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Acute retinal necrosis is a viral syndrome characterized by a panuveitis with necrotizing retinitis that may be complicated by retinal detachment, vaso-occlusion, optic neuropathy, and other causes of decreased visual acuity. Polymerase chain reaction testing provides a rapid and sensitive method of identifying the viral etiology of acute retinal necrosis, which is most commonly caused by herpes simplex virus type 1, herpes simplex virus type 2, and varicella zoster virus. Prompt diagnosis and treatment is paramount to prevent further vision loss. We review the management of acute retinal necrosis including systemic, local intravitreal, and combination antiviral medications. We also discuss the appropriate and inappropriate use of corticosteroids, laser retinopexy, surgical therapy, and other adjunctive measures.

Background Juvenile idiopathic arthritis-associated uveitis (JIA-U) can lead to poor visual outcomes and impact a child’s quality of life (QOL) and function. Our aim is to identify risk markers of JIA-U and examine differences in the QOL of children with JIA and JIA-U. Methods Rheumatology and ophthalmology record reviews and questionnaires were completed every 4–6 months on 287 children with JIA. We collected arthritis, uveitis, and QOL data. We examined data through last study visit. Results There were 52/287 (18 %) children with JIA-U who were younger at arthritis diagnosis, had oligoarticular persistent JIA, and ANA positive. Confirmed uveitis predictors were age at JIA diagnosis (OR = 0.86) and oligoarticular subtype (OR = 5.92). They had worse vision specific QOL and function, but similar general QOL. Blindness occurred in 17.5 % of children but was more common in African American children compared to non-Hispanic Caucasian children ((5/7 (71 %) vs. 2/29 (7 %), p

To review the available evidence on the effectiveness of prophylactic topical nonsteroidal anti-inflammatory drugs (NSAIDs) in preventing vision loss resulting from cystoid macular edema (CME) after cataract surgery.Literature searches of the PubMed and the Cochrane Library databases were last conducted on January 21, 2015, with no date restrictions. The searches retrieved 149 unique citations. The first author reviewed the abstracts of these articles and selected 27 articles of possible clinical relevance for full-text review. Of these 27 articles, 12 were deemed relevant to analyze in full. Two additional articles were identified from the reference list of the selected articles, and another article was identified from a national meeting. The panel methodologist assigned ratings of level of evidence to each of the selected citations.Nonsteroidal anti-inflammatory drug therapy was effective in reducing CME detected by angiography or optical coherence tomography (OCT) and may increase the speed of visual recovery after surgery when compared directly with placebo or topical corticosteroid formulations with limited intraocular penetration. However, the use of NSAIDs did not alter long-term (≥3 months) visual outcomes. Furthermore, there was no evidence that the benefits observed with NSAID therapy could not be obtained similarly with equivalent dosing of a corticosteroid. The reported impression that there is a pharmacologic drug synergy from the use of both an NSAID and a corticosteroid is not supported by the literature. There is no uniform method of reporting CME in the literature, which prevents accurate assessment of its incidence and response to anti-inflammatory therapies.Cystoid macular edema after cataract surgery has a tendency to resolve spontaneously. There is a lack of level I evidence that supports the long-term benefit of NSAID therapy to prevent vision loss from CME at 3 months or more after cataract surgery. Although dosing of NSAIDs before surgery may hasten the speed of visual recovery in the first several weeks after cataract surgery, there is no evidence that this practice affects long-term visual outcomes. Standardized reporting of CME based on OCT may allow for more uniform quantitation of its incidence and more reliable assessment of treatment outcomes.

Among the survivors of Ebola virus disease (EVD), complications that include uveitis can develop during convalescence, although the incidence and pathogenesis of EVD-associated uveitis are unknown. We describe a patient who recovered from EVD and was subsequently found to have severe unilateral uveitis during convalescence. Viable Zaire ebolavirus (EBOV) was detected in aqueous humor 14 weeks after the onset of EVD and 9 weeks after the clearance of viremia.

Cytotoxic T-lymphocyte-associated antigen is a naturally occurring inhibitor of T-cell costimulation. Monoclonal antibody inhibition of cytotoxic T-lymphocyte-associated antigen with ipilimumab blocks this negative regulator of costimulation, promoting T-cell activation and survival, and leads to melanoma regression. Findings of the Vogt-Koyanagi-Harada (VKH) syndrome, an uveomeningitic syndrome that features neurological, auditory, ophthalmologic, and cutaneous involvement because of autoimmune targeting of melanocytic antigen, have rarely been described in association with melanoma immunotherapy. We describe a case of VKH-like syndrome in a 45-year-old HLA-A02-positive patient with metastatic melanoma treated with ipilimumab. Disruption of immune tolerance by ipilimumab led to melanoma remission while also inciting systemic and ophthalmic autoimmunity toward melanocytic antigen. These observations provide insight into the pathophysiology of the VKH syndrome, and the balance between tumor-associated tolerance and autoimmunity.

CAPN5 mutations have been linked to autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV), a blinding autoimmune eye disease. Here, we link a new CAPN5 mutation to ADNIV and model the three-dimensional structure of the resulting mutant protein. In our study, a kindred with inflammatory vitreoretinopathy was evaluated by clinical eye examinations, DNA sequencing, and protein structural modeling to investigate the disease-causing mutation. Two daughters of an affected mother demonstrated symptoms of stage III ADNIV, with posterior uveitis, cystoid macular edema, intraocular fibrosis, retinal neovascularization, retinal degeneration, and cataract. The women also harbored a novel guanine to thymine (c.750G>T, p.Lys250Asn) missense mutation in exon 6 of CAPN5, a gene that encodes a calcium-activated cysteine protease, calpain-5. Modeling based on the structures of all known calpains revealed the mutation falls within a calcium-sensitive flexible gating loop that controls access to the catalytic groove. Three-dimensional modeling placed the new mutation in a region adjacent to two previously identified disease-causing mutations, all three of which likely disrupt hydrogen bonding within the gating loop, yielding a CAPN5 with altered enzymatic activity. This is the third case of a CAPN5 mutation leading to inherited uveitis and neovascular vitreoretinopathy, suggesting patients with ADNIV features should be tested for CAPN5 mutations. Structural modeling of novel variants can be used to support mechanistic consequences of the disease-causing variants.

The Effects of Youngsters' Eyesight on Quality of Life (EYE-Q) is a novel measure of vision-related quality of life (QOL) and function in children. We aim to determine the validity of the EYE-Q in childhood uveitis.We abstracted medical record data on arthritis and uveitis in a convenience sample of children with juvenile idiopathic arthritis (JIA) and/or uveitis. In addition to the EYE-Q, parents and patients completed questionnaires on overall QOL (Pediatric Quality of Life Inventory [PedsQL]), and physical functioning (Childhood Health Assessment Questionnaire [C-HAQ]).Among 57 children (8 JIA, 24 JIA and uveitis, 25 uveitis alone), 102 ocular examinations were performed within 1 month of completing questionnaires. Uveitis patients had bilateral disease (69%), anterior involvement (78%), synechiae (51%), and cataracts (49%). Children with vision loss in their better eye (visual acuity [VA] 20/50 or worse) had worse EYE-Q (P = 0.006) and PedsQL (P = 0.028) scores, but not C-HAQ scores. The EYE-Q moderately correlated with logMAR VA (rs = -0.43), PedsQL (rs = 0.43), and C-HAQ (rs = -0.45), but was not correlated with anterior chamber cells or intraocular pressure. The PedsQL and C-HAQ did not correlate with VA or cells. There were strong correlations between the parent and child EYE-Q (rs = 0.62). Cronbach's α for the child report was 0.91. The EYE-Q had strong test-retest reliability (rs = 0.75).The EYE-Q may be an important tool in the assessment of visual outcomes in childhood uveitis and an improvement over general measures in detecting changes in vision-related function.

Cecilia S Lee,1 Aaron Y Lee,2 Farzin Forooghian,3 Chris S Bergstrom,1 Jiong Yan,1 Steven Yeh11Section of Vitreoretinal Disease and Surgery, Department of Ophthalmology, Emory Eye Center, Emory University, Atlanta, GA, USA; 2Department of Ophthalmology and Visual Sciences, Washington University in St Louis, St Louis, MO, USA; 3Department of Ophthalmology and Visual Sciences, University ofBritish Columbia, Vancouver, BC,CanadaPurpose: To describe the fundus autofluorescence (FAF) features of the inflammatory ­maculopathies and develop a quantification method for FAF analysis.Methods: This is a retrospective, consecutive case series of patients with inflammatory maculopathies from two tertiary centers. The clinical findings, demographics, and FAF imaging characteristics were reviewed. Foveal autofluorescence (AF) was analyzed. Median and standard deviation (SD) of foveal AF intensity were measured.Results: Thirty eyes of 15 patients were evaluated with both qualitative and quantitative FAF analysis. In acute macular neuroretinopathy, the active phase showed foveal hypoautofluorescence, which became hypoautofluorescent with resolution. In acute posterior multifocal placoid pigment epitheliopathy, multiple lesions with hypoautofluorescent centers with hyperautofluorescent borders were observed in active disease and became hypoautofluorescent with disease convalescence. In multifocal choroiditis and punctate inner choroiditis, the active hyperautofluorescent lesions progressed to inactive, hypoautofluorescent scars. Active serpiginous choroiditis showed hyperautofluorescent borders adjacent to a helicoid-shaped, hypoautofluorescent scar. Active unilateral acute idiopathic maculopathy (UAIM) showed a complex pattern of hypo- and hyperautoflourescence in the macula. The median foveal AF was the greatest in acute macular neuroretinopathy and UAIM among the maculopathies, while the greatest SD of foveal AF intensity was observed in UAIM.Conclusion: The active phase of the majority of inflammatory maculopathies was characterized by hyperautofluorescent lesions. Increased SD of foveal AF correlated with a mixture of hypo- and hyperautoflourescence. Median and SD may be useful metrics in foveal AF and quantifiable values that may be assessed over time as a disease process evolves. Improvements in quantification methods of FAF imaging may allow us to objectively evaluate posterior uveitis.Keywords: posterior uveitis, foveal autofluorescence, quantification, fundus autofluorescence imaging

To describe the long-term outcomes of patients with cytomegalovirus (CMV) retinitis and AIDS in the modern era of combination antiretroviral therapy.Prospective, observational cohort study.Patients with AIDS and CMV retinitis.Immune recovery, defined as a CD4+ T-cell count100 cells/μl for ≥3 months.Mortality, visual impairment (visual acuity20/40), and blindness (visual acuity ≤20/200) on logarithmic visual acuity charts and loss of visual field on quantitative Goldmann perimetry.Patients without immune recovery had a mortality of 44.4/100 person-years (PYs) and a median survival of 13.5 months after the diagnosis of CMV retinitis, whereas those with immune recovery had a mortality of 2.7/100 PYs (P0.001) and an estimated median survival of 27.0 years after the diagnosis of CMV retinitis. The rates of bilateral visual impairment and blindness were 0.9 and 0.4/100 PYs, respectively, and were similar between those with and without immune recovery. Among those with immune recovery, the rate of visual field loss was approximately 1% of the normal field per year, whereas among those without immune recovery it was approximately 7% of the normal field per year.Among persons with CMV retinitis and AIDS, if there is immune recovery, long-term survival is likely, whereas if there is no immune recovery, the mortality rate is substantial. Although higher than the rates in the population not infected by human immunodeficiency virus, the rates of bilateral visual impairment and blindness are low, especially when compared with rates in the era before modern antiretroviral therapy.

To compare the outcomes of combination systemic and intravitreal antiviral therapy vs systemic antiviral therapy alone for treating acute retinal necrosis syndrome (ARN). We hypothesize that combination therapy might result in superior visual acuity (VA) and retinal detachment (RD) outcomes vs traditional systemic antiviral therapy alone.A retrospective, interventional, comparative single-center study of patients with ARN. We reviewed demographic data, herpesvirus diagnoses, polymerase chain reaction (PCR) results, VA, RD, and the use of systemic and intravitreal antiviral therapy. Outcome measures included VA improvement by 2 or more lines, severe visual loss, VA ≤20/200, and RD.We studied 29 eyes of 24 patients, treated from 1987 through 2009. Mean age was 42.6 years and mean follow-up was 44.0 months. Twelve patients (14 eyes) were treated with combined systemic and intravitreal antiviral therapy and 12 patients (15 eyes) with systemic therapy alone. Kaplan-Meier survival analysis revealed that patients receiving combination intravitreal and systemic antiviral therapy were more likely to have VA improved by 2 lines or greater (P=.006). Patients receiving combination therapy also showed a decreased incidence of progression to severe visual loss (0.13/patient-years [PY]) compared to patients receiving systemic therapy alone (0.54/PY, P=.02) and had decreased incidence of RD (0.29/PY vs 0.74/PY, P=.03).Combination oral and intravitreal antiviral therapy may improve visual and functional outcomes in patients with ARN. Clinicians should consider prompt administration of combination systemic and intravitreal antiviral therapy as first-line treatment for patients with clinical features of ARN.

Primary intraocular lymphoma (PIOL) can present with uveitis, vitritis, or chorioretinal lesions. While fluorescein angiography, fundus autofluorescence, and optical coherence tomography may aid in the diagnosis of PIOL, a definitive diagnosis can only be achieved with tissue analysis. The specimen may be obtained with a vitreous or aqueous tap, however, a diagnostic pars plana vitrectomy with acquisition of diluted and undilated vitreous fluid or a chorioretinal biopsy is preferable. Once a specimen is obtained, cytopathology, flow cytometry, immunohistochemistry, gene rearrangement studies with polymerase chain reaction, and cytokine analysis can be performed to confirm the diagnosis of PIOL. Treatment is dependent on the presentation of PIOL and may include systemic chemotherapy, local intravitreal chemotherapy, radiation or a combination of therapies.

BACKGROUND Panuveitis and chorioretinitis in infants with Langerhans cell histiocytosis are rare and fundus photographic documentation is lacking in the literature. METHODS Case report with fundus photography. RESULTS The authors report a case of an infant with disseminated Langerhans cell histiocytosis who presented with panuveitis in the right eye and chorioretinitis in left eye. Treatment with chemotherapy led to resolution of chorioretinal lesions; however, the visual prognosis remained guarded because of the macular involvement and amblyopia. CONCLUSION The authors recommend examination of infants with Langerhans cell histiocytosis for chorioretinal involvement.

Uveitis is the most common extra-articular manifestation of juvenile idiopathic arthritis, which is the most common systemic cause of uveitis in children. Known risk factors for uveitis include antinuclear antibody seropositivity, young age of arthritis onset, specific juvenile idiopathic arthritis subtype and short duration of disease. Risk markers for severe ocular disease include gender, age and complications at initial visit. Due to the risk for vision-compromising sequelae such as cataracts, band keratopathy, glaucoma, vision loss and blindness, an understanding of the risk factors for uveitis development and severe ocular disease is crucial to help prevent serious visual disability and complications. This paper reviews the pathogenesis of uveitis, known risk factors for uveitis development and severe visual outcome, and addresses the need for additional biomarkers of uveitis risk, prognosis and remission.

Optical coherence tomography (OCT) has become an integral tool in the imaging of numerous diseases of the posterior segment. The diagnostic investigation of infectious and noninfectious uveitic conditions often requires multiple imaging modalities in the appropriate clinical context. Modern OCT technology has proved useful not only in the diagnostic investigation of these conditions, but also in monitoring of their clinical course and therapeutic response. Inflammation-induced changes at the level of the retina, retinal pigment epithelium, and choroid can now easily be identified in these conditions using OCT. Prognostic information on visual acuity outcome can also be estimated based on OCT findings. Numerous OCT findings have been described in the setting of the various uveitides. Although none of these findings appear to be pathognomonic for diagnosis of specific uveitic syndromes, in the appropriate clinical context they can add a great deal of information in the diagnosis and management of uveitis.

Inflammatory eye diseases are an important manifestation of many pediatric rheumatologic conditions. Early screening and diagnosis are imperative as these illnesses can not only result in significant visual morbidity but are also an indicator of systemic inflammation. Time to presentation of ocular inflammation varies significantly and can range from many years prior to the onset of systemic symptoms to well after the diagnosis of the rheumatologic disorder. Due to this variability in presentation, careful monitoring by an ophthalmologist is vital to preventing ocular complications and preserving vision. Both local and systemic immunosuppressive medications have been effective in the management of ocular disease. In this review, we will focus on the known ophthalmologic manifestations of common pediatric rheumatologic diseases and discuss recent advances in therapeutic considerations for these conditions.

To describe the risk factors, clinical course, and complications of migration of a dexamethasone (DEX) intravitreal implant (OZURDEX; Allergan, Inc., Irvine, CA) into the anterior chamber and subsequent management strategies.Retrospective, observational case series.Fifteen patients had 18 episodes of migration of the DEX implant into the anterior chamber.The medical records of 15 patients with spontaneous migration of a DEX implant were retrospectively reviewed.Migration of the DEX implant into the anterior chamber.Migration of a DEX intravitreal implant into the anterior chamber occurred in 6 patients who were aphakic, 4 patients with an anterior chamber intraocular lens, 2 patients with a scleral-fixated posterior chamber intraocular lens (PCIOL), 2 patients with a PCIOL, and 1 patient with an iris-fixated PCIOL. All 15 patients had prior pars plana vitrectomy, and 14 patients (93%) had no lens capsule. The average interval from DEX implant injection to detection of the implant migration into the anterior chamber was 13 days (range, 5-44 days). In 14 patients, corneal edema developed. Among those eyes undergoing surgical removal of the implant, earlier intervention reduced the likelihood of permanent corneal edema (0.5 days [from diagnosis of migration to surgical removal of the implant] vs. 5.5 days; P = 0.04). Aspiration was necessary to remove the implant in 6 patients. Among the 14 patients with corneal edema, the corneal edema did not resolve in 10 patients (71%), 6 (43%) of whom required corneal transplantation.Absence of lens capsule and prior vitrectomy are risk factors for migration of the DEX implant into the anterior chamber. Early removal of the implant may be necessary to minimize the risk of chronic corneal edema.

A 34-year-old man with severe, recurrent epistaxis underwent external anterior and posterior ethmoidal artery ligation on the right side. Visual loss from optic neuropathy and complete ophthalmoplegia developed after surgery. Computed tomography revealed surgical clips within the right orbital apex. Emergent removal of the surgical clips and medial wall decompression were performed. Four weeks later, his visual acuity and extraocular motility were unchanged, and diffuse pallor of the right optic disc was observed. Orbital apex syndrome can be a complication of ethmoidal artery ligation. Severe visual loss and ophthalmoplegia may persist despite prompt recognition and treatment.

Introduction of highly active antiretroviral therapy has altered the course of disease for persons infected with human immunodeficiency virus by elevating CD4+ T-lymphocyte levels. Changes in the spectrum of systemic diseases encountered in human immunodeficiency virus-positive individuals are reported in the general medical literature.Retrospective case series.Sixty-one individuals infected with human immunodeficiency virus, who presented with uveitis when the peripheral CD4+ T-lymphocyte count was over 200 cells/μL.Standardized data collection at seven tertiary-referral inflammatory eye disease clinics.Standardization of Uveitis Nomenclature anatomic classification and descriptors, cause of uveitis, and visual acuityPeripheral CD4+ T cell counts varied between 207 and 1777 (median = 421) cells/μL at the time of diagnosis of uveitis. Uveitis was classified anatomically as anterior (47.5%), intermediate (6.6%), anterior/intermediate (16.4%), posterior (14.8%) and pan (14.8%). Specific causes of uveitis included infections (34.4%), with syphilis responsible for 16.4% of all cases, and defined immunological disorders (27.0%); no cause for the inflammation was identified in 34.4% of persons. Visual acuity was better than 6/15 in 66.7% and 6/60 or worse in 11.8% of 93 eyes at presentation, and better than 6/15 in 82.4% and 6/60 or worse in 8.8% of 34 eyes at 1 year of follow-up.Both infectious and non-infectious forms of uveitis occur in individuals who are infected with human immunodeficiency virus and have preserved or restored peripheral CD4+ T cell levels. Individuals who are human immunodeficiency virus-positive and present with uveitis should be evaluated in the same way all patients with uveitis are assessed.

Cystoid macular edema (CME) is a rarely reported side effect of nanoparticle albumin bound (nab)-paclitaxel therapy-an antimitotic agent used for breast cancer. We describe a patient with bilateral CME secondary to Abraxane that was minimally responsive to intravitreal bevacizumab. To our knowledge, this is the first reported case of the use of intravitreal bevacizumab for this condition. A previous report has described the ineffectiveness of concurrent intravenous bevacizumab with Abraxane. This lack of efficacy and knowledge of the mechanism of paclitaxel may provide insights into the mechanisms of CME without angiographic leakage.Retrospective, interventional case report of a patient with bilateral CME after starting Abraxane therapy for recurrent breast cancer treated with intravitreal bevacizumab (1.25 mg/0.05 mL) every 4 weeks. Records were reviewed for visual acuity and macular edema as assessed by spectral-domain optical coherence tomography (SD-OCT).A 73-year-old patient with recurrent, metastatic breast cancer presented with bilateral visual loss 3 months after nab-paclitaxel was initiated. Baseline visual acuities (VA) were 20/50 in the right eye (OD) and 20/80 in the left eye (OS). Fundus exam showed marked CME in both eyes (OU). Fluorescein angiography was notable for the marked absence of petalloid late-phase leakage characteristic of vascular, ischemic, and inflammatory causes of CME. SD-OCT showed marked cystoid spaces predominantly involving the outer and inner nuclear layers with central subfield thicknesses (CST) of 398 μm OD and 441 μm OS. Serial intravitreal bevacizumab injections (OD, 2 injections; OS, 3 injections) were administered on a 4-week basis with an improvement and stabilization of VA at 20/50 OD and 20/70 OS. However, CME on SD-OCT persisted with CST of 492 μm OD and 478 μm OS.The pathogenesis of CME without leakage is poorly understood; however, fluid accumulation in Muller cells due to toxicity has been proposed. The persistence of CME suggests that additional nonvascular endothelial growth factor-mediated mechanisms are involved. Improved understanding of the mechanisms underlying paclitaxel-associated CME is needed, especially in patients with limited systemic options for metastatic carcinoma.

Despite recent downtrends, tuberculosis remains a worldwide public health concern. This review provides an update on recent demographic data, clinical and experimental data, and diagnostic modalities.Quantitative PCR showing mycobacterial load in intraocular fluids may have an emerging role in the diagnosis of ocular tuberculosis when used in combination with ophthalmic features of tuberculosis. Recent investigations in porcine models of ocular tuberculosis have provided valuable insight into the microbiologic, histologic, and clinical features of Mycobacterium tuberculosis infection of the choroid. Differentiating features between sarcoidosis and tuberculosis include tuberculin skin test status, the presence of ocular surface disease, and the anatomic relationship between vasculitis and choroiditis.The diagnosis of presumed ocular tuberculosis remains a clinical challenge with currently available diagnostic modalities. Although newer interferon-γ release assays can distinguish exposure to M. tuberculosis from the Bacille-Calmette-Guérin vaccine strain, they currently lack the specificity to distinguish between latent tuberculosis infection and active tuberculosis. Continued improvement in the currently available molecular diagnostic techniques including quantitative PCR may be valuable in our ability to establish an earlier etiologic diagnosis and institute appropriate antimycobacterial therapy.

Objective The purpose of this study was to evaluate systemic and ophthalmic features associated with Cytomegalovirus (CMV) retinitis in immunocompetent patients. Design Retrospective chart review. Participants Nine patients with CMV retinitis who were clinically immunocompetent at the time of diagnosis. Methods Retrospective chart review of patients evaluated at the Casey Eye Institute, University of Washington, Bascom Palmer Eye Institute and University of Toronto. IRB approval was obtained. Retrospective chart review of patients evaluated at the Casey Eye Institute, University of Washington, Bascom Palmer Eye Institute and University of Toronto. IRB approval was obtained. Results Seven of the nine patients in our series were 60 years of age or above. Three patients had intravitreal steroid and VEGF inhibitor injections in the preceding year in the affected eye. Five of the nine patients were initially misdiagnosed to have acute retinal necrosis (ARN) for which they were on Valacyclovir for a variable period of time. Seven patients had anterior chamber and eight had vitreous inflammation. Six of the nine patients exhibited vascular attenuation or obliteration. All patients showed improvement in retinitis within weeks following therapy with intravitreal foscarnet and/or ganciclovir and systemic valganciclovir.

To describe the outcomes of different treatment approaches for cytomegalovirus (CMV) retinitis in the era of highly active antiretroviral therapy (HAART).Prospective cohort study, the Longitudinal Study of the Ocular Complications of AIDS.A total of 250 patients with CMV retinitis and a CD4+ T-cell count100 cells/μl (n = 221) at enrollment or incident retinitis (n = 29) during cohort follow-up.The effects of systemic therapy (vs. intraocular therapy only) on systemic outcomes and the effect of intraocular therapies (ganciclovir implants, intravitreal injections) on ocular outcomes were evaluated.Mortality, CMV dissemination, retinitis progression, and treatment side effects.Regimens containing systemic anti-CMV therapy were associated with a 50% reduction in mortality (adjusted hazard ratio [HR], 0.5; 95% confidence interval [CI], 0.3-0.7; P = 0.006), a 90% reduction in new visceral CMV disease (adjusted HR, 0.1; 95% CI, 0.04-0.4; P = 0.004), and among those with unilateral CMV retinitis at presentation, an 80% reduction in second eye disease (adjusted HR, 0.2; 95% CI, 0.1-0.5; P = 0.0005) when compared with those using only intraocular therapy (implants or injections). Compared with systemic treatment only, regimens containing intravitreal injections had greater rates of retinitis progression (adjusted HR, 3.4; P = 0.004) and greater visual field loss (for loss of one half of the normal field, adjusted HR, 5.5; P0.01). Intravitreal implants were not significantly better than systemic therapy (adjusted HR for progression, 0.5; P = 0.26; adjusted HR for loss of one half of the visual field, 0.5; P = 0.45), but the sample size was small. Hematologic and renal side effect rates were similar between those groups with and without systemic anti-CMV therapy. The rate of endophthalmitis was 0.017 per eye-year (EY) (95% CI, 0.006-0.05) among those treated with intravitreal injections and 0.01 per EY (95% CI, 0.002-0.04) among those treated with an implant.In the HAART era, systemic anti-CMV therapy, while there is immune compromise, seems to provide benefits in terms of longer survival and decreased CMV dissemination.Proprietary or commercial disclosure may be found after the references.

PURPOSE Oxygen-induced retinopathy in the mouse is the standard experimental model of retinopathy of prematurity. Assessment of the pathology involves in vitro analysis of retinal vaso-obliteration and retinal neovascularization. The authors studied the clinical features of oxygen-induced retinopathy in vivo using topical endoscopy fundus imaging (TEFI), in comparison to standard investigations, and evaluated a system for grading these features. METHODS Postnatal day (P)7 mice were exposed to 75% oxygen for five days to induce retinopathy or maintained in room air as controls. Retinal vascular competence was graded against standard photographs by three masked graders. Retinal photographs were obtained at predetermined ages using TEFI. Postmortem, retinal vaso-obliteration was measured in whole mounts with labeled vasculature, and retinal neovascularization was quantified in hematoxylin- and eosin-stained ocular cross sections. RESULTS Fundus photography by TEFI was possible from P15, when retinal vascular incompetence, including dilatation and tortuosity, was significant in mice with oxygen-induced retinopathy in comparison to controls. Vascular incompetence peaked in severity at P17 and persisted through P25. Comparison with in vitro analyses indicated that vascular changes were most severe after retinal avascularity had begun to decrease in area, and coincident with the maximum of retinal neovascularization. A weighted Fleiss-Cohen kappa indicated good intra- and interobserver agreement for a 5-point grading system. CONCLUSIONS Topical endoscopy fundus imaging demonstrates retinal vascular incompetence in mice with oxygen-induced retinopathy. The technique complements standard postmortem analysis for following the course of the model. TRANSLATIONAL RELEVANCE Topical endoscopy fundus imaging has application in the evaluation of novel biologic drugs for retinopathy of prematurity.

To determine the prevalence of serous retinal detachments (SRD) using optical coherence tomography (OCT) in a large database of patients with uveitis from a tertiary referral setting, to describe clinical features of patients with SRD, and to ascertain retinal architectural features found in association with SRD. Main outcome measures Prevalence of SRD in uveitis patients imaged with OCT, correlation of visual acuity with SRD, anatomic subtypes of uveitis identified, and association of SRD with various subtypes of macular edema (focal and diffuse) and retinal architectural abnormalities. Design Retrospective, single-setting cross-sectional study of all OCTs in a digital imaging base ordered on patients from a tertiary referral uveitis clinic between July 2006 and March 2008. Results SRD were identified in 17 of 111 uveitis patients (15 %) reviewed; bilateral SRD were found in 5 of 17 patients (29 %). Intermediate uveitis was the most common disease association (47 %), but other conditions identified included Vogt-Koyanagi-Harada syndrome, multifocal choroiditis/panuveitis, and sarcoidosis. Retinal architectural features identified in association with SRD included focal macular edema (59 %), diffuse macular edema (50 %), any intraretinal edema (77 %), both diffuse and focal macular edema (32 %), and retinal pigment epithelial alteration (27 %). Moderate or severe visual impairment, defined as visual acuity 20/50 or poorer was seen in 71 % of patients with SRD. Poorer visual acuity was correlated with increased central subfield thickness in patients with SRD (r2=0.41, p, This work is supported in part by an Unrestricted Grant from Research to Prevent Blindness (Casey Eye Institute, Emory Eye Center), the Stan and Madelle Rosenfeld Family Trust (JTR), the William and Mary Bauman Foundation (JTR), the William C. Kuzell Foundation (JTR), and the Ronald G. Michels Fellowship Foundation (SY).

Susac syndrome features the triad of multiple branch retinal artery occlusions, hearing loss due to microinfarctions of the cochlea, and encephalopathy due to brain microangiopathy.1 Initial misdiagnosis as multiple sclerosis (MS) is not uncommon.1,2 Magnetic resonance imaging evidence of microinfarctions of the corpus callosum and multiple yellow retinal arterial wall plaques on fundus examination are helpful in differentiating this condition from demyelinating diseases.2,3

Male breast carcinoma is a rare entity that often goes undiagnosed until advanced stages. The authors describe the case of a patient with profound vision loss who was found to have bilateral choroidal metastases as well as advanced cerebral metastatic disease. Further medical work-up revealed widespread infiltrative ductal breast carcinoma. Given the presence of large occipital lobe lesions, the etiology of the patient’s vision loss was thought to be cortical blindness. Prompt diagnosis and neurologic evaluation with this presentation is crucial because it can be associated with significant morbidity and mortality.

Cytomegalovirus (CMV) retinitis typically presents as a hemorrhagic, full-thickness retinitis in immunosuppressed individuals, often in the setting of HIV infection. The management of CMV retinitis includes systemic and locally administered intravitreal antivirals (i.e. foscarnet or ganciclovir), as well as the surgical intravitreal ganciclovir implant. In chronically immunosuppressed patients (i.e. transplant recipients, cancer chemotherapy) and in HIV/AIDS patients who fail to immune reconstitute, chronic CMV prophylaxis with valganciclovir may lead to ganciclovir- and foscarnet-resistant CMV strains. Moreover, the identification of drug-resistant CMV may influence the choice or dosing of antiviral medication.1–2 Ganciclovir-resistance is classified into genotypic resistance defined as CMV DNA harboring a mutation known to confer antiviral resistance or phenotypic resistance meaning that ganciclovir at a therapeutic dose fails to exceed the concentration required to inhibit 50% of CMV growth on viral culture media. We characterize a series of patients with CMV retinitis who were evaluated for genotypic ganciclovir-resistance using PCR-based analysis of ocular fluids and describe its influence on management.

To identify factors associated with best-corrected visual acuity (BCVA) presentation and 2-year outcome in 479 intermediate, posterior, and panuveitic eyes.Cohort study using randomized controlled trial data.Multicenter Uveitis Steroid Treatment (MUST) Trial masked BCVA measurements at baseline and at 2 years follow-up used gold-standard methods. Twenty-three clinical centers documented characteristics per protocol, which were evaluated as potential predictive factors for baseline BCVA and 2-year change in BCVA.Baseline factors significantly associated with reduced BCVA included age ≥50 vs50 years; posterior vs intermediate uveitis; uveitis duration10 vs6 years; anterior chamber (AC) flaregrade 0; cataract; macular thickening; and exudative retinal detachment. Over 2 years, eyes better than 20/50 and 20/50 or worse at baseline improved, on average, by 1 letter (P = .52) and 10 letters (P.001), respectively. Both treatment groups and all sites of uveitis improved similarly. Factors associated with improved BCVA included resolution of active AC cells, resolution of macular thickening, and cataract surgery in an initially cataractous eye. Factors associated with worsening BCVA included longer duration of uveitis (6-10 or10 vs6 years), incident AC flare, cataract at both baseline and follow-up, pseudophakia at baseline, persistence or incidence of vitreous haze, and incidence of macular thickening.Intermediate, posterior, and panuveitis have a similarly favorable prognosis with both systemic and fluocinolone acetonide implant treatment. Eyes with more prolonged/severe inflammatory damage and/or inflammatory findings initially or during follow-up have a worse visual acuity prognosis. The results indicate the value of implementing best practices in managing inflammation.

Objective Safety and efficacy of daclizumab during an 11-year period. Design Structured, retrospective chart review. Participants Thirty-nine patients. Methods Patients with chronic, noninfectious intermediate and/or posterior uveitis. Results Thirty-nine patients (78 eyes) were treated for a mean of 40.3 months. Visual acuity improved by ≥2 lines in the better eye in 7 patients (18.4%) and worsened by 2 lines in 6 patients (15.8%) with a mean of 2.8 Snellen lines of vision lost per eye. Six eyes with vitreous cell less than grade 2 lost 2 lines of vision and 7 eyes with less than grade 2 vitreous cell improved 2 lines. Mean number of immunosuppressive medications per patient decreased from 1.89 medications/patient to 1.17 medications/patient. The average number of periocular injections per patient was 1.46 (range, 0-9). The mean number of flares was 2.05/patient (range, 0-12), with the rate being 0.62 flares per patient-year. Four patients developed cancer during the course of this study. Mean time to onset of malignancy was 26 months and the mean age in this group was 49 years. Conclusions Daclizumab demonstrated efficacy in the reduction of concomitant immunosuppressive medication, stabilization of visual acuity, and the prevention of uveitic flares in most cases. Dermatologic complications were the most frequently observed adverse event in our series. Four patients developed solid tumor malignancies during this 11-year period.

Sir, Progressive outer retinal necrosis (PORN) is a herpetic retinitis characterized by multifocal deep retinal lesions progressing to confluent necrosis and a high likelihood of retinal detachment. The visual prognosis in patients with PORN is extremely poor, with final visual acuity of no light perception reported in up to 67% in some series.1, 2 We report a therapeutic challenge of AIDS-associated viral retinitis in which the ganciclovir implant contributed to the eradication of PORN recalcitrant to systemic and intravitreal antivirals.

Cystoid macular edema (CME) is seen in 33% of patients with uveitis and requires local or systemic treatment for resolution.1 Standard systemic immunosuppressive medications can be associated with significant adverse effects.2–4 Management and prevention of the iatrogenic complications of immunosuppressive therapy accounts for most of the medical resources devoted to these individuals. Consequently, an effective treatment with a safer side effect profile is highly desirable. Efalizumab (Raptiva; Genentech Inc., San Francisco, CA) is a humanized form of a murine IgG1 antibody directed against CD11a, the α-subunit of lymphocyte function–associated antigen-1 (LFA-1).5 LFA-1 expression is increased in memory T-cells, and ICAM-1 is expressed on vascular endothelial cells at sites of inflammation in a variety of T-cell-mediated disorders, including uveitis.6 Both LFA-1 and intercellular adhesion molecule (ICAM)-1 are thought to play important roles in the pathogenesis of autoimmune disorders, and prior studies have shown that interference with adhesion molecule function, including CD11a, decreases histologic and clinical expression of endotoxin-induced uveitis.7,8 In vitro studies have shown that by binding to CD11a, efalizumab can inhibit T-cell activation, T-cell trafficking, and T-cell adhesion without depleting the T cells.9 Efalizumab was approved for use in moderate to severe plaque psoriasis in adults.10–12 Human NK cells have typically been identified as CD56+CD3− lymphocytes, with two subsets of human NK cells identified based on expression levels of cell surface CD56, CD56dim, and CD56bright.13–15 CD56bright regulatory NK cells have been proposed to play a regulatory role in immune responses based on their lower cytotoxic potential, higher secretion of downregulatory cytokines, and unique surface receptor expression profile.14,16–18 Previous studies have demonstrated that a low-dose infusion of recombinant human IL-2 selectively induces the CD56bright regulatory NK subset.19 Studies in the Laboratory of Immunology of the National Eye Institute and other work have revealed that infusion of daclizumab, a humanized IL-2R (CD25)-blocking antibody, also induces upregulation of this subset in both uveitis and multiple sclerosis.20,21 Patients with active uveitis had a significantly lower level of CD56bright regulatory NK cells in their peripheral blood compared with normal donors.20 The expansion of CD56bright cells correlates with decreased ocular and brain inflammation.20,21 Induced CD56bright regulatory NK cells have the ability to secrete large amounts of IL-10, whereas CD56dim NK cells do not, suggesting that the induction of the CD56bright regulatory NK cells may have a beneficial effect on the remission of active uveitis.20 This study was designed to evaluate the safety and potential efficacy of subcutaneous humanized anti-CD11a antibody treatments for macular edema associated with uveitis, while reducing or eliminating standard medications commensurate with the standard of care.

Background/Purpose: The Effects of Youngsters Eyesight on Quality of Life (EYE-Q) is a valid instrument that measures visual function and vision related quality of life (QOL) in children with ocular disease. Our objective is to determine whether the EYE-Q is valid in children with uveitis. Methods: Children with uveitis (JIA-associated uveitis (JIA-U) and idiopathic uveitis (IU)) who have had a slit lamp examination within one month of completing the EYE-Q were included. We collected data related to uveitis clinical characteristics and the ocular examination (LogMAR visual acuity (VA), intraocular pressure (IOP), and # of cells) in the worse and better eye. Questionnaires were completed on overall QOL (Pediatric QOL Inventory—MPedsQL), and vision (EYE-Q). We determined associations between the EYE-Q and the ocular exam using Spearman's correlation coefficients. Results: There were 19 children with JIA-U and 17 with I-U included with a total of 42 ocular examinations performed. Females comprised 75%, with a mean age of diagnosis of 5.6 (SD = 3.6) years. There were 29 (80%) children with bilateral disease, with varied uveitis locations (anterior (69%), intermediate (25%), and panuveitis (6%)), and numerous ocular complications (synechiae (53%), cataracts (44%), band keratopathy (25%), cystoid macular edema (22%), and glaucoma (6%)). At time of examination, 28% were being treated with oral methotrexate, 52% with subcutaneous methotrexate, 14% with infliximab, and 2% with adalimumab. There were 71% of children on eye drops, 59% on ocular steroids, and 2% on mydriatics. Mean PedsQL score was 80.9 ± 15.6. Mean EYE-Q score was 3.1 ± 0.6. Slit lamp examination showed worse LogMAR VA in children with I-U compared to JIA-U but similar IOP and # of cells (Table 1). For both the child and parent reports, the EYE-Q had moderate correlations with LogMAR VA and the PedsQL, and mild to no correlations with # of cells and IOP (Table 2). The PedsQL had mild to no correlations with LogMAR VA, # of cells and IOP. Table 1. Ocular examinations in children with uveitis Ophthalmology exam Uveitis (n=21) JIA-U (n=21) Worse eye Intraocular pressure, mean±SD 17.9 ± 7.9 17.6 ± 7.3 LogMar visual acuity, mean±SD 0.75 ± 0.67 0.18 ± 0.20 Slit lamp exam Cells, N (%) 0 (

A healthy 31-year-old woman developed decreased vision due to vitreous hemorrhage from 2 retinal vascular lesions. Because of the patient's desire for visual rehabilitation and the concern for von Hippel-Lindau (VHL) disease-associated retinal hemangioblastomas, pars plana vitrectomy and endoresection of both lesions were performed. Surgical techniques included the use of chandelier illumination to enable bimanual manipulation of tissue, endolaser around the tumor prior to resection, endodiathermy to cauterize the tumor's feeder vessels, and long-acting gas tamponade following the retinectomy. Histopathology showed a vascular anomaly within the retina that consisted of multiple large vessels with multiple lumens and no evidence of VHL-associated retinal hemangioblastoma. Long-term follow-up revealed no evidence of recurrent disease. This surgical technique may be valuable in select patients to differentiate between retinal vasoproliferative tumors and VHL disease-associated retinal hemangioblastomas for diagnostic, therapeutic, and counseling purposes.

Background/aims Chronic natural killer lymphocytosis (CNKL) has been associated with systemic autoimmunity; however, its association with scleritis or ocular autoimmunity has not been characterised. The natural killer (NK) cell function and immunophenotype of a patient with CNKL who developed bilateral scleritis and multiple systemic autoimmune findings were evaluated. Methods The ophthalmic records of a patient with CNKL and scleritis were reviewed over a 6-year period. Flow cytometry was performed to evaluate T cell, NK and B cell populations. NK cellular functions (ie, NK cytotoxicity and cytokine/chemokine production following interleukin 2 (IL2) stimulation) were evaluated. Results A 56-year-old woman with vitiligo, psoriatic arthritis, thyroiditis, erythema nodosum, bilateral anterior scleritis and Sjogren syndrome was managed with multiple immunosuppressive medications, including prednisone, mycophenolate mofetil and methotrexate. Flow cytometry showed a persistent elevation of CD56+CD3− NK cells greater than 40%, which was consistent with CNKL. NK cell cytotoxicity assay identified a deficiency of K562 cell lysis in the patient (1.46 mean-fold greater in control vs patient). NK cytokine/chemokine production following IL2 stimulation was also deficient (2.5–32.5-fold greater in control). Cytokines/chemokines assessed included pro-inflammatory (interferon γ, tumor necrosis factor α, IL1, monocyte chemotactic protein 1) and immunoregulatory cytokines (IL4, IL5 and IL10). An abnormal elevation of TCRα/β+ CD3+CD4−CD8− T cells suggestive of autoimmune lymphoproliferative syndrome was observed; however, apoptosis dysfunction was not found. Conclusion The association of increased but dysfunctional NK cells in the context of multiple systemic and ocular manifestations suggests a role of NK cells in the pathogenesis of our patient9s disease. Further studies regarding NK cell dysfunction and ocular autoimmunity are needed.

The pathogenic mechanisms underlying uveitis syndromes continue to be evaluated using animal models and in the clinical setting. As the complex interactions between ocular immune cells, proinflammatory cytokines, chemokines and cellular adhesion molecules are uncovered, targeted therapies directed against these immune mediators will continue to be developed. Traditional immunosuppressive medications, such as corticosteroids and steroid-sparing immunomodulatory agents, have demonstrated efficacy in the treatment of uveitic syndromes, but side effects and drug toxicities often limit the use of these medications. The biologic agents, a newer class of medications, target specific immune pathways and have demonstrated efficacy in rheumatologic, dermatologic and neurologic conditions. Biologic therapies (e.g., TNF-alpha inhibitors and IL-2 receptor inhibitor) targeting ocular immune cell surface receptors, cytokines and chemokines continue to be developed and have shown promise in the treatment of uveitis and ocular inflammatory diseases. Clinical and basic aspects of monoclonal antibody therapy for uveitis are presented in this review. Additional studies are needed to further evaluate the role of monoclonal antibodies in the therapeutic armamentarium for uveitis.

The purpose of this study was to describe fundus autofluorescence imaging features of cytomegalovirus (CMV) retinitis and to correlate fundus autofluorescence features with clinical activity.A retrospective case series was undertaken to evaluate nine eyes of six patients with active CMV retinitis. Patients were evaluated with a comprehensive ophthalmic examination, fundus autofluorescence imaging, and fundus photography. Oral valganciclovir, intravitreal ganciclovir, intravitreal foscarnet, or an ganciclovir implant was administered as clinically indicated.In all nine eyes with active CMV retinitis, a hyperautofluorescent signal on fundus autofluorescence imaging was correlated spatially with the border of advancing CMV retinitis. Stippled areas of alternating hyperautofluorescence and hypoautofluorescence were observed in regions of retinal pigment epithelium atrophy from prior CMV retinitis. In three eyes with subtle CMV reactivation, a hyperautofluorescent border was helpful in the detection and localization of active CMV retinitis. In another patient, diffuse, punctate hyperautofluorescence after intravitreal ganciclovir and foscarnet was a concern for medication-related toxicity.Fundus autofluorescence imaging was valuable in highlighting areas of active CMV retinitis in all patients in this series, including two patients with subtle clinical features. Fundus autofluorescence may be useful as an adjunctive imaging modality for the detection of CMV activity and aid in our understanding of the structural changes during episodes of CMV retinitis.

Three patients with aplastic anemia were evaluated by the ophthalmology service within 2 months of the aplastic anemia diagnosis for bilateral visual loss. The mean age of diagnosis of aplastic anemia was 14.3 years (range: 5 to 19 years) and the mean follow-up was 25 months (range: 15 to 44 months). All 6 eyes demonstrated choroidal ischemia and vitreous hemorrhage. Pars plana vitrectomy was performed in four eyes of two patients for non-clearing vitreous hemorrhage; one patient was observed. Successful anatomic outcomes were achieved in 3 of 4 eyes that underwent vitrectomy. Initial visual acuity ranged from 20/80 to bare light perception and final visual acuity ranged from 20/20 to no light perception. All patients received immunosuppressive therapy including cyclosporine and anti-thymocyte globulin, and two underwent hematopoietic stem cell transplantation. All patients received perioperative platelet and blood transfusions. Pars plana vitrectomy resulted in functional and anatomic success in the majority of eyes in this series. Coordination of medical and surgical care with the hematology service is advisable to stabilize hematologic parameters prior to undertaking a vitreoretinal procedure.

Patients with autoimmune polyendocrinopathy-candiasis-ectodermal dystrophy (APECED) develop severe keratoconjunctivitis, corneal scarring and visual loss, but the precise pathogenesis is unknown. This study evaluated the ocular surface immune cell environment, conjunctival goblet cell density and response to desiccating environmental stress of the autoimmune regulatory (Aire) gene knockout murine model of APECED.Aire-deficient and wild type (WT) mice were subjected to desiccating stress from a drafty, low-humidity environment and pharmacological inhibition of tear secretion for 5 days. Immune cell populations (CD4(+), CD8(+), CD11b(+), CD45(+)) and goblet cell density were measured in ocular surface tissues and meibomian glands, and compared with baseline values.Greater CD4(+) T cell populations were observed in the conjunctival epithelium of Aire-deficient mice (p0.001) compared with WT. Aire-deficient mice also had greater numbers of CD4(+), CD8(+), and CD11b(+) cells in the peripheral cornea at baseline and following desiccating stress. The meibomian glands of Aire-deficient mice demonstrated greater CD4(+), CD8(+), CD45(+) and CD11b(+) cells at baseline (p0.001) and following desiccating stress. Conjunctival goblet cell density was lower at baseline and following desiccating stress in Aire-deficient compared with WT mice (p0.001).Aire-deficiency leads to infiltration of CD4(+) and CD8(+) T cells on the ocular surface and meibomian glands, which is accompanied by goblet cell loss. Desiccating stress promotes this proinflammatory milieu. Immune-mediated mechanisms play a role in the severe blepharitis and keratoconjunctivitis in the murine model of APECED.

To evaluate CD4(+)Foxp3(+) (forkhead box P3) T-regulatory cell populations in patients with uveitis and to determine if T-regulatory cell populations are associated with disease features.Patients with uveitis were evaluated for CD4(+)Foxp3(+) T-regulatory cells by flow cytometry. Systemic and ocular diagnoses, disease activity, and the presence of cystoid macular edema were reviewed. Percentages of CD4(+)Foxp3(+) lymphocytes were compared for patients with inactive vs active disease, systemic vs ocular diagnoses, and the presence or absence of cystoid macular edema. Real-time polymerase chain reaction testing was performed on 2 patients with extremely low CD4(+)Foxp3(+) cell populations to assess Foxp3 mRNA.A total of 20 patients with intermediate uveitis, posterior uveitis, and panuveitis were evaluated. The mean age was 40.6 years and the mean visual acuity was 20/57. Percentages of CD4(+)Foxp3(+) cells were lower in patients with active compared with inactive uveitis (P.05). No differences in T-regulatory cells were observed between the other subgroups. Two patients with recalcitrant uveitis who demonstrated less than 1% CD4(+)Foxp3(+) lymphocytes showed extremely low or absent Foxp3 mRNA.T-regulatory cells are reduced in patients with active compared with inactive disease. Severe depletion of CD4(+)Foxp3(+) T cells and Foxp3 mRNA in 2 patients with severe uveitis suggests that loss of the T-regulatory cells of uveitis may be a salient feature in certain patients.

To characterize foveal atrophy in a heterogeneous group of patients with uveitis using clinical findings and high-definition (HD) optical coherence tomography (OCT).Cross-sectional, retrospective case series.The HD-OCT scans of 140 patients seen in a tertiary referral center were reviewed and 23 patients (33 eyes) with foveal atrophy were identified. All of the patients with foveal atrophy were diagnosed with intermediate uveitis, posterior uveitis, or panuveitis. The status of the photoreceptor layer as visualized with HD-OCT was associated with significant differences in mean visual acuity (P.001). Clinical findings associated with foveal atrophy included atrophy of the retinal pigment epithelium and choroid (30 eyes [91%]), macular ischemia (13 eyes [39%]), cystoid macular edema (5 eyes [15%]), choroidal neovascularization (4 eyes [12%]), retinal detachment involving the macula (2 eyes [6%]), and serum antiretinal antibodies (2 eyes [6%]).Foveal atrophy can be a complication of intraocular inflammation in a variety of uveitic syndromes. The cause of foveal atrophy is multifactorial and may include dysfunction and atrophy of the retinal pigment epithelium and choroid, cystoid macular edema, macular ischemia secondary to occlusive retinal vasculitis, choroidal neovascularization, retinal detachment, and possibly antibody-mediated damage directed against photoreceptors. Careful observation of the photoreceptor layer using HD-OCT may help to identify patients who are at risk for visual loss secondary to foveal atrophy.