and/or cataract in 4 cases, visible mass/uveitis/iris neovascularization and buphthalmos each presenting in 50% of the patients. The diagnosis was established clinically in 2 cased and by ultrasound in 4 cases. The tumour was mostly malignant (4/6) and 2 cases were classified as teratoid (1 benign and 1 malignant). All patients were successfully treated by enucleation with a follow-up period up to 18 years., Conclusions: Medulloepithelioma is a rare intraocular tumour often misdiagnosed and treated as glaucoma or uveitis. Ultrasonography is a useful additional tool for diagnosis. Enucleation seems to be an appropriate method of treatment specially when diagnosis is delayed and malignancy is suspected. Ophthalmologists need to be more familiar with this tumour to allow early clinical recognition and diagnosis., (Copyright © 2011 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Diabetic papillopathy is an uncommon hyperemic optic disc swelling that occurs in patients with long-standing diabetes, is typically self-resolving with minimal effect on vision, and is likely ischemic in origin. The purpose of the current report is to document the response of diabetic papillopathy to intravitreal injection of bevacizumab (Avastin, Genentech Inc, San Francisco, California, USA), a humanized monoclonal antibody to vascular endothelial growth factor.

To identify clinical features and prognostic factors in patients with Fuchs' uveitis (FU). All patients diagnosed with FU at King Khaled Eye Specialist Hospital between 1983 and 2005 were reviewed. Data collected included age, gender, initial and final visual acuities, clinical findings at presentation and during follow-up visits, interval between onset of symptoms and presentation to our institute, ocular complications, details of surgical procedures, and duration of follow-up. One hundred and sixty-six patients (174 eyes) were identified. There were 92 (55.4%) males and 74 (44.6%) females with a mean age of 35.2 ± 10.5 years (range 10-70 years). The mean follow-up period was 57.3 ± 96.7 months (range 1-146 months). The most common presenting symptom was decreased vision (79.9%). Characteristic keratic precipitates (90.2%), diffuse iris stromal atrophy (100%), heterochronmia (13.9%), iris nodules (13.8%), vitreous opacity (50%) (not including sparse vitreous infiltration), cataract (85.6%) and glaucoma (27.6%) were the major clinical findings. The following factors were significantly associated with final visual acuity of 20/40 or better by univariate analysis: shorter interval between onset of symptoms and presentation (p = 0.0087), good initial visual acuity of 20/40 or better (p = 0.0356), absence of glaucoma at presentation (p = 0.0264), no glaucoma surgery (p = 0.0035), and absence of glaucoma and cataract at last visit (p = 0.0126). Logistic regression analysis revealed that final visual acuity of 20/40 or better was negatively correlated with a longer interval between onset of symptoms and presentation (odds ratio (OD) 0.201; 95% confidence interval (CI) 0.0734-0.548), and glaucoma surgical intervention (OD 0.247; 95% CI 0.0796-0.766). Initial visual acuity of 20/40 or better was positively associated with final visual acuity of 20/40 or better (OD 3.34; 95% CI 1.13-9.90). A shorter interval between onset of symptoms and presentation to our institute, better initial visual acuity, and no glaucoma surgery were significantly associated with better final visual acuity.

Purpose: To report unusual ocular manifestations of branchio-oculo-facial syndrome (BOFS) caused by a novel mutation in activating enhancer binding protein 2 alpha (TFAP2A)., Methods: Full ophthalmological evaluation and direct sequencing of TFAP2A., Results: A 10-year-old girl with unusual ocular manifestations of BOFS such as elliptical shaped microcornea and a novel de novo TFAP2A mutation was identified., Conclusions: This report expands the ocular phenotypic spectrum of BOFS and adds to the small number of reported TFAP2A mutations.

Untreated cystoid macular edema (CME) is a major cause for visual loss in intermediate and posterior forms of uveitis. Conventional treatments for inflammatory CME include steroids, nonsteroidal antiinflammatory agents, and carbonic anhydrase inhibitors; however, not all patients respond, even after quieting of the uveitis. Intravitreal injection of bevacizumab, a monoclonal antibody to vascular endothelial growth factor, has recently been suggested as a short-term treatment for inflammatory CME in adults. Because unilaterally injected bevacizumab can reach the contralateral eye via the systemic circulation there may be a contralateral clinical effect; however, the few reports that evaluate this in adults are conflicting. The purpose of this report is to document bilateral reduction of uveitic CME following unilateral intravitreal bevacizumab injection in an 8-year-old girl.

A 37-year-old man developed retinal detachment after laser in situ keratomileusis (LASIK) in both eyes. Causes for the retinal breaks were a horseshoe tear and a giant tear. Both eyes were treated by vitrectomy and scleral buckle. Four months later, the patient developed a recurrent detachment in the right eye with cataract that was managed surgically. Both retinas were flat after a 20-month follow-up period. Bilateral rhegmatogenous retinal detachment after LASIK for correction of myopia can be a serious complication. Patients should be informed about the possibility of this complication. Previous reports of retinal detachments after LASIK and possible related mechanisms were also reviewed.

To describe uncommon association between central retinal artery obstruction and dominant macular drusen in two young female patients. First patient, a 22-year-old female was presented with right central artery obstruction associated with bilateral dominant macular drusen. Systemic evaluation disclosed the presence of mitral valve regurge. Second patient, a 34-year-old female with a previous history of right central retinal artery obstruction diagnosed elsewhere. Fundus exam showed bilateral dominant macular drusen and her systemic evaluation revealed severe rheumatic valve stenosis, moderate aortic regurge with moderate to severe tricuspid regurge and she underwent mitral valve replacement. To the best of our knowledge, the association between central retinal artery obstruction and dominant macular drusen was not previously reported.

Purpose: To present a case of scleral buckle infection with Aspergillus flavus in a tertiary eye center in Saudi Arabia., Methods: A retrospective case report of a 28-year-old Saudi male who presented with a six-month history of conjunctival injection and discharge from the left eye which had undergone uncomplicated conventional retinal detachment surgery, at the King Khaled Eye Specialist Hospital in Riyadh, Saudi Arabia, in the form of cryopexy, subretinal fluid drainage and scleral buckle (grooved segmental sponge and circumferential band with sleeve) for a macula on retinal detachment four years earlier. A diagnosis of infected extruded scleral buckle was made and the buckle was removed., Results: The infected scleral buckle was removed under local anesthesia with administration of sub-conjunctival irrigation of 50 mg solution of Vancomycin, and sub-conjunctival injection of 25mg of Vancomycin. Post operative microbiological studies revealed infection with silver staining of moderate Aspergillus flavus hyphae. Visual acuity of the left eye improved from 20/200 before surgery to 20/60 in the two years follow-up visit., Conclusion: This case report indicates the importance of considering infection with multiple organisms - including fungal ones - in cases of scleral buckle infections in our population.

Purpose: To report the initial diagnosis of leprosy in patients seeking treatment from an ophthalmologist in a tertiary eye care center, its confirmation by histopathologic and polymerase chain reaction analysis, and review of literature., Design: Noncomparative retrospective case series., Participants: Patients with no known history of leprosy who were selected based on either a clinical suspicion or a histopathologic diagnosis and were found to have ocular or periocular lesions suggestive of leprosy., Methods: Review of clinical records of patients with no known history of leprosy seen by an ophthalmologist at a tertiary eye care referral center., Main Outcome Measures: Patient demographics, presenting symptoms and signs, diagnostic studies, complications, and treatment., Results: Among the 6 patients (5 women and 1 man; average age, 55 years), only 2 were found to have leprosy from clinical examination alone. Histopathologic characteristics or demonstration of acid-fast bacilli, suggestive of leprosy, were found in 5 patients. Definite confirmation of leprosy was made by polymerase chain reaction performed on formalin-fixed, paraffin-embedded tissues of 4 patients having suspected leprosy based on clinicohistopathologic examination results., Conclusions: The diagnosis of leprosy relies on the clinical symptom complex, epidemiologic factors, and demonstration of acid-fast bacilli in the tissue sample. Considering that the global leprosy population is 12 million, a patient with leprosy may be found anywhere. The ophthalmologist may be the first one to encounter such patient, in which case suspicion and detection of ocular findings may lead to early treatment of the infection. Polymerase chain reaction may be a new tool in the definite diagnosis of leprosy when suspicion of the diseases is raised by clinicohistopathologic studies.

Isolated retinal venous occlusion rarely occurs in otherwise-healthy children.(1-3) We report a unilateral case of central retinal vein occlusion with secondary proliferative retinopathy and neovascular glaucoma in a 6-year-old girl. Despite an extensive workup, no etiology was identified.

Purpose: To estimate the incidence of sclerotomy-related retinal breaks (SRRBs) and identify possible associated risk factors., Methods: Medical records of patients who underwent pars plana vitrectomy (PPV) from January 1994 to January 1999 at the King Khaled Eye Specialist Hospital in Riyadh, Saudi Arabia, were reviewed. The diagnosis, procedure, and possible associated risk factors were documented, in addition to the presence of SRRBs and time of discovery., Results: Of the 441 eyes, 3.1% developed early SRRBs, while 2.9% of 350 eyes developed late SRRBs. Most of the eyes that developed SRRBs were phakic, and penetrating trauma was found statistically to increase the risk of SRRBs., Conclusions: SRRBs are an uncommon complication of PPV. Early detection and proper management can prevent serious sequelae of such complications.