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To report 6 cases of diffuse choroidal hemangioma (DCH) in children treated with iodine-125 plaque brachytherapy at a single tertiary care center.Retrospective case series.Six pediatric patients diagnosed with DCH were included in the study. Pre-plaque visual acuity (VA) ranged from 20/150 to no light perception (NLP). All patients had extensive serous retinal detachment at presentation. An iodine-125 radioactive plaque was placed on the affected eye to administer a dose of 34.2-42.1 Gy to the tumor apex over a median of 4 days. Tumor regression and subretinal fluid resolution were observed in all eyes within 17 months of treatment. Visual acuity improved in 2 patients. Radiation-induced cataract and subretinal fibrosis were documented in 1 case, and 1 patient developed radiation retinopathy. No patients developed neovascular glaucoma within a follow-up range of 12-65 months.Iodine-125 plaque radiotherapy is an effective option for DCH, although there is a risk for radiation-induced complications.

Purpose Determine the efficacy of combination intravitreal and systemic antiviral therapy for the treatment of acute retinal necrosis (ARN) and risk factors impacting visual acuity (VA) and retinal detachment (RD) outcomes. Design Single-center retrospective case series. Participants Patients with an ARN diagnosis based on clinical features and polymerase chain reaction confirmation who were treated at a tertiary referral, university-based academic practice. Methods Patient records were reviewed for demographic information including age and gender. Snellen VA, disease findings including RD outcomes, optic nerve involvement, and treatments were recorded. Incidence rates of major VA and RD outcomes were calculated based on the number of events and exposure times. Cox proportional hazards regression modeling and survival analyses were used to identify factors related to VA and RD outcomes over time. Main Outcome Measures Logarithm of the minimal angle of resolution VA, 2-line or more VA gain, severe vision loss (SVL) of 20/200 or worse, RD development, and fellow eye involvement. Results Twenty-three eyes of 21 patients (11 male, 10 female) were reviewed. Thirteen patients (62%) had herpes simplex virus and 8 patients (38%) had varicella zoster virus. The event rate for 2-line or more VA gain was 0.49 events/eye-year (95% confidence interval [CI], 0.26–0.86 events/eye-year), whereas the rate of SVL was 0.61 events/eye-year (95% CI, 0.34–1.02 events/eye-year). Retinal detachment development was observed at a rate of 0.59 events/eye-year (95% CI, 0.33–1.00 events/eye-year). Thirteen of 23 eyes (57%) demonstrated RD with a mean time of 120 days after ARN diagnosis. With each additional quadrant of retina involved, a greater risk of RD development over time was observed (hazard ratio, 2.21; 95% CI, 1.12–4.35). Nine percent of eyes progressed with additional quadrantic involvement, despite combination systemic and intravitreal antiviral therapy; however, none of the 19 patients demonstrating unilateral ARN showed fellow-eye involvement after initiation of therapy. Conclusions Combination intravitreal and systemic antiviral therapy for ARN can be effective in improving VA and limiting retinitis progression. Each additional quadrant of retina involved was associated with a 2.2-fold greater risk of RD, which may impact monitoring, timing of intervention, and patient counseling.

Purpose Cutaneous melanoma metastatic to the vitreous is very rare. This study investigated the clinical findings, treatment, and outcome of patients with metastatic cutaneous melanoma to the vitreous. Most patients received checkpoint inhibition for the treatment of systemic disease, and the significance of this was explored. Design Multicenter, retrospective cohort study. Participants Fourteen eyes of 11 patients with metastatic cutaneous melanoma to the vitreous. Methods Clinical records, including fundus photography and ultrasound results, were reviewed retrospectively, and relevant data were recorded for each patient eye. Main Outcome Measures Clinical features at presentation, ophthalmic and systemic treatments, and outcomes. Results The median age at presentation of ophthalmic disease was 66 years (range, 23–88 years), and the median follow-up from diagnosis of ophthalmic disease was 23 months. Ten of 11 patients were treated with immune checkpoint inhibition at some point in the treatment course. The median time from starting immunotherapy to ocular symptoms was 17 months (range, 4.5–38 months). Half of eyes demonstrated amelanotic vitreous debris. Five eyes demonstrated elevated intraocular pressure, and 4 eyes demonstrated a retinal detachment. Six patients showed metastatic disease in the central nervous system. Ophthalmic treatment included external beam radiation (30–40 Gy) in 6 eyes, intravitreous melphalan (10–20 μg) in 4 eyes, enucleation of 1 eye, and local observation while receiving systemic treatment in 2 eyes. Three eyes received intravitreous bevacizumab for neovascularization. The final Snellen visual acuity ranged from 20/20 to no light perception. Conclusions The differential diagnosis of vitreous debris in the context of metastatic cutaneous melanoma includes intravitreal metastasis, and this seems to be particularly apparent during this era of treatment with checkpoint inhibition. External beam radiation, intravitreous melphalan, and systemic checkpoint inhibition can be used in the treatment of ophthalmic disease. Neovascular glaucoma and retinal detachments may occur, and most eyes show poor visual potential. Approximately one quarter of patients demonstrated ocular disease that preceded central nervous system metastasis. Patients with visual symptoms or vitreous debris in the context of metastatic cutaneous melanoma would benefit from evaluation by an ophthalmic oncologist.

With fewer than 100 peer-reviewed cases reported in the world to date, the underlying etiology of torpedo maculopathy has remained elusive. In this literature review, we provide new evidence to better support, reject and unify claims regarding cause, diagnosis, and proper clinical management of this disease. We reviewed 44 case reports and case series, which included 77 patients (after exclusions). We additionally introduced 3 new cases from our clinical practice for a total of 80 cases. Ages at presentation ranged from 6 months old to 73 years old (mean: 24.2 years old). The nasal aspects of torpedo maculopathy lesions pointed toward the optic disc and localized to a kite-shaped region of the temporal macula, correlating with the anatomic junction of the superior arcuate, inferior arcuate, and papillomacular bundles of retinal nerve fiber layer distribution. No patterns were observed among the temporal aspects of the lesions. These findings support a congenital etiology of torpedo maculopathy and a possible influence of the retinal nerve fiber layer in the development of mature retinal pigment epithelium. Keywords: Congenital, Distribution, Embryology, Hypopigmentation, Nerve fiber layer, Torpedo

Background The management of an outbreak of endophthalmitis associated with intravitreal bevacizumab represents a challenging real-time process involving identification of cases, treatment and mitigation measures during the outbreak. We summarize the clinical presentation and management of a cluster of endophthalmitis cases from contaminated bevacizumab, in addition to mathematical probabilistic assessment of the number of cases that define an outbreak. Methods A retrospective study was conducted to assess the management of an endophthalmitis outbreak after intravitreal bevacizumab (IVB) administration. Demographic data, clinical information, individual patient management and public health reporting measures were reviewed. Outcomes of patients who received prophylactic antibiotics for endophthalmitis prevention were also reviewed. Binomial tail probability calculations were performed to determine the likelihood of clusters of endophthalmitis that could inform when an outbreak was evolving that would warrant more public health notification measures and communication. Results Forty-five eyes of 42 patients who received IVB from a single batch were reviewed. Four cases of endophthalmitis from Granulicatella adiacens, a nutritionally-variant Streptococcus species, were treated successfully with intravitreal antibiotics ± vitrectomy. Thirty-four of the remaining 41 eyes were treated with prophylactic intravitreal vancomycin with no additional cases of endophthalmitis. Outbreak management also included CDC, ASRS and public health authority notification. Binominal tail probabilities demonstrated the rarity of clusters from a single batch (i.e. ~ 1/10,000 for 2 cases; 1/2 million for 3 cases). However, given the U.S. scale of IVB administration, there is an 87% chance of a cluster ≧ 2 and a 1% chance of a cluster ≧ 3 cases annually, which may guide outbreak management. A process diagram was developed to incorporate patient management and public health measures when an outbreak is suspected. Conclusion Intravitreal antibiotics and vitrectomy were effective in the individual management of cases of endophthalmitis, and no serious adverse events occurred with prophylactic intravitreal vancomycin for at-risk eyes. Best practices for outbreaks should be evaluated, given their likelihood within the U.S. and the sight-threatening consequences of endophthalmitis.

Purpose To investigate the natural history and genetic associations of drusenoid pigment epithelial detachment (DPED) associated with age-related macular degeneration (AMD). Design Retrospective analysis of a prospective cohort study. Participants Of the 4203 Age-Related Eye Disease Study 2 (AREDS2) participants, 391 eyes (325 participants) had DPED without late AMD at the time of DPED detection. Genetic analyses included 120 white AREDS2 participants and 145 Age-Related Eye Disease Study (AREDS) participants with DPED. Methods Baseline and annual stereoscopic fundus photographs were graded centrally to detect DPED, a well-defined yellow elevated mound of confluent drusen ≥433 μm in diameter, and to evaluate progression rates to late AMD: geographic atrophy (GA) and neovascular (NV)-AMD. Five single nucleotide polymorphisms (CFH [rs10611670], C3 [rs2230199], CFI [rs10033900], C2/CFB [rs114254831], ARMS2 [rs10490924]) and genetic risk score (GRS) group were investigated for association with DPED development. Kaplan–Meier analyses and multivariable proportional hazard regressions were performed. Main Outcome Measures Progression rates to late AMD and decrease of ≥3 lines in visual acuity (VA) from the time of DPED detection; association of rate of DPED development with genotype. Results Mean (standard deviation [SD]) follow-up time from DPED detection was 4.7 (0.9) years. DPED was associated with increased risk of progression to late AMD (hazard ratio [HR], 2.36; 95% confidence interval [CI], 1.98–2.82; P Conclusions This study replicates the results of previous natural history studies of eyes with DPED including the high rates of progression to late AMD and vision loss (regardless of progression to late AMD). The genetic associations are consistent with genes associated with AMD progression.

Purpose To analyze the prevalence, incidence, and clinical characteristics of eyes with geographic atrophy (GA) in age-related macular degeneration (AMD), including clinical and genetic factors affecting enlargement. Design Prospective cohort study within a controlled clinical trial. Participants Age-Related Eye Disease Study 2 (AREDS2) participants, aged 50–85 years. Methods Baseline and annual stereoscopic color fundus photographs were evaluated for GA presence and area. Analyses included GA prevalence and incidence rates, Kaplan-Meier rates, mixed-model regression, and multivariable analysis of the square root of GA, area adjusted for covariates, including clinical/imaging characteristics and genotype. Main Outcome Measures (1) Presence or development of GA; (2) change in the square root of GA area over time. Results At baseline, 517 eyes (6.2%) of 411 participants (9.8%) had pre-existing GA (without neovascular AMD), with the following characteristics: 33% central, 67% noncentral; and the following configurations: 36% small, 26% solid/unifocal, 24% multifocal, 9% horseshoe/ring, and 6% indeterminate. Of the remaining 6530 eyes at risk, 1099 eyes (17.3%) of 883 participants developed incident GA without prior neovascular disease during mean follow-up of 4.4 years. The Kaplan-Meier rate of incident GA was 19% of eyes at 5 years. In eyes with incident GA, 4-year risk of subsequent neovascular AMD was 29%. In eyes with incident noncentral GA, 4-year risk of central involvement was 57%. GA enlargement rate (following square root transformation) was similar in eyes with pre-existing GA (0.29 mm/year; 95% confidence interval 0.27–0.30) and incident GA (0.28 mm/year; 0.27–0.30). In the combined group, GA enlargement was significantly faster with noncentrality, multifocality, intermediate baseline size, and bilateral GA (P Conclusions Analyses of AREDS2 data on natural history of GA provide representative data on GA evolution and enlargement. GA enlargement, which was influenced by lesion features, was relentless, resulting in rapid central vision loss. The genetic variants associated with faster enlargement were partially distinct from those associated with risk of incident GA. These findings are relevant to further investigations of GA pathogenesis and clinical trial planning.

Purpose To evaluate postoperative pain level using a supplemental peribulbar injection at the conclusion of retinal surgery. Design Prospective, parallel-assigned, single-masked, randomized clinical trial. Participants Fifty-eight patients undergoing scleral buckle, vitrectomy, or combined surgery. Methods In a single academic institutional practice, 58 patients undergoing scleral buckle, vitrectomy, or combined surgery were enrolled. Exclusion criteria included those with a risk for glaucoma, a pre-existing chronic pain disorder, among others. Patients were assigned randomly to receive a postoperative peribulbar formulation of either bupivacaine, triamcinolone acetonide, and cefazolin (group A) or bupivacaine, balanced salt solution, and cefazolin (group B). The postoperative pain score and ocular motility were assessed by a masked observer on the first postoperative day. Main Outcome Measures The primary outcome measure was the postoperative pain score. Secondary outcome measures included oral analgesic use, ocular motility, and intraocular pressure (IOP). Results The mean pain scores were 2.8±2.9 for group A and 3.8±2.6 for group B (P = 0.095). Pain was absent in 28% of group A patients versus 14% of group B patients (P = 0.11). Group A required less narcotic pain medication (hydroxycodone: group A, 0.7±3 mg vs. group B, 3±6 mg; P = 0.05; oxycodone: group A, 7±7 mg vs. 9±13 mg; P = 0.2) than group B. Motility was full in group B and limited in group A (P ≤ 0.001), with no differences in mean IOP measurements at any point after surgery. Conclusions We did not demonstrate a statistically significant reduction in mean postoperative pain scores. However, patients in group A required less hydroxycodone use and had greater akinesia, suggesting prolonged neural blockade.

PURPOSE: For treatment of choroidal melanoma, Palladium-103 ((103)Pd) and Ruthenium-106 ((106)Ru) plaque brachytherapy demonstrates reduced toxicity compared to the historical standard Iodine-125 ((125)I). No report has directly compared clinical outcomes between (103)Pd and (106)Ru, and reasons for selection of one over the other remain purely theoretical. MATERIALS/METHODS: Patients with choroidal melanoma with apical tumor height ≤ 5 mm were included. Patients from Emory University were treated with (103)Pd between 1993 and 2012. Patients from Cleveland Clinic were treated with (106)Ru between 2005 and 2010. Medical records were retrospectively reviewed. We compared post-treatment visual acuity (VA), toxicity, and oncologic outcomes. RESULTS: (103)Pd patients (n = 124) and (106)Ru patients (n = 42) had median follow up of 4.2 and 5.0 years, respectively. Radiation retinopathy-free survival was similar for both radioisotopes but (106)Ru had lower grades of retinopathy (p=0.006). (103)Pd was associated with worse VA preservation (≥20/40) by year 3 (OR 3.8, 95% CI 1.01–14.31, p=0.048). (103)Pd was associated with higher distant metastases-free survival (DMFS) in multivariate analysis (MVA) (HR 0.10, 95% CI 0.02–0.38, p

Purpose To evaluate outcomes of choroidal melanoma patients treated with 125I or 103Pd plaque brachytherapy. Methods and Materials From 1993 to 2012, our institution treated 160 patients with 103Pd (56.1%) and 125 patients with 125I (43.9%) plaque brachytherapy. Tumor outcomes, visual acuity (VA), and toxicity were compared. Multivariate analyses (MVAs) and propensity score analysis were used to help address differences in baseline characteristics. Results Median followup was longer for 125I patients, 52.7 vs. 43.5 months (p 90% 3-year overall survival and >93% 5-year secondary enucleation-free survival. On MVA, radionuclide was not predictive for tumor outcomes. A higher percentage maintained vision better than 20/40 with 103Pd (63% vs. 35%, p = 0.007) at 3 years. MVA demonstrated 103Pd radionuclide (odds ratio [OR]: 2.12, p = 0.028) and tumor height ≤5 mm (OR: 2.78, p = 0.017) were associated with VA better than 20/40. Propensity score analysis matched 23 125I with 107 103Pd patients. 103Pd continued to predict better VA at 3 years (OR: 8.10, p = 0.014). On MVA for the development of VA worse than 20/200 or degree of vision loss, radionuclide was not significant. Lower rates of radiation retinopathy were seen with 103Pd than 125I (3 years: 47.3% vs. 63.9%, p = 0.016), with radionuclide significant in MVA. Conclusions Both 125I and 103Pd achieve excellent tumor control. An increased probability of long-term VA better than 20/40 and reduced risk of radiation retinopathy is associated with 103Pd.

Purpose: To report a case of panuveitis, retinal vasculitis, and optic disk granuloma due to sarcoidosis. Methods: Case report and literature review. Results: A 26-year-old previously healthy African American male presented with four months of gradual progressive visual decline in the right eye. Clinical examination revealed severe panuveitis, retinal vasculitis, and large optic nerve mass lesion. Diffuse supraclavicular lymphadenopathy was also present. Histopathologic examination of the lymph node biopsy revealed granulomatous inflammation with some areas of caseous necrosis consistent with sarcoidosis. Conclusion: Sarcoidosis is a common cause of uveitis and retinal vasculitis. In rare cases, an optic disk granuloma may occur and can be treated with immunosuppressive therapy.

To evaluate postoperative pain level using a supplemental peribulbar injection at the conclusion of retinal surgery.Prospective, parallel-assigned, single-masked, randomized clinical trial.Fifty-eight patients undergoing scleral buckle, vitrectomy, or combined surgery.In a single academic institutional practice, 58 patients undergoing scleral buckle, vitrectomy, or combined surgery were enrolled. Exclusion criteria included those with a risk for glaucoma, a pre-existing chronic pain disorder, among others. Patients were assigned randomly to receive a postoperative peribulbar formulation of either bupivacaine, triamcinolone acetonide, and cefazolin (group A) or bupivacaine, balanced salt solution, and cefazolin (group B). The postoperative pain score and ocular motility were assessed by a masked observer on the first postoperative day.The primary outcome measure was the postoperative pain score. Secondary outcome measures included oral analgesic use, ocular motility, and intraocular pressure (IOP).The mean pain scores were 2.8±2.9 for group A and 3.8±2.6 for group B (P = 0.095). Pain was absent in 28% of group A patients versus 14% of group B patients (P = 0.11). Group A required less narcotic pain medication (hydroxycodone: group A, 0.7±3 mg vs. group B, 3±6 mg; P = 0.05; oxycodone: group A, 7±7 mg vs. 9±13 mg; P = 0.2) than group B. Motility was full in group B and limited in group A (P ≤ 0.001), with no differences in mean IOP measurements at any point after surgery.We did not demonstrate a statistically significant reduction in mean postoperative pain scores. However, patients in group A required less hydroxycodone use and had greater akinesia, suggesting prolonged neural blockade.

Vision loss associated with the idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome most commonly occurs from macular edema or complications related to neovascularization. The authors present a case of advanced IRVAN associated with a massive exudative response characterized by peripheral retinal telangiectasias, exudative retinal detachment, and macular edema with lipid maculopathy. The patient was managed successfully with visual acuity from hand motion to 20/150 using a combination of local corticosteroids, intravitreal bevacizumab, panretinal photocoagulation, and eventually pars plana vitrectomy for progressive vitreomacular traction. VEGF- and non-VEGF-mediated mechanisms appear to be involved in the pathogenesis of IRVAN given the efficacy of combination therapy. [ Ophthalmic Surg Lasers Imaging Retina. 2013;44:599–602.]

To evaluate the use of microscope mounted spectral domain optical coherence tomography (SD-OCT) to detect changes in retinal anatomy during macular surgery.Retrospective, observational case series.We included 25 eyes of 24 consecutive patients who underwent SD-OCT during macular surgery.A retrospective review of operative techniques, outcomes, and imaging for all patients who underwent intraoperative microscope mounted SD-OCT during surgery for macular hole or epiretinal membrane (ERM) from April 2009 to April 2010 was performed. Qualitative and quantitative characteristics of intraoperative and postoperative changes in retinal anatomy were studied.Intraoperative change in macular hole dimensions and retinal thickness in patients with ERM owing to surgical manipulation measured using SD-OCT.Intraoperative SD-OCT from 13 eyes of 13 patients undergoing surgery for macular hole was reviewed. Two cases had images of suboptimal quality and were excluded. The remaining 11 eyes were subjected to quantitative analysis, which revealed stability of macular hole height and central hole diameter after internal limiting membrane (ILM) peeling, but an increase in the diameter of subretinal fluid under the macula in ten of 11 eyes (average 87% wider). Intraoperative imaging from 12 eyes of 11 patients undergoing surgery for ERM was analyzed. Quantitative analysis revealed an average increase of retinal thickness after ILM peel of2%. Ten of 12 eyes developed a new subretinal hyporeflectance, which likely represents shallow detachment of the macula, after uncomplicated membrane peel.Use of intraoperative SD-OCT has provided new insight into the changes to retinal anatomy during macular surgery and may prove to be a useful tool for vitreoretinal surgery. Further study is warranted to determine whether intraoperative changes such as the creation of shallow retinal detachments during uncomplicated macular surgery affects visual recovery.Proprietary or commercial disclosure may be found after the references.

Purpose: To report the macular hole closure rate and visual outcomes of patients with residual triamcinolone acetonide in the fovea after macular hole repair. Methods: We reviewed the medical records of consecutive patients who underwent macular hole surgery at our institution between 2005 and 2008. Only patients with visible triamcinolone in the fovea in the first postoperative month were included. Results: Six patients with Stage III or IV macular holes were included. All patients underwent pars plana vitrectomy, internal limiting membrane peeling, and gas tamponade. Triamcinolone acetonide was used to visualize the vitreous in every patient, and diluted indocyanine green was used to stain the internal limiting membrane in five patients. The median preoperative best-corrected visual acuity was 20/200, which improved to a median of 20/40 at the last follow-up. Five patients eventually developed retinal pigment epithelial alterations. Anatomic hole closure was achieved in every patient. The mean follow-up was 23 months (range, 3-36 months). Conclusion: Residual triamcinolone sequestered in the fovea after macular hole surgery did not affect hole closure or prevent improvement in visual acuity. It is unclear whether the retinal pigment epithelial alterations in our patients represent toxicity or are unrelated to the triamcinolone exposure.

Intravitreal triamcinolone injection effectively reduces subretinal fluid in pediatric patients with exudative retinal detachments in severe Coats disease. However, when combined with cryotherapy, a large percentage of patients develop rhegmatogenous retinal detachments with proliferative vitreoretinopathy.

Purpose: To report on the clinical data of seven patients with T-cell intraocular lymphoma (IOL). Methods: Retrospective case series. Results: Seven immunocompetent patients, 12 eyes, 6 women, with T-cell-IOL were included from five countries. Mean age was 53.5 years (range: 25–82). Four patients had systemic-ocular lymphoma, two had CNS-ocular lymphoma, and one had systemic-CNS- ocular lymphoma. Vitritis was the most frequent clinical sign, followed by anterior uveitis and serous retinal detachment. Cytopathologic examination was performed on all ocular specimens (vitreous in six and iris mass biopsy in one patient). Adjunctive diagnostic procedures included immunohistochemistry, molecular tests, and cytokine profiling of vitreous samples. Treatment modalities included systemic chemotherapy (five patients), intravitreal methotrexate (three patients), globe radiotherapy, and intrathecal chemotherapy. Mean survival from diagnosis was 21.7 months (range: 2–69). Two patients are still alive. Conclusions: T-cell IOL has variable clinical manifestations and prognosis. Systemic involvement, SRD, and vitreoretinal involvement were frequently observed.

Background: Errors in neural crest development are responsible for a number of ocular disorders. In some cases, these ocular abnormalities may be associated with systemic disorders, some of which may be life threatening. Methods: We reviewed the medical record of 3 children from 2 institutions with a similar constellation of ocular and cardiac abnormalities. Results: All 3 children had iris hypoplasia with pupils 6–7 mm in diameter that had minimal or no response to light or pharmacological agents. The ocular examinations were otherwise normal. All 3 children also had large aorticopulmonary septal defects which were surgically repaired. Conclusions: We report an association between iris hypoplasia and aorticopulmonary septal defects. Like the iris stroma, the aorticopulmonary septum forms from neural crest cells during embryogenesis which divide the truncus arteriosus into the ascending aorta and pulmonary trunk. Children with iris hypoplasia should undergo an immediate cardiac evaluation. It is important that opthalmologists be aware of the association between these two disorders since an aorticopulmonary septal defect is a life-threatening disorder.

An 11-year-old boy underwent enucleation of his left eye for an intraocular tumor. Examination showed a small, round blue cell tumor arising in the peripheral retina near the ciliary body. Immunohistochemical stain results were positive for neuron-specific enolase, synaptophysin, cluster of differentiation 99 (CD99), Friend leukemia integration 1, and CD56. Ultrastructural findings included occasional intracytoplasmic dense core granules. Polymerase chain reaction of the tumor showed a Ewing sarcoma/Friend leukemia integration gene fusion product. The tumor was classified as a primitive neuroectodermal tumor/Ewing sarcoma of the retina and should be distinguished from retinoblastoma. To our knowledge, this is the first case of primary primitive neuroectodermal tumor of the retina.

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.

PURPOSE To report optical coherence tomography (OCT) progressive changes in chloroquine maculopathy. DESIGN Observational case report. METHODS Both eyes of a patient with chloroquine maculopathy were examined serially with OCT. RESULTS OCT demonstrated diffuse retinal thinning involving the fovea and perifoveal area in each eye, particularly involving the outer retina, with loss of the photoreceptor outer hyperreflective lamina proximal to the lamina corresponding to the retinal pigment epithelium (RPE). The temporal nerve fiber layer was mildly thinned. Repeat OCT showed progressive macular thinning. CONCLUSIONS OCT findings are consistent with histopathologic studies that advanced chloroquine toxicity affects the nerve fiber layer and the outer retinal layers, and confirm reports that severe maculopathy may progress despite cessation of therapy. The possible use of OCT in screening of patients at risk for macular toxicity is suggested.

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

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.

Purpose To present the American Brachytherapy Society (ABS) guidelines for plaque brachytherapy of choroidal melanoma and retinoblastoma. Methods and Materials An international multicenter Ophthalmic Oncology Task Force (OOTF) was assembled to include 47 radiation oncologists, medical physicists, and ophthalmic oncologists from 10 countries. The ABS-OOTF produced collaborative guidelines, based on their eye cancer–specific clinical experience and knowledge of the literature. This work was reviewed and approved by the ABS Board of Directors as well as within the journal's peer-reivew process. Results The ABS-OOTF reached consensus that ophthalmic plaque radiation therapy is best performed in subspecialty brachytherapy centers. Quality assurance, methods of plaque construction, and dosimetry should be consistent with the 2012 joint guidelines of the American Association of Physicists in Medicine and ABS. Implantation of plaque sources should be performed by subspecialty-trained surgeons. Although there exist select restrictions related to tumor size and location, the ABS-OOTF agreed that most melanomas of the iris, ciliary body, and choroid could be treated with plaque brachytherapy. The ABS-OOTF reached consensus that tumors with gross orbital extension and blind painful eyes and those with no light perception vision are unsuitable for brachytherapy. In contrast, only select retinoblastomas are eligible for plaque brachytherapy. Prescription doses, dose rates, treatment durations, and clinical methods are described. Conclusions Plaque brachytherapy is an effective eye and vision-sparing method to treat patients with intraocular tumors. Practitioners are encouraged to use ABS-OOTF guidelines to enhance their practice.

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.

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.

A 62-year-old man with lung cancer presented with a 2-week history of decreased vision and clinical features of cytomegalovirus retinitis. The patient was empirically treated for viral retinitis, but microbiological testing of the vitreous fluid was negative. Based on the suspicion for retinal metastasis, the patient underwent pars plana vitrectomy with retinal biopsy. Surgical techniques included the use of a chandelier illumination to enable bimanual manipulation of the retinal tissue, creation a focal retinal detachment with a 41-gauge subretinal cannula, diathermy demarcation of the biopsy site, localized retinectomy with vertical scissors, endolaser, and long-acting gas tamponade. Histopathologic examination revealed sheets of tumor cells with pleomorphic nuclei and positive staining for cytokeratins consistent with metastatic adenocarcinoma. The patient subsequently underwent external beam radiation and was alive 10 months after presentation. This surgical technique may be valuable in select patients with retinal metastasis for diagnostic, therapeutic, and counseling purposes.

An unusual case of central retinal artery occlusion (CRAO) occurring in the setting of a chronic retinal detachment was reported. A 47-year old woman with a history of slowly declining peripheral vision in her left eye presented with acute painless hand motion vision. She had an extensive chronic retinal detachment in the left eye with multiple demarcation lines and marked whitening of the fovea. Her right eye was normal. A CRAO was confirmed with fluorescein angiography and optical coherence tomography testing. An exhaustive work-up was negative and there was no history of antecedent trauma.

A 37-year-old man, previously well, presented with 2 weeks of conjunctival redness and burning discomfort of the left eye. Roughly 7 days after noticing these initial symptoms, he developed blurry vision of the left eye with fl oaters and the appearance of spider webs. Slit-lamp examination of the left eye (fi gure, A) showed marked conjunctival injection with a fi brinous anterior-chamber reaction and posterior synechiae. Funduscopic examination showed mild vitritis, with the appearance of ground glass retinitis and frosted branch angiitis (fi gure, B). At that time, the patient was diagnosed with HIV infection with an initial CD4+ T-cell count of 405 cells per μl (15%). Rapid plasma reagin (RPR) titre was initially negative, but on recheck about a week later was positive with a titre of 1/512. Serum treponemal IgG was positive. The patient was diagnosed with secondary syphilis, presenting as anterior uveitis and frosted branch angiitis of the retina. His condition improved dramatically with intravenous penicillin treatment. Syphilis in HIV-infected patients has been resurgent in recent years and ocular involvement is not uncommon. Anterior uveitis is seen frequently with secondary syphilis and this patient’s initial non-reactive RPR may have represented a false negative test due to a high titre prozone eff ect. Though much less frequent, retinal vasculitis in the form of frosted branch angiitis has been reported as a manifestation of ocular syphilis. Frosted branch angiitis has also been reported with a variety of other conditions, including herpes simplex, tuberculosis, haematological malignancies, and autoimmune diseases. In the setting of HIV, frosted branch angiitis is most commonly associated with cytomegalovirus infection. In this case, cytomegalovirus involvement was felt to be unlikely due to high CD4+ count, which on recheck was 392 cells per μl.

To evaluate the incidence, effect on visual recovery, and predisposing risk factors of postvitrectomy macular edema (ME).Prospective cohort study.One-hundred nine eyes undergoing nonemergent vitrectomy surgery.Eyes were evaluated for postoperative day 1 inflammation, 1-month retinal thickness using optical coherence tomography, and preoperative and 1-month postoperative best-corrected visual acuity (BCVA). Macular edema was defined as central subfield thicknessor =272 microm.Retinal thickness, inflammation, and BCVA.Incidence of ME on optical coherence tomography was 47% (95% confidence interval [CI], 37%-56%). Mean 1-month visual acuity improved 3.3 lines (0.33 logarithm of minimum angle of resolution [logMAR] units) to 20/80(+1) (0.58+/-0.46 logMAR units) from 20/150(-2) (0.91+/-0.63 logMAR units) before surgery (P0.001). Mean 1-month center point thickness (CPT), central subfield (CSF), and total macular volume were 265+/-107 microm, 288+/-94 microm, and 7.8+/-1.2 mm(3), respectively. Severity of postoperative inflammation predicted retinal thickness at 1 month (P0.05). Intraoperative epinephrine use was associated with increased postoperative inflammation (P = 0.02). Eyes with greater reduction in CSF (or CPT) from baseline experienced more rapid visual recovery (r = -0.36; 95% CI, -0.61 to -0.06; P = 0.02).Postvitrectomy ME is common and delays visual recovery. Degree of postoperative inflammation is an important risk factor for ME and, in this series, was increased in the setting of intraocular epinephrine. Efforts to reduce or prevent inflammation after vitrectomy should be beneficial and therefore are encouraged.

Background/aim : This is a long-term follow-up report investigating primary TTT for choroidal melanoma. Methods : Retrospective case series of 135 patients harboring choroidal melanoma treated with primary TTT. Patient demographics, tumor characteristics, treatment responses and complications, visual acuity outcomes, and mortality data were captured and reported. Statistical analysis was performed for predictors of treatment failure. Results : Successful tumor regression was achieved in 76% of patients. Of the 32 patients that failed, 12 had enucleation, and 20 had irradiation. Metastatic disease has occurred in three patients, two patients have died (3/135, or 2%). Multivariate analysis determined that tumor diameter, tumor thickness greater than 3 mm and tumors exhibiting high risk characteristics were significant predictors of failure. Patient age, gender, number of treatments, and proximity of the tumor to the disc or fovea were not predictive of failure. Kaplan-Meier cumulative probability predicted a 19% 5-year treatment failure, and 33% 10-year treatment failure. Treatment failure occurred as late as 118 months. Final visual acuity was 20/40 or better in 50% of patients; 32% had a final visual acuity of 20/200 or worse. Thirty-two percent of patients developed one or more complications as a result of the TTT, the most concerning of which was intra- or extrascleral extension of tumor (occurring in 11 patients). Conclusions : Though not as successful as radiation therapy, TTT successfully induced regression in 76% of patients. TTT may still have a role in our treatment paradigm but should probably be reserved for specific cases, such as monocular patients with tumors near critical visual structures, surgically unstable patients, or patients with advanced diabetic retinopathy. All patients considering TTT as monotherapy for choroidal melanoma must be selected, counseled and followed appropriately.

Purpose To describe a series of chorioretinal folds (CRFs) representing a clinical sign that may be associated with multiple systemic, orbital, and ophthalmologic disorders. We report the associations with systemic disease and describe 3 stages of a CRF-related maculopathy. Design Observational, retrospective case series. Methods We reviewed 57 affected eyes from 40 patients with the clinical sign of CRF from 1 of 2 academic institutions. A careful review of the medical histories and systemic diagnostic evaluations were conducted. Imaging studies were conducted. Results The mean age at diagnosis was 64 ± 17 years. Most eyes (n = 18) were hyperopic (+2.60 ± +2.90 diopters). There were 20 patients (50%) with some form of autoimmune disorder. Overall, the mean presenting visual acuity was 20/50, declining slightly to 20/60 over 19 ± 30 months. Ten eyes had stage 3 CRF-related maculopathy, more common in older individuals with more chronic CRFs. Four stage 3 eyes had associated choroidal neovascularization, and these eyes had 20/60 presenting visual acuity that decreased to 20/100 over approximately 1.5 years. Stage 3 eyes without choroidal neovascularization had a mean presenting visual acuity of 20/40 that decreased to 20/65 over 2.1 years. Conclusions CRFs are associated with numerous ophthalmic and systemic disorders. A careful medical history and evaluation are essential. We describe 3 stages of a unique CRF-related maculopathy. Stage 3 resembles occult choroidal neovascularization, occurs primarily in older individuals with chronic CRFs, and is accompanied by a slow deterioration in central acuity.