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To determine the relationship between macular microvascular abnormalities on optical coherence tomography angiography and silent cerebral infarctions (SCIs) on cerebral magnetic resonance imaging in sickle cell disease.Patients (age18 years old) from our previous pediatric sickle cell disease study cohort who had prior optical coherence tomography angiography and brain magnetic resonance imaging were identified. Brain magnetic resonance imaging images were compared with macular optical coherence tomography angiography scans to identify macular vascular density differences between patients with SCI and without SCI.Sixty-eight eyes from 34 patients who underwent optical coherence tomography angiography were evaluated, of whom 28 eyes from 14 patients met the inclusion criteria for this study. Eight patients (57%) with SCI and 6 patients (43%) without SCI were identified. The mean age (17 years in SCI and 16.3 years in non-SCI) was comparable between groups. There was no statistically significant difference in systemic complications. Deep capillary plexus vessel density was lower in the temporal quadrant in patients with SCI (49.3% vs. 53.7%, P = 0.014).Patients with SCI were found to have lower vessel density in the deep capillary plexus compared with those without SCI. This finding suggests that deep capillary plexus vessel density may have utility as an imaging biomarker to predict the presence of SCI.

Choriocapillaris insufficiency may play a role in centripetal retinitis pigmentosa (RP) progression involving the fovea. However, the relationship between choriocapillaris integrity and foveal damage in RP is unclear. We examined the relationship between choriocapillaris flow and the presence of foveal photoreceptor involvement in RP. We categorized the severity of central involvement in RP by the occurrence of foveal ellipsoid zone (EZ) disruption: present (severe RP) or absent (mild RP). Using optical coherence tomography angiography (OCTA, AngioVue, Optovue) in cases and unaffected age-matched controls, we compared vessel density (VD) between the groups using the generalized linear mixed model, controlling for age, gender, and scan quality. Fifty-seven eyes (20 severe RP, 18 mild RP, and 19 controls) were included. Foveal and parafoveal mean outer retinal thickness (µm) were lower in severe RP (fovea: 101.3 ± 14.5; parafovea: 68.4 ± 11.7) than controls (fovea: 161.2 ± 8.9; parafovea: 142.1 ± 11.8; p ≤ 0.001) and mild RP (fovea: 162.0 ± 14.7; parafovea: 116.8 ± 29.4; p ≤ 0.0001). Foveal choriocapillaris VD (%) was lower in severe RP (56.7 ± 6.8) than controls (69.9 ± 4.6; p = 0.008) and mild RP (65.3 ± 5.3; p = 0.01). The parafoveal choriocapillaris VD was lower in severe RP than controls (64.4 ± 5.9 vs. 68.3 ± 4.1; p = 0.04) but no different than in mild RP (p = 0.4). Choriocapillaris flow loss was associated with fovea-involving photoreceptor damage in RP. Further research is warranted to validate this putative association and clarify causation. Choriocapillaris imaging using OCTA may provide information to supplement structural OCT findings when evaluating subjects with RP in neuroprotective or regenerative clinical trials.

To compare the severity of macular vascular changes in children with sickle cell disease (SCD) vs age- and race-matched controls.Cross-sectional study.Children (18 years old) with HbSS and HbS variant (HbSC and HbS thalassemia) genotypes, and their age- and race-matched controls, were recruited between January 2017 and December 2018. All subjects underwent optical coherence tomography angiography (OCTA) scans centered on the fovea and temporal macula. Retinal thickness, superficial capillary plexus (SCP) and deep capillary plexus (DCP) vessel density (VD), and foveal avascular zone (FAZ) size were measured and compared between HbSS and HbS variant vs controls.Thirty-four HbSS, 34 HbS variant (Goldberg staging 0-3 for SCD eyes), and 24 control eyes (total 48 children, aged 5-17 years) were included. Total VD (3-mm ETDRS circle) was lower in HbS variant eyes than in controls for both the SCP (42.9% vs 47.7%, P = .02) and DCP (47.4% vs 52.6%, P = .01). In HbSS eyes, VD was lower in the DCP (47.7%, P = .008) but not in the SCP (45.5%, P = .5), compared to controls. A higher proportion of HbSS (n = 18, 55%) than HbS variant eyes (n = 9, 26%) had pathologic areas of retinal thinning associated with SCP and DCP flow loss (P = .03). However, retinal thickness measurements and FAZ size did not differ between either HbSS or HbS variant group vs controls.Children with SCD have similar retinal thickness but less dense vasculature on OCTA compared to age and race-matched controls, suggesting that microvascular insult may precede structural thinning.

To determine the practice pattern for treating giant retinal tear (GRT) related detachments, and their anatomic and visual outcomes with pars plana vitrectomy (PPV) with or without scleral buckling (SB).Retrospective cohort study.Eyes with GRT detachments repaired from 2008 to 2020 with at least 6 months of follow-up from 7 institutions in North and South America, Europe, and Asia.Eyes repaired using PPV versus PPV/SB were compared.Anatomic and functional outcomes.A comparable number of eyes underwent PPV (n = 101) and PPV/SB (n = 99). Except for history of developmental abnormalities, prior intraocular surgery, and lens status, no differences in baseline demographics, ocular characteristics, or intraoperative surgical adjuncts were observed. The overall single surgery anatomic success (SSAS) at 6 months and 1 year were similar between the groups (82.2% and 77.2% of PPV, and 87.9% and 85.7% of PPV/SB). When stratified by age, the 1-year SSAS rate was higher for PPV/SB (88.5%) than PPV (56.3%) (P = 0.03) for children18 years. For both children and adults, the mean best-corrected visual acuity (BCVA) at baseline did not differ between the PPV and PPV/SB groups. However, for children, mean BCVA at 1 year was better in the PPV/SB than PPV groups (P = 0.001) while for adults, no difference was found between the 2 groups. The mean time to the first redetachment was 7.9 months in the PPV group and 5.5 months in the PPV/SB group (P = 0.8). Proliferative vitreoretinopathy was the most common cause for redetachment (70.4% of PPV and 93.8% of PPV/SB in redetached eyes; P = 0.1). Postoperative complications were also similar between the 2 groups, including ocular hypertension, epiretinal membrane, and cataract formation.PPV and PPV/SB are equally popular among surgeons globally for managing GRT detachments and have comparable anatomic and visual outcomes in adults. In children, PPV/SB is superior to PPV for anatomic and functional success at 1 year. In adults, the relief of traction by the GRT may reduce peripheral traction and obviate the need for an SB. However, in children, a supplemental SB can be beneficial as complete vitreous shaving and posterior hyaloid detachment, and postoperative positioning, are difficult in this group.

To investigate practice patterns and clinical outcomes in the repair of uncomplicated rhegmatogenous retinal detachments (RRD) in a real-world setting over a 10-year period.We compared preferences for scleral buckling (SB), pars plana vitrectomy (PPV), PPV/SB, or pneumatic retinopexy (PR) over time, and examined the 1-year single surgery anatomic success (SSAS) and best-corrected visual acuity (BCVA) at a tertiary academic institution from 2008-2018.Eight hundred eight eyes had RRD repair between 2008-2011 (n = 240), 2012-2014 (n = 271), and 2015-2017 (n = 297). Compared to 2008-2011, PPV was preferred over SB in 2012-2014 (OR: 2.93; 95% CI: 1.86-4.63) and 2015-2017 (OR: 5.94; 95% CI: 3.76-9.38), and over PPV/SB in 2012-2014 (OR: 2.74; 95% CI: 1.65-4.56) and 2015-2017 (OR: 3.16; 95% CI: 31.96-5.12). PR was uncommonly utilized (10%). Younger surgeons (graduating 2010-2017) favored PPV over SB when compared to older surgeons [graduating 1984-2000 (OR: 1.77; 95% CI: 1.18-2.65) and 2001-2009 (OR 1.73; 95% CI: 1.14-2.65)], but similarly selected PPV vs. PPV/SB as their older counterparts (p 0.05). Compared to PPV, SSAS was higher with SB (OR: 1.53; 95% CI: 1.03-2.26) and PPV/SB (OR: 2.55; 95% CI: 1.56-4.17). One-year BCVA was markedly improved compared to baseline only for eyes that achieved SSAS (p 0.001).Over the past 10 years, PPV has become the favored approach to repair uncomplicated RRD and this appears to be driven by younger surgeons' preferences. Given the superior long-term SSAS in SB and PPV/SB as compared to PPV, SB and PPV/SB should be more frequently considered when determining the appropriate repair strategy for uncomplicated RRD.

Purpose: This work aimed to examine the microvasculature of macular fibrosis in Coats disease. Methods: Three boys (aged 3, 4, and 6 years) with Coats disease (stages 2B to 3A2) and macular fibrotic nodules were imaged using optical coherence tomography angiography (OCTA) on the Spectralis spectral-domain OCT tabletop and investigational portable Spectralis Flex module (version 6.9, Heidelberg Engineering). Results: In 2 eyes, a neovascular complex was observed in the avascular slab on OCTA. This neovascular complex had vessels connected to diving vessels from the superficial vascular complex that traveled through the deep vascular complex to the avascular complex. In the third eye, no neovascular complex was observed on OCTA at presentation, but on subsequent examinations fluorescein leakage was observed and cross-sectional OCTA further confirmed the presence of angiographic flow in the nodule. Conclusions: OCTA demonstrates the presence of type 3 neovascularization in fibrotic nodules in Coats disease.

Choriocapillaris insufficiency may play a role in centripetal retinitis pigmentosa (RP) progression involving the fovea. However, the relationship between choriocapillaris integrity and foveal damage in RP is unclear. We examined the relationship between choriocapillaris flow and the presence of foveal photoreceptor involvement in RP.We categorized the severity of central involvement in RP by the occurrence of foveal ellipsoid zone (EZ) disruption: present (severe RP) or absent (mild RP). Using optical coherence tomography angiography (OCTA, AngioVue, Optovue) in cases and unaffected age-matched controls, we compared vessel density (VD) between the groups using the generalized linear mixed model, controlling for age, gender, and scan quality.Fifty-seven eyes (20 severe RP, 18 mild RP, and 19 controls) were included. Foveal and parafoveal mean outer retinal thickness (µm) were lower in severe RP (fovea: 101.3 ± 14.5; parafovea: 68.4 ± 11.7) than controls (fovea: 161.2 ± 8.9; parafovea: 142.1 ± 11.8; p ≤ 0.001) and mild RP (fovea: 162.0 ± 14.7; parafovea: 116.8 ± 29.4; p ≤ 0.0001). Foveal choriocapillaris VD (%) was lower in severe RP (56.7 ± 6.8) than controls (69.9 ± 4.6; p = 0.008) and mild RP (65.3 ± 5.3; p = 0.01). The parafoveal choriocapillaris VD was lower in severe RP than controls (64.4 ± 5.9 vs. 68.3 ± 4.1; p = 0.04) but no different than in mild RP (p = 0.4).Choriocapillaris flow loss was associated with fovea-involving photoreceptor damage in RP. Further research is warranted to validate this putative association and clarify causation. Choriocapillaris imaging using OCTA may provide information to supplement structural OCT findings when evaluating subjects with RP in neuroprotective or regenerative clinical trials.

Purpose To investigate the structural and microvascular changes in the retina and choriocapillaris in patients with sickle cell disease (SCD) demonstrating acute vision loss and acute macular neuroretinopathy (AMN) or paracentral acute middle maculopathy (PAMM) using multimodal imaging including OCT and OCT angiography (OCTA). Design Retrospective case series. Participants Four hemoglobin SS (HbSS) or hemoglobin SC (HbSC) patients who demonstrated vision loss attributed to AMN (n = 2) or PAMM (n = 2). Methods Clinical characteristics including best-corrected visual acuity and multimodal imaging features from fundus photography, fluorescein angiography, OCT, and OCTA were analyzed. Main Outcome Measures Longitudinal changes in the structure and microvasculature of the retina and choriocapillaris were examined. Results In 2 patients with AMN, characteristic hyperreflective changes were observed in the outer nuclear layer with involvement of the ellipsoid and interdigitation zones. In 1 patient, the lesion was located in the foveal avascular zone, and only flow deficits in the choriocapillaris were noted. In the second patient, no flow loss was observed in the superficial capillary plexus (SCP) corresponding to the lesion, but flow loss was seen in the deep capillary plexus (DCP) and choriocapillaris. At the respective 1-year and 4-month follow-up examinations, the hyperreflectivities improved with no ensuing retinal atrophy. Deep capillary plexus and choriocapillaris flow also improved. Of the 2 patients with PAMM, 1 had PAMM in association with branch retinal arteriolar occlusions. In both patients, a hyperreflective band involving the inner plexiform, inner nuclear, and outer plexiform layers was seen in or adjacent to areas of prior macular thinning. These areas corresponded to flow deficits in both the SCP and DCP. At the respective 1-month and 1-year follow-up examinations, the hyperreflective band improved with ensuing atrophy of the middle retinal layers. OCT angiography demonstrated worsening of flow deficits in the corresponding SCP and DCP. Conclusions To the best of our knowledge, this is the first report of AMN and the largest case series of AMN or PAMM in SCD to date. Paracentral acute middle maculopathy may precede macular thinning commonly observed in SCD. OCT angiography is a useful tool to demonstrate associated microvascular changes in AMN and PAMM in SCD.

Coats disease, or Leber multiple miliary aneurysm disease, is characterized by idiopathic retinal vascular telangiectasias that can occur with retinal exudates and exudative retinal detachments. In this chapter, we discuss the clinical features, optical coherence tomography (OCT) features, ancillary testing, and treatment of children with Coats disease.

Myelinated nerve fiber layer is a developmental anomaly that results from an abnormal extension of myelination anterior to the lamina cribrosa of the sclera. In this chapter, we discuss the clinical features, OCT features, ancillary testing, and treatment of children with myelinated nerve fiber layer.

Optical coherence tomography (OCT) imaging can now be done in diverse settings, including the nursery, clinic, and operating room. Compared with imaging in adults, imaging in young children and toddlers presents unique challenges because of the patient’s smaller habitus, limited ability to comprehend directions and to cooperate, and changing axial lengths with age. Special considerations for OCT imaging must be taken for infants and children in a variety of settings. For all imaging sessions, regardless of patient age and the setting, it is important to take certain factors into consideration, as discussed below.

Persistent fetal vasculature (PFV), previously known as persistent hyperplastic primary vitreous (PHPV), is a congenital anomaly that occurs when vessels present during fetal development fail to regress. In this chapter, we discuss the clinical features, optical coherence tomography (OCT) features, ancillary testing, and treatment of children with persistent fetal vasculature.

Different scan protocols are useful when performing optical coherence tomography (OCT) on a cooperative patient who is able to fixate versus on an infant or young child. Although there are overlaps in scan acquisition patterns across different commercial systems, the interpretation of the OCT frequently requires scans of the retina, the optic nerve, the peripapillary retinal nerve fiber layer (RNFL), and the macular map. Before obtaining images on any infant or child, the imager should have a plan or protocol of a series of OCT scans in advance to facilitate efficiency and ensure complete OCT documentation.

Nephropathy may be an independent and contributory risk factor for diabetic macular edema (DME). A 69-year-old man who had previously been treated with panretinal laser photocoagulation for proliferative diabetic retinopathy as well as with steroid and anti-vascular endothelial growth factor injections for DME declined additional treatment for the DME, which was worse in the right eye. The DME was observed without further treatment for the next 36 months. Despite well-controlled blood sugar, blood pressure, and lipid levels, the DME remained unchanged. Peritoneal dialysis was started due to end-stage renal disease. Three months after commencing dialysis, the DME improved significantly. [ Ophthalmic Surg Lasers Imaging Retina . 2017;48:834–837.]

Purpose To report differences in visual acuities among patients with Coats' disease who sought treatment at a tertiary care university-based practice. Design Single-center retrospective cohort study. Participants Patients with Coats' disease diagnosed clinically, angiographically, or both from 1995 through 2015. Methods Patients were divided into 2 groups based on date of presentation: decade 1 (1995–2005) and decade 2 (2006–2015). Main Outcome Measures Visual acuity (VA). Results Thirty-nine eyes of 39 patients were included with 19 eyes presenting in decade 1 and 20 eyes presenting in decade 2. Three patients demonstrated bilateral disease, but only the worse eye was included for analysis. Forty-seven percent of eyes in decade 1 demonstrated advanced stages of disease (stage 3B or worse) compared with 20% of eyes in decade 2. There was a trend for the mean initial presenting VA (±standard deviation) for decade 1 eyes to be worse (2.05±1.29 logarithm of the minimum angle of resolution [logMAR]) than for decade 2 eyes (1.45±0.99 logMAR; P = 0.1). From initial to final follow-up visit, mean VA also worsened for decade 1 eyes ( P = 0.03), but remained stable for decade 2 eyes ( P = 1.0). At the end of follow-up, there was a trend for mean VA for decade 1 eyes (2.28±1.17 logMAR) to be worse than for decade 2 eyes (1.60±1.15 logMAR; P = 0.07). Eight eyes were observed initially in decade 1 compared with 1 eye in decade 2, and only 1 of the observed eyes (in decade 2) developed painful glaucoma requiring enucleation. Decade 2 eyes had a higher average number of procedures per eye (6.5±4.9) compared with decade 1 eyes (1.4±1.7; P Conclusions The earlier presentation of disease in decade 2 suggests improvements in disease detection over time. Furthermore, there was a trend for eyes to have better final VA in this decade. This is due to a combination of factors, including earlier presentation of disease, fewer eyes being observed without treatment, and eyes, when treated, receiving a higher number of procedures.

Optical coherence tomography angiography (OCTA) is a novel, noninvasive imaging modality that allows depth-resolved imaging of the microvasculature in the retina and the choroid. It is a powerful research tool to study the pathobiology of retinal diseases, including inherited retinal dystrophies. In this review, we provide an overview of the evolution of OCTA technology, compare the specifications of various OCTA devices, and summarize key findings from published OCTA studies in inherited retinal dystrophies including retinitis pigmentosa, Stargardt disease, Best vitelliform macular dystrophy, and choroideremia. OCTA imaging has provided new data on characteristics of these conditions and has contributed to a deeper understanding of inherited retinal disease.

PURPOSE: Compared with fluorescein angiography (FA), the gold standard for diagnosing choroidal neovascularization (CNV) activity, optical coherence tomography angiography (OCTA) is non-invasive without risks associated with fluorescein dye use, and may be especially advantageous in the diagnosis and monitoring of children with CNV. METHODS: Eight eyes from eight patients aged 12 months to 18 years were imaged with the investigational Spectralis OCTA (version 6.9, Heidelberg Engineering, Heidelberg, Germany) and the RTVue XR Avanti (Optovue Inc., Fremont, CA, USA). Two patients were imaged during examination under anesthesia while six patients were imaged in the clinic. Demographic information, ocular characteristics, treatment history, and imaging studies (color photos, fluorescein angiography, OCT) were collected and reviewed. RESULTS: Three eyes had active CNV while five had quiescent CNV at the time of imaging. CNV was idiopathic or secondary to trauma, retinal vascular dysgenesis versus retinopathy of prematurity, pigmentary retinopathy, Best vitelliform macular dystrophy, panuveitis, morning glory disc anomaly, and optic disc drusen. OCTA of two active CNV demonstrated presence of a main trunk with multiple fine capillaries, vessel loops, and anastomoses. OCTA was repeated after treatment for two CNV and demonstrated a decrease in size with loss of fine capillaries, vessel loops, and anastomoses. For the third active CNV, OCTA verified flow in the CNV complex despite the uncertainty of FA hyperfluorescence in the setting of grossly abnormal retinal vasculature. The five quiescent CNV all lacked fine capillaries, vessel loops, and anastomoses on OCTA. CONCLUSION: OCTA demonstrates morphological differences between active and quiescent pediatric CNV.

Importance Coats disease is a rare pediatric vitreoretinopathy that can cause devastating visual and anatomic outcomes. Objective To compare optical coherence tomography (OCT) with fundus photographs, fluorescein angiography (FA), and histopathologic findings in Coats disease. Design, Setting, and Participants This retrospective cohort study was conducted in a single tertiary institution (Duke Eye Center) and identified 28 children with Coats disease through a review of medical records from December 2002 to January 2018. Four eyes were obtained from a biorepository for histopathologic analysis. Main Outcomes and Measures Macular OCT, fundus photographs, and FA results were reviewed and compared for morphological changes. These were compared with retinal histopathological findings. Results The mean (SD) age was 9.5 (5.5) years for the 28 children (and 29 eyes) with clinical imaging results, and 24 (86%) were boys. A comparison between imaging modalities revealed OCT features that were not visible in photographs or FA, including exudates in multiple retinal layers (23 [82.1%]), small pockets of subretinal fluid (4 [14.3%]), an outer retinal atrophy overlying fibrotic nodules (7 [25.0%]), and small preretinal hyperreflective OCT dots (25 [89.3%]). Next, a comparison with light micrographs introduced an association of OCT findings with possible pathological features, including hyperreflective linear structures on OCT that appeared consistent with cholesterol crystals, small hyperreflective dots with macrophages, outer retinal tubulations with rosettes, and analogous OCT histopathology features such as intraretinal vessels entering fibrotic nodules and retinal pigment epithelium excrescences under the subretinal fluid. An OCT analysis revealed intraretinal cystoid spaces in 19 eyes, but in 9 of 19 (47.4) this was not associated with cystoid macular leakage; rather, fluorescein leakage was observed from peripheral telangiectatic vessels. Additionally, exudates were intraretinal only (6 [21.4%]) or both intraretinal and subretinal (17 [60.7%]); none were subretinal only. In eyes with follow-up results, new fibrosis developed in 8 of 17 eyes (47.1%). Fibrosis developed in 5 of 5 eyes (100%) with baseline subretinal fluid vs 3 of 12 without (25%; 95% CI, 22%-92%) and in 7 of 9 eyes (77.8%) with subretinal exudates vs 1 of 8 (12.5%) without (95% CI, 16%-89%). Conclusions and Relevance Optical coherence tomography may show the transient and permanent effects of Coats disease on the retina. These results suggest that exudates and fluid in the macular subretinal space appear later in the disease and may result in fibrosis formation. Further studies are needed to confirm if early treatment could prevent vision-threatening macular fibrosis.

Purpose To investigate the association between macular features on spectral-domain optical coherence tomography (SD-OCT) and visual acuity (VA) in Coats' disease. Methods Thirty-nine eyes (39 patients) with SD-OCT from January 1, 2008 to December 31, 2016 were reviewed for SD-OCT features. Central subfield (CSF) SD-OCT findings were analyzed relative to VA (logarithm of the minimum angle of resolution) at baseline and final visit (when follow-up ≥ 6 months) and across visits. Results Mean VA ± standard deviation at baseline (37 eyes) was 0.92 ± 0.82. SD-OCT features associated with worse VA included, for treatment-naïve eyes (n = 21), outer retinal atrophy (ORA) (1.18 ± 0.34 with versus 0.20 ± 0.30 without, P = 0.005), subretinal fluid (SRF) (1.80 ± 0.63 vs. 0.63 ± 0.50, P = 0.008), bright hyperreflectivities (1.23 ± 0.68 vs. 0.52 ± 0.53, P = 0.02), thicker foveal subretinal space (r2 = 0.32, P = 0.01), and CSF (r2 = 0.39, P = 0.007); and for previously treated eyes (n = 16), a compact hyperreflective structure (1.60 ± 0.88 vs. 0.56 ± 0.64, P = 0.02) and ORA (1.34 ± 0.86 vs. 0.30 ± 0.44, P = 0.01). At final follow-up (n = 22), mean VA was 0.81 ± 0.83. Eyes with final VA 20/60, n = 11) more commonly had a compact hyperreflective structure and ORA at baseline and final visit (P < 0.05). Mean change in VA from baseline (n = 20) was −0.20 ± 0.59. Mean improvement in VA (range, −0.525 to −1.127) occurred in eyes with baseline SRF (P = 0.02) and bright hyperreflectivities (P = 0.03). Changes in thickness that correlated with change in VA included those for the foveal subretinal space (r2 = 0.52, P < 0.001) and CSF (r2 = 0.26, P = 0.045). Conclusions A compact hyperreflective structure (fibrosis) and ORA were associated with poor final VA while SRF, bright hyperreflectivities (exudation), and foveal subretinal thickness were associated with VA improvement post treatment.

Background/purpose Iluvien (Alimera Science, Alpharetta, GA) is an injectable, nonbiodegradable, sustained-release 0.19-mg fluocinolone acetonide intravitreal implant. Although currently approved by the Food and Drug Administration only for diabetic macular edema previously treated with a course of corticosteroids without a clinically significant intraocular pressure response, the 0.19-mg fluocinolone acetonide implant could theoretically be used to treat other noninfectious inflammatory conditions including persistent cystoid macular edema because of nondiabetic etiologies. Methods Interventional case report. A 79-year-old man had persistent cystoid macular edema after pars plana vitrectomy in both eyes that was refractory to topical treatments and intravitreal anti-vascular endothelial growth factor. His cystoid macular edema was responsive to preservative-free intravitreal triamcinolone acetonide after which he developed noninfectious endophthalmitis or pseudoendophthalmitis in both eyes precluding further intravitreal triamcinolone acetonide injections. He was subsequently treated with bilateral intravitreal 0.19-mg fluocinolone acetonide implants. Results At the most recent post-treatment follow-up (11 months for the right eye and 13 months for the left eye), the patient demonstrated an improvement in visual acuity, 20/126 to 20/50 in the right eye and 20/80 to 20/40 in the left eye, and in central subfield thickness, 592 μm to 288 μm in the right eye and 565 μm to 287 μm in the left eye, without intraocular pressure elevation. Conclusion The intravitreal 0.19-mg fluocinolone acetonide implant is an effective and potentially safe off-label therapeutic option for persistent nondiabetic cystoid macular edema after vitrectomy.

Alzheimer's disease (AD) and age-related macular degeneration (AMD) are important disorders of aging, but significant challenges remain in diagnosis and therapy. Amyloid-beta (Aβ), found in the brain and a defining feature of AD, has also been observed in the retina in both AD and AMD. While current diagnostic modalities for detecting Aβ in the brain are costly or invasive, Aβ in the retina can be noninvasively and conveniently imaged using modern photonic imaging systems such as optical coherence tomography (OCT). Moreover, since many of these retinal changes occur before degenerative changes can be detected in the brain, ocular amyloid biomarkers could be utilized to detect AD as well as AMD in their earliest stages when therapy may be most effective in halting disease progression. Novel technologies to quantify retinal biomarkers have the potential to facilitate early diagnosis and noninvasive monitoring of disease progression with important therapeutic implications.

Purpose Inflammatory bowel disease may be associated with extraintestinal manifestations. We report a case of severe reactive epithelial atypia resembling ocular surface squamous neoplasia (OSSN) in a patient with ulcerative colitis (UC). Methods Case report. Results A 32-year-old woman presented with sequential, progressive keratoconjunctival lesions in the left and right eyes, and both lesions were excised. Anterior segment optical coherence tomography demonstrated features similar to OSSN, whereas histological examination revealed severe reactive epithelial atypia mimicking severe dysplasia. Shortly after treatment of the second eye, the patient was diagnosed with UC. Residual disease improved dramatically in response to systemic corticosteroids. Conclusions Severe ocular surface epithelial atypia resembling OSSN may be seen in association with UC.

To report differences in visual acuities among patients with Coats' disease who sought treatment at a tertiary care university-based practice.Single-center retrospective cohort study.Patients with Coats' disease diagnosed clinically, angiographically, or both from 1995 through 2015.Patients were divided into 2 groups based on date of presentation: decade 1 (1995-2005) and decade 2 (2006-2015).Visual acuity (VA).Thirty-nine eyes of 39 patients were included with 19 eyes presenting in decade 1 and 20 eyes presenting in decade 2. Three patients demonstrated bilateral disease, but only the worse eye was included for analysis. Forty-seven percent of eyes in decade 1 demonstrated advanced stages of disease (stage 3B or worse) compared with 20% of eyes in decade 2. There was a trend for the mean initial presenting VA (±standard deviation) for decade 1 eyes to be worse (2.05±1.29 logarithm of the minimum angle of resolution [logMAR]) than for decade 2 eyes (1.45±0.99 logMAR; P = 0.1). From initial to final follow-up visit, mean VA also worsened for decade 1 eyes (P = 0.03), but remained stable for decade 2 eyes (P = 1.0). At the end of follow-up, there was a trend for mean VA for decade 1 eyes (2.28±1.17 logMAR) to be worse than for decade 2 eyes (1.60±1.15 logMAR; P = 0.07). Eight eyes were observed initially in decade 1 compared with 1 eye in decade 2, and only 1 of the observed eyes (in decade 2) developed painful glaucoma requiring enucleation. Decade 2 eyes had a higher average number of procedures per eye (6.5±4.9) compared with decade 1 eyes (1.4±1.7; P0.001).The earlier presentation of disease in decade 2 suggests improvements in disease detection over time. Furthermore, there was a trend for eyes to have better final VA in this decade. This is due to a combination of factors, including earlier presentation of disease, fewer eyes being observed without treatment, and eyes, when treated, receiving a higher number of procedures.

Age-related macular degeneration (AMD) represents the leading cause of vision loss in the elderly. Accumulation of lipid- and protein-rich deposits under the retinal pigment epithelium (RPE) heralds the onset of early AMD, but the pathogenesis of subretinal deposit formation is poorly understood. Numerous hypothetical models of deposit formation have been proposed, including hypotheses for a genetic basis, choroidal hypoperfusion, abnormal barrier formation, and lysosomal failure. This review explore the RPE injury hypothesis, characterized by three distinct stages (1) Initial RPE oxidant injury, caused by any number of endogenous or exogenous oxidants, results in extrusion of cell membrane “blebs,” together with decreased activity of matrix metalloproteinases (MMPs), promoting bleb accumulation under the RPE as basal laminar deposits (BLD). (2) RPE cells are subsequently stimulated to increase synthesis of MMPs and other molecules responsible for extracellular matrix turnover (i.e., producing decreased collagen), affecting both RPE basement membrane and Bruchs membrane (BrM). This process leads to progression of BLD into basal linear deposits (BLinD) and drusen by admixture of blebs into BrM, followed by the formation of new basement membrane under the RPE to trap these deposits within BrM. We postulate that various hormones and other plasma-derived molecules related to systemic health cofactors are implicated in this second stage. (3) Finally, macrophages are recruited to sites of RPE injury and deposit formation. The recruitment of nonactivated or scavenging macrophages may remove deposits without further injury, while the recruitment of activated or reparative macrophages, through the release of inflammatory mediators, growth factors, or other substances, may promote complications and progression to the late forms of the disease.