Peripheral and central retinal vascular changes in asymptomatic family members of patients with familial exudative vitreoretinopathy.

PURPOSE: To evaluate the peripheral vascular changes and effects of these on macular microvasculature in asymptomatic family members of familial exudative vitreoretinopathy (FEVR) patients. METHODS: This is a retrospective study including 61 eyes of asymptomatic family members of FEVR patients. Retinal abnormalities were assessed via ultra-widefield fluorescein angiography (UWF-FA) and optical coherence tomography angiography (OCTA). The eyes were grouped into 3: the first group comprised of eyes with normal findings on UWF-FA; the second group comprised of eyes with abnormal findings on UWF-FA but without any retinal ischemia; and the third group involved eyes with retinal ischemia or neovascularization. RESULTS: Best corrected visual acuity (BCVA) was 20/20 in all eyes. Forty eyes (65.6%) had abnormalities on UWF-FA. The most common feature was peripheral vascular looping, increased tortuosity, and anastomosis (63.9%). ODM/ODD ratio was higher in group 3 compared to groups 1 and 2. Deep foveal VD was lower in group 1 compared to groups 2 and 3. The mean FAZ area and perimeter were smaller in groups 2 and 3 compared to group 1. CONCLUSION: Even asymptomatic family members of FEVR patients may have significant peripheral retinal vascular abnormalities which may be associated with smaller optic disc, macular ectopia, and macular microvascular changes.

Severe anterior persistent fetal vasculature: the role of anterior retinal elongation on prognosis.

PURPOSE: This study aims to investigate surgical outcomes of eyes with severe anterior persistent fetal vasculature (PFV) and the role of associated anatomical anomalies on prognosis. METHODS: This is a retrospective, comparative case series of 32 eyes of 31 patients who underwent vitreoretinal surgery for severe anterior PFV, defined as fibrovascular tissue totally covering the back of cataractous lens. Based on the degree of anterior retinal elongations, cases were classified as follows: group 1, eyes with well-developed pars plana and minor/no abnormalities (n = 11, 34%); group 2, eyes with partially developed pars plana and broad-based elongations (n = 9, 28%); and group 3, eyes with no visible pars plana and fibrovascular membrane having 360° continuity with peripheral retina (n = 12, 38%). Complications and functional and anatomical outcomes were investigated. RESULTS: The median surgical age was 2 (1-12) months. The median follow-up was 26 (6-120) months. Seventy-three percent in group 1 achieved finger counting or better vision with a single surgery and without any pupillary/retinal complication. Groups 2 and 3 required 2.1 ± 0.9 and 2.6 ± 1.2 surgeries on average. Pupillary obliteration and RD occurred in 33% and 22% in group 2 and 58% and 67% in group 3. Retina remained attached after silicone oil removal in 89% of group 2 and 25% of group 3. Phthisis developed in 50% in group 3. CONCLUSION: Peripheral retinal anomalies are common in severe anterior PFV and have a major impact on prognosis. Prognosis is favorable in cases with mild-to-moderate anomalies with appropriate management of possible retinal tears. In eyes with 360° retinal elongations, severe fibrous proliferation and eventual eye loss are common.

COMBINED HAMARTOMA OF THE RETINA AND RETINAL PIGMENT EPITHELIUM AT PEDIATRIC AGE: Surgical Versus Conservative Approach.

PURPOSE: To report outcomes of pediatric patients with combined hamartoma of the retina and the retina pigment epithelium followed up conservatively or after pars plana vitrectomy. METHODS: This retrospective multicenter study included 62 eyes of 59 pediatric patients with combined hamartoma of the retina and the retina pigment epithelium from 13 different international centers with an average age of 7.7 ± 4.7 (0.3-17) years at the time of the diagnosis and having undergone pars plana vitrectomy or followed conservatively. At baseline and each visit, visual acuity values, optical coherence tomography for features and central foveal thickness, and tumor location were noted. Lesions were called as Zone 1, if it involves the macular and peripapillary areas, and the others were called as Zone 2 lesions. RESULTS: Twenty-one eyes of 20 patients in the intervention group and 41 eyes of 39 patients in the conservative group were followed for a mean of 36.2 ± 40.4 (6-182) months. Best-corrected visual acuity improved in 11 (68.8%) of 16 eyes in the intervention group and 4 (12.9%) of 31 eyes in the conservative group ( P < 0.001). The mean central foveal thickness decreased from 602.0 ± 164.9 µ m to 451.2 ± 184.3 µ m in the intervention group, while it increased from 709.5 ± 344.2 µ m to 791.0 ± 452.1 µ m in Zone 1 eyes of the conservative group. Posterior location of tumor, irregular configuration of the foveal contour and ellipsoid Zone defect in optical coherence tomography, subretinal exudate and prominent vascular tortuosity were associated with poor visual acuity. CONCLUSION: Vitreoretinal surgery is safe and effective in improving vision and reducing retinal distortion in Zone 1 combined hamartoma of the retina and the retina pigment epithelium in children.

LONG-TERM EFFECTS OF ANTI-VEGF MONOTHERAPY FOR RETINOPATHY OF PREMATURITY ON THE RETINAL AND REFRACTIVE DEVELOPMENT OF EYE.

PURPOSE: To evaluate vascular maturation of retina, axial, refractive, and foveal development after anti-VEGF treatment including bevacizumab, ranibizumab, and aflibercept in infants with treatment requiring retinopathy of prematurity (ROP) in long term. METHODS: This retrospective chart review study included children with a history of any anti-VEGF monotherapy for ROP in Zone 1 or posterior Zone 2 who have reached at least 24 months of corrected age. The records of ophthalmologic examination including strabismus evaluation, visual acuity measurement, refractive and axial measurements, optical coherence tomography, and fundus fluorescein angiography examinations with RetCam III were reviewed. RESULTS: A total of 36 eyes of 18 children met the inclusion criteria. The mean gestational age at birth was 27.8 2.13 (23-30) weeks, and ocular assessments were performed between 33 and 61.5 (mean: 42.58 8.6) months of corrected age. Treatment was with bevacizumab in 10 eyes, with ranibizumab in 14 eyes, and with aflibercept in 12 eyes. The mean spherical equivalent was -0.25 1.82; axial length was 20.81 0.62 mm. There was no significant difference among three anti-VEGF groups regarding the abnormal vascular retinal development ( P = 0.183) or leakage ( P = 0.842) on fluorescein angiography. CONCLUSION: All the anti-VEGF agents provided similar adequate axial and refractive development as well as similar abnormal vascular development in the peripheral retina. The long-term impact of this abnormally vascularized peripheral retina is a curiosity issue. From this point of view, it is an important need to determine the follow-up period and method after the anti-VEGF treatment for ROP.

FLUID-BASED VISUAL PROGNOSTICATION IN TYPE 3 MACULAR NEOVASCULARIZATION-FLIP-3 STUDY.

PURPOSE: To analyze the effect of fluid on visual acuity in cases of Type 3 macular neovascularization. METHODS: This multicentric, retrospective cohort study included eyes with treatment-naïve Type 3 macular neovascularization. Analysis of fluid in different compartments was performed. Group A included eyes with isolated intraretinal fluid, whereas Group B included eyes with intraretinal fluid in conjunction with subretinal fluid and/or sub retinal pigment epithelial fluid. RESULTS: Eyes in Group A (31, 55.3%) had better best-corrected visual acuity of 20/50 snellen equivalent (0.42 ± 0.31 logarithm of the minimum angle of resolution) at baseline and 20/50 snellen equivalent (0.40 ± 0.28 logarithm of the minimum angle of resolution) at complete resolution compared with Group B with visual acuity of 20/80 snellen equivalent (0.64 ± 0.35 logarithm of the minimum angle of resolution) (P = 0.0181) at baseline and 20/100 snellen equivalent (0.70 ± 0.40 logarithm of the minimum angle of resolution) (P = 0.0021) at complete resolution. Subfoveal atrophy was more in Group B (82.6% 19/23) at complete resolution in comparison to Group A (16/31, 51.6%). Eyes in Group B needed more anti-vascular endothelial growth factor injections (10.3 ± 9.0) for complete resolution compared with Group A (5.7 ± 4.8). CONCLUSION: Intraretinal fluid may be associated with good visual acuity in Type 3 macular neovascularization in contrast to other forms of neovascular age related macular degeneration. Furthermore, intraretinal fluid in isolation may need fewer injections and could probably be associated with less subfoveal atrophy.

MYTHS AND TRUTHS OF THE ASSOCIATION OF RETINAL VASCULAR OCCLUSION WITH COVID-19.

PURPOSE: To critically review data published in the recent past to scrutinize a causal relationship between retinal vascular occlusion and COVID-19. METHOD: A comprehensive literature search was performed on Pubmed with the key words retinal vascular occlusion, retinal vein occlusion, retinal artery occlusion, and COVID-19. RESULTS: A total of 17 case reports were published during this period, and 10 were on retinal vein occlusion and 7 on retinal artery occlusion. Most of the published reports lacked convincing evidences in one or the other aspects, such as insufficient laboratory workup or presence of multiple confounding risk factors. CONCLUSION: In this index article, strength of the data is insufficient to establish a definitive cause-and-effect relationship of retinal vascular occlusive disorders with COVID-19. Hence, clinicians can continue to manage these cases according to the standard guidelines until there are more robust evidences to support this association to alter the diagnostic and treatment modalities.

International Classification of Retinopathy of Prematurity, Third Edition.

PURPOSE: The International Classification of Retinopathy of Prematurity is a consensus statement that creates a standard nomenclature for classification of retinopathy of prematurity (ROP). It was initially published in 1984, expanded in 1987, and revisited in 2005. This article presents a third revision, the International Classification of Retinopathy of Prematurity, Third Edition (ICROP3), which is now required because of challenges such as: (1) concerns about subjectivity in critical elements of disease classification; (2) innovations in ophthalmic imaging; (3) novel pharmacologic therapies (e.g., anti-vascular endothelial growth factor agents) with unique regression and reactivation features after treatment compared with ablative therapies; and (4) recognition that patterns of ROP in some regions of the world do not fit neatly into the current classification system. DESIGN: Review of evidence-based literature, along with expert consensus opinion. PARTICIPANTS: International ROP expert committee assembled in March 2019 representing 17 countries and comprising 14 pediatric ophthalmologists and 20 retinal specialists, as well as 12 women and 22 men. METHODS: The committee was initially divided into 3 subcommittees-acute phase, regression or reactivation, and imaging-each of which used iterative videoconferences and an online message board to identify key challenges and approaches. Subsequently, the entire committee used iterative videoconferences, 2 in-person multiday meetings, and an online message board to develop consensus on classification. MAIN OUTCOME MEASURES: Consensus statement. RESULTS: The ICROP3 retains current definitions such as zone (location of disease), stage (appearance of disease at the avascular-vascular junction), and circumferential extent of disease. Major updates in the ICROP3 include refined classification metrics (e.g., posterior zone II, notch, subcategorization of stage 5, and recognition that a continuous spectrum of vascular abnormality exists from normal to plus disease). Updates also include the definition of aggressive ROP to replace aggressive-posterior ROP because of increasing recognition that aggressive disease may occur in larger preterm infants and beyond the posterior retina, particularly in regions of the world with limited resources. ROP regression and reactivation are described in detail, with additional description of long-term sequelae. CONCLUSIONS: These principles may improve the quality and standardization of ROP care worldwide and may provide a foundation to improve research and clinical care.

Relation of anatomy with function following the surgical treatment of idiopathic epiretinal membrane: a multicenter retrospective study.

PURPOSE: To investigate the prognostic factors associated with functional and anatomical outcomes and to assess the longitudinal course of visual acuity and retinal morphology after vitreoretinal surgery for idiopathic epiretinal membrane (ERM). METHODS: This multicenter, retrospective study included a total of 634 eyes who underwent surgery for idiopathic ERM in 22 academic centers nationwide in Turkey. Data on best-corrected visual acuity (BCVA) and optical coherence tomography features (central foveal thickness (CFT), ERM and foveal contour morphology, ellipsoid zone (EZ) integrity) were collected and compared at baseline, 6-month, 12-month, and 24-month follow-ups. Prognostic factors for functional (having ≥ 20/25 Snellen BCVA) and anatomical (having normal/shallow foveal contour) recoveries after surgery were investigated by means of multivariate regression analyses. A cutoff value of preoperative BCVA optimizing functional recovery was calculated using receiver operating characteristic curve analysis. RESULTS: At a median follow-up of 24 months, 37.4% of the eyes achieved ≥ 20/25 BCVA and 54% regained normal or shallow foveal contour. Functional recovery was more likely in eyes with better baseline BCVA and intact EZ (R2 = 0.356, p < 0.001). The cutoff baseline BCVA value for good visual prognosis was 0.35 logarithm of the minimum angle of resolution (Snellen 20/44) (sensitivity 60%, specificity 85%, p < 0.001). Anatomical recovery was negatively associated with advanced age, higher baseline CFT, foveal herniation-type ERM morphology, and internal limiting membrane (ILM) peeling (R2 = 0.225, p < 0.001). The negative effect of ILM peeling on anatomical recovery was not significant after the first postoperative year (p = 0.05). Mean BCVA values and foveal morphology progressively improved at each visit. Cases with convex baseline foveal contour continued to change towards normal foveal depression over 24 months of follow-up, which took longer than the eyes with shallow/flat contoured cases. One-third of eyes with severe baseline EZ defects showed recovery at follow-up and achieved significantly greater visual acuity gains than the remaining eyes with persistent defects (p < 0.001). CONCLUSIONS: Functional and anatomical restoration of the eyes appears to be a slow process after ERM surgery. This process may take much longer in eyes with worse foveal morphology at baseline. Although photoreceptor disruption may be reversible in some eyes, full functional recovery is unlikely when it persists.

PREOPERATIVE ANATOMICAL FEATURES ASSOCIATED WITH IMPROVED SURGICAL OUTCOMES FOR STAGE 5 RETINOPATHY OF PREMATURITY.

PURPOSE: To describe preoperative anatomical features of Stage 5 retinopathy of prematurity with results of surgery and patient-related and surgery-related factors affecting postoperative success. METHODS: Forty eyes of 33 infants who underwent vitrectomy with lensectomy or lens-sparing vitrectomy for Stage 5 retinopathy of prematurity were enrolled. Stage 5 cases were divided into different groups according to their preoperative anatomical features as follows: A: for eyes with ophthalmoscopically observable total retinal detachment; B: for eyes with total leucocoria associated with funnel retinal detachment; and C: for eyes with very shallow anterior chamber and corneal opacity. Only A and B eyes underwent surgery. The effect of the presence of vascular dilation and tortuosity, preoperative treatment status, surgically induced posterior hyaloid detachment, and sparing the lens on anatomical and functional results was evaluated. RESULTS: Thirty-five percentage of the eyes were in Group A and 65% were in Group B. The mean follow-up was 30.6 months (12-68). Anatomical success was 55.5% for Group A and 15.4% for Group B (P: 0.047), and functional success was 33.3% for Group A and 7.7% for Group B at the third year (P: 0.125). The presence of vascular dilation and tortuosity and postoperative vitreous hemorrhage were found to have negative effects; preoperative treatment and surgical induction of posterior hyaloid detachment were found to have positive effects on anatomical and functional results. CONCLUSION: Surgery for ophthalmoscopically observable retinopathy of prematurity-associated retinal detachment resulted in better anatomical and functional outcomes, and the results are even better in eyes with preoperative treatment, lens-sparing vitrectomy, and surgically induced posterior hyaloid detachment.

VISUAL OUTCOME OF EARLY VITRECTOMY AND INTRAVITREAL ANTIBIOTICS IN ACUTE POSTSURGICAL AND POSTINTRAVITREAL INJECTION ENDOPHTHALMITIS: European Vitreo-Retinal Society Endophthalmitis Study Report Two.

PURPOSE: To evaluate the visual outcome associated with intravitreal antibiotics (IVA) and pars plana vitrectomy (PPV) for acute postprocedure endophthalmitis. METHODS: Data from 237 eyes presenting with acute postprocedure endophthalmitis were collected from 57 retina specialists in 28 countries. All eyes were treated with IVA on the day of presentation. We classified eyes according to the method of treatment used as IVA and early PPV (IVA + PPV within 1 week of presentation) groups. RESULTS: After exclusion of ineligible eyes, data from 204 eyes were analyzed. The mean (SD) age of patients was 62.7 (21.8) years and 69.3 (12.7) years in the IVA and PPV groups, respectively (P = 0.18). Endophthalmitis secondary to cataract, intravitreal injections, PPV, and other intraocular procedures represented 64.2%, 16.2%, 13.7%, and 5.9% of cases, respectively. Intravitreal antibiotics alone were administered in 55 eyes (27.0%), and early PPV was performed in 149 eyes (73.0%). No difference was found between groups in the final visual acuity of ≥20/60 (43.6%, 65 eyes vs. 34.5%, 19 eyes) and ≤counting fingers (30.9%, 46 eyes vs. 36.4%, 20 eyes) for IVA versus early PPV groups, respectively. Vision of light perception (odds ratio = 12.2; 95% confidence interval: 2.0-72.6) and retinal detachment (odds ratio = 7.7; 95% confidence interval: 1.5-409) at baseline were predictive of vision of ≤counting fingers. Retinal detachment at baseline (odds ratio = 20.4; 95% confidence interval: 1.1-372.1) was predictive of final retinal detachment status. CONCLUSION: The current retrospective multicenter cohort of eyes with acute postprocedure endophthalmitis reports similar outcomes after treatment with IVA alone when compared with IVA and early PPV within 1 week of presentation.

Inherited eye diseases in Turkey: Current approaches and future directions.

The aim of this review is to reveal Turkey's current status of medical practice in inherited eye diseases and the necessary steps to improve healthcare services and research activities in this area. Since consanguinity rate is high, disease burden is estimated to be high in Turkey. Universal health insurance system, easily accessible medical specialists, increasing genetic test, and counseling opportunities are the key advantages of Turkey's healthcare system. However, specialized clinics for inherited eye diseases, low-vision rehabilitation services, training of ophthalmologists about the recent developments in ocular genetics, and multidisciplinary translational research are the main headlines needed to be focused for better health services and successful research in Turkey.

Intravitreal dexamethasone implant as an option for anti-inflammatory therapy of tuberculosis uveitis.

INTRODUCTION: Tuberculosis-associated uveitis remains a diagnostic and therapeutic challenge. After diagnosis of tuberculosis and initiation of anti-tuberculosis therapy for tuberculosis uveitis, the clinical responses are favorable. However, at 4-6 weeks of the therapy, there commonly occurs paradoxical deterioration due to an increase in inflammation which is often accompanied by cystoid macular edema. Thus, adjuvant administration of anti-inflammatory regimen should be considered. For this purpose, systemic and periocular steroids, systemic and intravitreal immunosuppressive agents have been tested. Nevertheless, there is no report in the literature about intravitreal dexamethasone slow-release implants for the treatment of this inflammatory condition. METHODS: Case presentation. RESULTS: We presented a tuberculosis uveitis case whose ocular inflammation is partially modified by systemic and periocular steroid injections and then well controlled by the intravitreal dexamethasone implant. CONCLUSION: Intravitreal dexamethasone implant injection seems to be a safe and potent option for the treatment of macular edema secondary to tuberculosis uveitis.

Acute lupus choroidopathy: multimodal imaging and differential diagnosis from central serous chorioretinopathy.

IMPORTANCE: Systemic lupus erythematosus (SLE) is a thoroughly examined multi-organ disease whose common ocular complications are also well documented. However, SLE choroidopathy, being a rare ocular complication, is needed to be better understood, in order to make the differential diagnosis from clinical conditions such as central serous chorioretinopathy and provide adequate prompt treatment. OBJECTIVE: To provide a clear understanding for the clinical course of SLE choroidopathy by the help of enhanced depth imaging optical coherence tomography, fluorescein angiography, and indocyanine angiography. DESIGN: The study is designed as a case presentation, consisting of the physical examination results and the data gathered by the relevant screening methods for each visit, applied by the same ophthalmologists and technicians. SETTING: The visits were organized as same day examinations as needed, in a university hospital which works as a referral center.

Choroidal thickness measurements in migraine patients during attack-free period.

To identify any structural differences in macular choroidal thickness in migraine patients and compare them with that of control subjects by using spectral domain optic coherence tomography (SD-OCT). In this prospective study, choroidal thicknesses of 32 migraine patients during migraine attack-free period and 32 age- and sex-matched healthy subjects were measured using SD-OCT. All patients underwent a complete ophthalmic examination before the measurements. The migraine patients were classified into the migraine with aura group or the migraine without aura group. Migraine severity was assessed by visual analog scale (VAS), migraine disability questionnaire (Migraine Disability Assessment Score (MIDAS), and Wong-Baker faces pain rating scale. Thirty eyes of 32 subjects (31 female and 1 male) in the migraine group and 32 eyes of 32 subjects (31 female and 1 male) in control group were evaluated. In the study group, 16 patients suffered migraine without aura (MWA) and 16 patients were diagnosed as migraine with aura (MA). The mean subfoveal choroidal thickness (SFCT) was 353.3 ± 66.5 µm in the control group versus 304.3 ± 72.9 µm and 276.1 ± 61.4 µm in MWA and MA groups, respectively. The difference in SFCT between the migraine patients and the controls was significant (p < 0.001). Additionally, a moderate correlation was found between SFCT and the VAS score and W baker score (r = 0.48, p = 0.008 and r = 0.43, p = 0.02, respectively). The choroidal thickness was found to decrease significantly not only in migraine patients with aura but also in those without aura during the attack-free period.

Pediatric open-globe injuries: clinical characteristics and factors associated with poor visual and anatomical success.

PURPOSE: We determine the clinical characteristics, visual and anatomical outcomes, and factors associated with poor visual outcome and anatomical success in pediatric open-globe injuries (OGI). METHODS: The records of patients aged 17 years and younger who had undergone surgical intervention after OGI between January 2000 and June 2010 were reviewed retrospectively. RESULTS: In total, 182 patients were enrolled in the study (70.9 % males, 29.1 % females; mean age: 8.6 ± 4.5 years [range 1-17 years]). Injuries occurred mostly at home (45.1 %). Objects most often causing the ocular trauma were sharp objects (74.1 %). Over one-third of the eyes (39.7 %) had final visual acuities of 20/200 or better. Anatomical success was achieved in 76.9 % of eyes. Time between primary globe repair and secondary surgical intervention was 61.4 ± 144 days. Visual outcomes were poor in eyes with initial visual acuity less than for counting fingers, retinal detachment, proliferative vitreoretinopathy, and endophthalmitis. Anatomical success rates were low with retinal detachment. CONCLUSION: Visual outcomes seem to be poor in more than half of the patients despite advanced microsurgical techniques.

CFH Y402H and VEGF Polymorphisms and Anti-VEGF Treatment Response in Exudative Age-Related Macular Degeneration.

PURPOSE: The aim of this study was to evaluate the prevalence of single nucleotide polymorphisms (SNPs) in complement factor H (CFH) Y402H and VEGF rs2146323 and rs699947 in exudative age-related macular degeneration (AMD) and their relationship with intravitreal anti-VEGF treatment response. METHODS: A total of 109 exudative AMD patients and 70 controls were included. Patients were classified as 'good responders' and 'nonresponders' based on the changes in best corrected visual acuity, central foveal thickness, lesion size, and the persistence of retinal hemorrhage after three dosages of anti-VEGF. We examined CFH, VEGF rs2146323 and rs699947 SNPs, and plasma interleukin-6 (IL-6) levels in both groups. RESULTS: In total, 42 patients (38.5%) and 11 controls (15.7%) had homozygote wild genotype TT (p = 0.002). The variant C allele frequency was 45% in controls and 31.7% in patients (p = 0.011). A and C allele frequencies for VEGF rs699947 and rs2416323 were similar between the control and patient groups (p = 0.947, p = 0.378). Both SNPs were similar in responders and nonresponders. No significant difference was detected between plasma IL-6 levels of the control and AMD groups (p = 0.594), but the levels were higher in good responders than nonresponders (p < 0.001). CONCLUSION: CFH Y402H SNP might be protective for AMD in the Turkish population. VEGF rs2146323 and rs699947 SNPs have no relationship to exudative AMD formation, and none of these seem to have any effect on anti-VEGF response.