Retinal artery and vein occlusion in calciphylaxis.

Purpose: To report a case of branch retinal artery occlusion (BRAO) followed by branch retinal vein occlusion (BRVO) and paracentral acute middle maculopathy (PAMM) in a patient with confirmed calciphylaxis. Observations: A 52-year-old female with a history of BRAO in the right eye one-year prior presented with decreased vision and a new inferotemporal scotoma. Computed tomography angiography of the head and neck demonstrated vascular calcifications at the origin of both ophthalmic arteries, which were otherwise poorly visualized. Ophthalmic examination demonstrated retinal whitening superiorly with intraretinal hemorrhages inferiorly. Optical coherence tomography (OCT) demonstrated middle retinal hyperreflectivity and a mild epiretinal membrane. Fluorescein angiography (FFA) demonstrated delayed perfusion of superior retinal arcade. On further questioning, patient was found to have a history of IgA nephropathy with end-stage renal disease, secondary hyperparathyroidism and calciphylaxis. Calciphylaxis is a systemic disease, characterized by high levels of calcium and progressive calcification of the vascular medial layer leading to ischemia. Anterior ischemic optic neuropathy (AION) and crystalline retinopathy have been reported as ocular manifestations of calciphylaxis, however, there are very few reports on ophthalmic manifestations of calciphylaxis. Conclusion and importance: Clinical manifestations of calciphylaxis are variable and a detailed clinical history is important to suspect calciphylaxis. Calciphylaxis should be considered in the differential diagnosis of BRAO, BRVO, PAMM or any ophthalmic vascular manifestation in patients with end-stage renal disease.

Chorioretinal Findings as the Initial Presentation of Chronic Granulomatous Disease.

To describe a case of chronic granulomatous disease (CGD) with ocular lesions as the presenting findings. An ocular examination of a 4-month-old male infant with poor vision revealed punched-out macular and perivascular chorioretinal fibrosis and atrophy with peripheral retinal nonperfusion. Subsequently, he was hospitalized for recurrent infections and was diagnosed with CGD. Repeated examination demonstrated enlargement of the chorioretinal lesions without evidence of active inflammation or neovascularization. CGD causes recurrent, severe, life-threatening infections in children and should be considered in the differential diagnosis of chorioretinal lesions with peripheral retinal nonperfusion. [Ophthalmic Surg Lasers Imaging Retina. 2022;53(4):234-238.].

Factors Affecting Ophthalmology Trainees to Pursue Vitreoretinal Surgery Fellowship.

Objective The aim of this study was to understand the factors that ophthalmology trainees consider in pursuing vitreoretinal surgery (VRS) fellowship training. Methods This is a prospective observational survey study. Survey invitations were disseminated to postgraduate year 4 (PGY)-4 ophthalmology residents at Accreditation Council for Graduate Medical Education-accredited residency programs and surgical retina fellows at Association of University Professors of Ophthalmology Fellowship Compliance Committee-compliant fellowship programs in the United States. Survey questions on factors related to VRS were administered employing a 5-point Likert scale. Responses from ophthalmology residents pursuing surgical retina were combined with surgical retina fellows' responses and compared with responses from PGY-4 residents not pursuing vitreoretinal surgery. Results Eighty-one resident surveys were completed. Forty-three fellow surveys were completed. Fifty-seven out of eighty-one (70.4%) residents were not pursuing surgical retina, and a total of 67 trainees (24 residents, 43 fellows) were pursuing surgical retina. The following factors were associated with pursuing VRS training: male gender ( p = 0.031); having performed retina research during residency ( p ≤ 0.001); enjoying surgical retina procedures ( p ≤ 0.001), enjoying surgical retina patient outcomes ( p ≤ 0.001), and working with vitreoretinal surgeons ( p ≤ 0.001); finding surgical retina prestigious ( p ≤ 0.001); perceiving their residency having a strong record of matching surgical retina ( p = 0.039); liking the potential financial income from surgical retina ( p ≤ 0.001); and having vitreoretinal mentors during residency ( p = 0.014). A majority of trainees (31/57, 54.4%) not pursuing surgical retina disagreed or strongly disagreed with enjoying the patient outcomes in surgical retina. A third of female residents not pursuing surgical retina felt having a female surgical retina mentor would have made them more likely to pursue the field. Conclusion Longer retina rotations, encouraging resident participation in retina research, and increasing mentorship opportunities of female trainees from female retina specialists may increase resident interest in pursuing surgical retina fellowship.

The Effect of Obstructive Sleep Apnea Treatment and Severity on Choroidal Thickness in Patients With Central Serous Chorioretinopathy.

Purpose: This work aimed to analyze the association of obstructive sleep apnea (OSA) with choroidal thickness (CT) in patients with central serous chorioretinopathy (CSC). Methods: We identified patients in the Stanford Research Repository with a diagnosis of CSC and OSA. Age- and sex-matched controls with either CSC or OSA only were also identified. CT was measured at 5 points (subfoveal, and 1500 and 3000 µm nasal and temporal) by 2 graders. In addition to OSA treatment and severity, we also investigated the association of Oxygen Desaturation Index and nocturnal oxygen saturation nadir with subfoveal CT (SFCT). Results: A total of 57 patients and 72 eyes met the study inclusion criteria. The mean SFCT was significantly different across the 3 groups: OSA-only was the thinnest, followed by CSC with OSA, and CSC-only was the thickest (194.2 µm, 295.1 µm, and 357.8 µm, respectively, P < .001). SFCT was thicker in CSC with OSA compared with those with only OSA (P < .05). OSA treatment status and OSA severity did not show a significant difference in SFCT in multivariable modeling. Nocturnal oxygen saturation nadir was positively associated with SFCT, but this did not reach significance.. Conclusions: SFCT is significantly different in patients with OSA alone, CSC with OSA, and CSC alone. While OSA treatment status did not demonstrate a significant difference in SFCT in this study, future studies should evaluate patients for OSA in patients known to have CSC and atypically thin CT to further investigate the novel metrics leveraged in this study.

Quality, Readability, and Accessibility of Online Content From a Google Search of "Macular Degeneration": Critical Analysis.

Purpose: This work aims to assess the quality, accountability, readability, accessibility, and presence of Spanish translation in online material through a Google search of "macular degeneration". Methods: In this retrospective cross-sectional analysis of website results from a Google search of "macular degeneration", the quality and accountability for each website were assessed using the DISCERN criteria and the Health on the Net Foundation Code of Conduct (HONcode) principles. All 31 sites were independently graded by 2 ophthalmologists. Readability was evaluated using an online tool. The presence of accessibility features on the website and Spanish translation was recorded. The primary outcome measure was the DISCERN and HONcode quality and accountability scores of each website. Secondary outcome measures included the readability, accessibility, and presence of Spanish translation. Results: The mean ± SD of each criterion across all 15 DISCERN questions was 2.761 ± 0.666 (out of 5). The mean HONcode score for all websites was 7.355 ± 3.123. The mean consensus reading grade level was 10.258 ± 2.49. There were no statistically significant differences in any score between the top 5 websites and the bottom 26 websites evaluated. Accessibility was available on 10 of 31 websites. Spanish translation was available on 10 of 31 websites. Conclusions: The top 5 websites that appeared on a Google search did not have better quality or readability of online content. Improving quality, accountability, and readability can help improve patients' health literacy regarding macular degeneration.

Discontinuation and loss to follow-up rates in clinical trials of intravitreal anti-vascular endothelial growth factor injections.

PURPOSE: Clinical trials are often designed to include homogenous, highly specific patient populations with many resources to reduce patient dropout. Results may not translate to real-world settings. We evaluated discontinuation and loss to follow-up (LTFU) rates in clinical trials of anti-vascular endothelial growth factor (anti-VEGF) injections for diabetic macular edema (DME), age-related macular degeneration (AMD), and retinal vein occlusion (RVO). METHODS: Retrospective meta-epidemiological study. The authors queried ClinicalTrials.gov for all completed trials of anti-VEGF injections for DME, AMD, or RVO. Of 658 trials identified, 582 were excluded for being non-interventional, <100 patients, terminating early, or missing study results. The remaining 76 trials of 27,823 patients were analyzed for discontinuation and LTFU rates. RESULTS: Mean discontinuation rate was 12.44% (SD 8.12%, range 0-54.12%), with higher rates among control (18.87%) than treatment arms (10.78%, p = .006). Mean LTFU rate was 1.84% (SD 1.78%, range 0-7.76%), with no differences by disease, treatment type, or treatment frequency. CONCLUSION: Discontinuation rates of major intravitreal anti-VEGF clinical trials were highly variable, suggesting even trials struggle with overall patient retention. Though trial LTFU rates were low, real-world outcomes may differ due to higher reported LTFU rates, which should be considered when extrapolating trial results to clinical practice.

Social Media and Retinal Detachment: Perspectives of Providers and Patients on Instagram.

BACKGROUND AND OBJECTIVE: To evaluate the social media interactions of patients who experience retinal detachment and/or repair and how ophthalmic medical providers utilize social media. PATIENTS AND METHODS: A cross-sectional study of public posts on Instagram was performed from September 2019 to January 2020, with filtering based on the following hashtags: #retinaldetachment, #detachedretina, #retinaldetachmentsurgery, and #scleralbuckle. A categorical classification system was used for each Instagram post to review descriptive variables. RESULTS: Of the 1,797 Instagram posts analyzed, 697 (39%) were made by patients, of which 395 (57%) were positive in tone and 599 (86%) were in the postoperative period. Posts by ophthalmologists were most commonly centered on colleague education (446, 81%). CONCLUSIONS: The majority of Instagram posts by patients were of a positive tone reflecting primarily on activities of daily living and rehabilitations. Retina specialists should consider providing more social media content directed toward patients, particularly regarding their rehabilitation course following retinal detachment repair. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:391-395.].

Anti-Vascular Endothelial Growth Factor and Panretinal Photocoagulation Use after Protocol S for Proliferative Diabetic Retinopathy.

PURPOSE: To characterize the rates of panretinal photocoagulation (PRP) and anti-vascular endothelial growth factor (VEGF) medications before and after publication of the Diabetic Retinopathy Clinical Research Network protocol S. DESIGN: A retrospective, cross-sectional study from January 2012, through September 2019, using a nationally representative claims-based database, Clinformatics Data Mart Database (OptumInsight, Eden Prairie, MN). PARTICIPANTS: Eyes newly diagnosed with proliferative diabetic retinopathy (PDR), continuous enrollment, and no prior treatment with PRP or anti-VEGF agents. METHODS: Interrupted time series regression analysis was performed to identify the annual change in treatment rates before and after the publication of Protocol S (November 2015). MAIN OUTCOME MEASURES: Annual rates of anti-VEGF or PRP treatments per 1000 treated eyes with PDR. RESULTS: From 2012 through 2019, 10 035 PRP or anti-VEGF treatments were administered to 3685 PDR eyes. Of these, 63.6% (n = 6379) were anti-VEGF agents, and 36.4% (n = 3656) were PRP treatments. Throughout treatment, 88.7% of eyes treated with anti-VEGF received the same agent and 7.7% were treated with both PRP and anti-VEGF agents. Panretinal photocoagulation rates declined from 784/1000 treated eyes in 2012 to 566/1000 in 2019 (pre-Protocol S: ß = -32 vs. post-Protocol S: -77; P = 0.005), whereas anti-VEGF rates increased from 876/1000 in 2012 to 1583/1000 in 2019 (ß = -48 vs. 161, respectively; P = 0.001). Panretinal photocoagulation rates in diabetic macular edema (DME) eyes did not significantly differ from 474/1000 in 2012 to 363/1000 in 2019 (ß = -9 vs. -58 respectively; P = 0.091), and anti-VEGF rates increased from 1533/1000 in 2012 to 2096/1000 in 2019 (ß = -57 vs. 187; P = 0.043). In eyes without DME, PRP use declined from 1017/1000 in 2012 to 707/1000 in 2019 (ß = -31 vs. -111, respectively; P < 0.001), and anti-VEGF use increased from 383/1000 in 2012 to 1226/1000 in 2019 (ß = -48 vs. 140, respectively; P < 0.001). CONCLUSIONS: Following the publication of Protocol S, PRP rates decreased, while anti-VEGF rates increased. Panretinal photocoagulation rates did not significantly change among eyes with DME. Our findings indicate the impact that randomized controlled trials can have on real-world practice patterns.

Rates of Reoperation in 10 114 Patients with Epiretinal Membranes Treated by Vitrectomy with or without Inner Limiting Membrane Peeling.

PURPOSE: To compare rates of reoperation in patients with idiopathic epiretinal membrane (ERM) who received pars plana vitrectomy (PPV) with or without inner limiting membrane (ILM) peeling and to assess trends in the overall use of ILM peeling over time. DESIGN: Retrospective cohort study. PARTICIPANTS: Patients included in the IBM Marketscan database between January 1, 2008, and December 31, 2016, who underwent surgery for idiopathic ERM. METHODS: Procedure claims with laterality codes were used to determine patients with idiopathic ERM who received PPV with or without ILM peel between 2008 and 2016, and to identify cases of reoperation and subsequent retinal detachment within 1 year of index surgery. MAIN OUTCOME MEASURES: The primary outcome was rate of reoperation for recurrent ERM according to whether or not patients receive an ILM peel during their index ERM surgery. We also assessed trends for index ERM surgery (ILM peel or no ILM peel) between 2008 and 2016, and the risk of developing retinal detachment within 1 year of the index ERM surgery. RESULTS: A total of 10 114 patients received ERM surgery and met inclusion criteria (5310 without ILM peel and 4804 with ILM peel). The reoperation rate was significantly lower among patients who received PPV with ILM peel (0.88%) compared with patients without ILM peel (1.48%; P = 0.007). In 2008, PPV without ILM peel represented 70% of ERM procedures, but PPV with ILM peeling accounted for 52% and 70% of ERM procedures in 2013 (P < 0.001) and 2016 (P < 0.001), respectively. The rates of retinal detachment were similar between patients who received an ILM peel (0.79%) and patients who did not receive an ILM peel (0.92%) during their primary ERM surgery (P = 0.474). CONCLUSIONS: The increasing use of PPV with ILM peeling to address ERM removal is associated with significantly reduced reoperation rates within 1 year. Future studies are needed to determine the cost-effectiveness of performing an ILM peel for initial idiopathic ERM repairs and evaluate long-term visual and structural changes related to ILM peeling.

Morning glory optic nerve in Aicardi syndrome: Report of a case with fluorescein angiography.

BACKGROUND: Aicardi syndrome is an X-linked condition that is associated with multiple ophthalmic malformations. Here, we report the first published fluorescein angiography (FA) study of a morning glory optic nerve in a patient with Aicardi syndrome and contralateral persistent fetal vasculature (PFV). CASE DESCRIPTION: A 12-day old full-term baby girl with a normal neurological exam was referred for evaluation of microphthalmia. The posterior segment of the right eye demonstrated chorioretinal lacunae typical of Aicardi syndrome and microphthalmos with a stalk consistent with PFV. The right eye imaging could not be captured due to the severe microphthalmos and cataract, however, fluorescein angioscopy was performed. The left eye demonstrated a morning glory appearing optic disc with peripapillary chorioretinal lacunae. Fluorescein angiography of the eye showed and late staining in the areas of ellipsoid chorioretinal lacunae emanating from the optic nerve and extensive peripapillary staining and late leakage of the optic nerve. CONCLUSION: Patients with Aicardi syndrome can have morning glory optic nerve anomaly and PFV. Using FA under anesthesia to detect these abnormalities help in estimating the extend of the disease and its complications, which allows for better management of the complications.

Assessment of Online Sites Reliability, Accountability, Readability, Accessibility, and Translation for Intravitreal Injections.

PURPOSE: Patients increasingly use the internet to access health-related information to better understand their treatments. This study compares the quality, accountability, readability, accessibility, and presence of translation between private and academic online source material available to the public regarding intravitreal injections (IVIs). DESIGN: Cross-sectional analysis. PARTICIPANTS: Top 20 websites on a Google search for the terms eye injections, intravitreal injections, and anti-VEGF injections. METHODS: Websites were classified as private or academic. Quality and accountability were assessed using the internationally recognized DISCERN criteria and the Health on the Net Code of Conduct (HONcode). Readability was evaluated using an online tool that provides a consensus readability grade. The presence of and languages available for translation were recorded. MAIN OUTCOME MEASURES: The primary outcome measure was a comparison of the DISCERN and HONcode quality and accountability scores between academic and private websites. Secondary outcome measures included evaluating readability, accessibility, and presence of translation (in particular, Spanish). RESULTS: Eleven academic and 9 private websites were included. The overall mean score using DISCERN criteria for the academic websites (3.11 ± 0.46) was significantly higher than that of private websites (2.23 ± 0.61; P < 0.007). Similarly, of a possible total of 14 points for the HONcode, the average quality score for academic websites (10.91 ± 2.66) was higher compared with that for private websites (6.44 ± 3.36; P < 0.009). The mean consensus reading grade level was similar between academic (11.73 ± 1.68) and private (11.78 ± 1.48) websites (P = 0.94). Spanish translation was offered by only 7 of the 20 websites (5 academic and 2 private websites). CONCLUSIONS: The overall quality and accountability of online content for academic sites was significantly higher compared with that of private websites. Translation was rarely provided, and the readability grade level was significantly higher for both groups than recommended. Improving the quality, accountability, readability, and accessibility and incorporating translation in websites can help to improve patients' health literacy regarding IVIs, potentially leading to increased adherence to therapy plans and improved treatment outcomes.

Complete RPE and outer retinal atrophy in patients receiving anti-VEGF treatment for neovascular age-related macular degeneration.

IMPORTANCE: Neovascular age-related macular degeneration (nAMD) is a leading cause of blindness with several intravitreal anti-vascular endothelial growth factor (anti-VEGF) agents available for its management such as aflibercept, bevacizumab, and ranibizumab. However, direct comparisons between these three agents among the same patient population are limited. OBJECTIVE: To assess the rate and growth of complete retinal pigment epithelium and outer retinal atrophy (cRORA) in eyes with nAMD treated with aflibercept, bevacizumab, and/or ranibizumab. METHOD: Retrospective cohort study of patients with treatment-naïve neovascular AMD seen at an academic hospital between October 2006 and February 2019. Study eyes were treated with intravitreal injections of aflibercept, bevacizumab, and/or ranibizumab and followed for two years. MAIN OUTCOMES AND MEASURES: cRORA prevalence, location, size, and growth rate. Eyes were imaged with Cirrus spectral domain optical coherence tomography (SD-OCT). Presence and size of cRORA were calculated using the FDA-approved Advanced RPE Analysis software. Linear regression models were used to correlate cRORA progression with baseline demographic and ocular characteristics, anti-VEGF drug, and number of injections. Unpaired t-tests, ANOVA, and linear regression models were computed with SAS 9.4. RESULTS: 197 eyes from 158 patients (mean age 78.9, 62.9% women) received an average of 13 anti-VEGF injections over 24 months. 22% developed new cRORA. Mean cRORA area increased from 1.71 mm2 to 2.93 mm2. At 24 months, eyes with 11+ injections had significantly less cRORA area (11+ injections, 4.02 mm2; ≤ 10 injections, 2.46 mm2; p = 0.01) and growth rate (11+ injections, 0.41 mm2/year; ≤ 10 injections, 1.05 mm2/year; p = 0.02). Choice of anti-VEGF drug yielded no significant difference in cRORA progression. CONCLUSIONS AND RELEVANCE: Treating nAMD with aflibercept, bevacizumab or ranibizumab demonstrated comparable cRORA development at 24 months. Number of injections inversely correlated with cRORA area and growth. These results warrant further investigation in the pathophysiology of cRORA in anti-VEGF treated eyes.

Anti-Vascular Endothelial Growth Factor Therapy for Radiation Retinopathy.

BACKGROUND AND OBJECTIVE: The purpose of this article is to review the role of anti-vascular endothelial growth factor (VEGF) therapy in treating patients with radiation retinopathy (RR). PATIENTS AND METHODS: RR can be associated with a significant decrease in visual acuity (VA) related to the development of cystoid macular edema, macular ischemia, and proliferative retinopathy leading to neovascular glaucoma. RESULTS: Anti-VEGF therapy is effective at stabilizing VA in around 80% of patients and achieving reductions in central macular thickness when it is administered using a constant algorithm. Furthermore, consistent prophylactic anti-VEGF therapy reduces the risk of development of RR, neovascularization of the iris, and neovascularization glaucoma. CONCLUSION: Future studies are needed to determine the optimal regimen for anti-VEGF therapy according to patient risk factors and likelihood of developing RR. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:S44-S49.].

Baseline choroidal thickness as a short-term predictor of visual acuity improvement following antivascular endothelial growth factor therapy in branch retinal vein occlusion.

PURPOSE: To evaluate the association between subfoveal choroidal thickness (SFCT) and branch retinal vein occlusion (BRVO) eyes treated with antivascular endothelial growth factor (anti-VEGF) therapy. METHODS: Retrospective cohort study of treatment naïve BRVO eyes treated with 3 monthly anti-VEGF injections. All patients received enhanced depth imaging spectral-domain optical coherence tomography scans to determine SFCT and central macular thickness (CMT). Baseline predictors (particularly SFCT) for functional response (best-corrected visual acuity (BCVA) gain ≥2 lines) were assessed at 3 months using univariate and multivariate analyses. RESULTS: Forty eyes from 39 patients were included. Mean baseline SFCT was higher in functional responders (240.4±73.1 µm), compared with both non-responders (193.3±63.6 µm; p=0.036) and their corresponding fellow eye (202.2±67.1 µm; p=0.022). A higher baseline SFCT (for every 100 µm increase in SFCT) was found to be a positive predictor for functional response (regression coefficient: 1.1; p=0.03) on univariate analysis but not multivariate analysis. A worse baseline BCVA (for every 0.1 logMAR increase) was a positive predictor for visual improvement with an adjusted OR of 1.30 (95% CI 1.03 to 1.63; p=0.0009) on multivariate analysis. CONCLUSIONS: Patients with BRVO with a worse initial BCVA are most likely to achieve visual improvement following anti-VEGF therapy. Additionally, baseline SFCT may also help predict which patients with BRVO have favourable visual outcomes. Patients with an initial choroidal thickness thicker than their fellow eye are more likely to have short-term visual improvement following treatment.

LONG-TERM VISUAL OUTCOMES AND CLINICAL FEATURES AFTER ANTI-VASCULAR ENDOTHELIAL GROWTH FACTOR INJECTION-RELATED ENDOPHTHALMITIS.

PURPOSE: To determine long-term visual outcomes in patients who developed endophthalmitis after intravitreal anti-vascular endothelial growth factor injections and to correlate visual outcomes with clinical features. METHODS: This is a retrospective, multicenter, consecutive case series of patients diagnosed with anti-vascular endothelial growth factor injection-related endophthalmitis who were treated at Mid Atlantic Retina, the Retina Service of Wills Eye Hospital, Philadelphia, PA, and the University of Southern California Roski Eye Institute, Los Angeles, CA. Patients were included if they had at least 1 year of follow-up. Primary outcome was to evaluate long-term visual outcomes up to 5 years of follow-up. The secondary outcome was to determine clinical features (e.g., culture results) that may predict long-term visual acuity outcomes. RESULTS: A total of 56 cases of endophthalmitis from 168,247 anti-vascular endothelial growth factor injections were identified (0.033%, 1/3,004 injections), from which 51 eyes met inclusion criteria. Mean follow-up period was 3.3 years (median 4 years; range 1-5 years). A total of 24 patients (47%) reached a maximum final follow-up of 5 years. Mean Snellen visual acuity at the causative injection visit was 20/102 and decreased to counting fingers at diagnosis (P < 0.001). At 6-month follow-up, mean visual acuity improved to 20/644 (P < 0.001) and remained stable up to 5 years (20/480, P = 0.003) follow-up compared with diagnosis. At the final follow-up, 20 eyes had visual acuity that returned to within one line of baseline visual acuity (visual recovery group), whereas 31 patients' visual acuity was at least one line worse than initial visual acuity (visual deterioration group). The cultures for the visual recovery group were more likely to grow coagulase-negative Staphylococcus, whereas the visual deterioration group primarily grew Streptococcus species, Staphylococcus aureus, and Enterococcus faecalis (P = 0.002, comparing organisms isolated in the visual recovery and deterioration group). CONCLUSION: Visual outcomes after anti-vascular endothelial growth factor injection-related endophthalmitis seem to reach peak improvement by 6 months and remain stable up to a median of 4-year follow-up. Patients who develop culture-negative endophthalmitis or endophthalmitis secondary to coagulase-negative Staphylococcus are more likely to regain baseline visual acuity compared with cases secondary to Streptococcus species.

Outcomes of choroidal melanomas treated with eye physics plaques: A 25-year review.

PURPOSE: To review long-term outcomes of the University of Southern California Plaque Simulator (PS) software and Eye Physics (EP) plaques. We hypothesize that the PS/EP system delivers lower doses to critical ocular structures, resulting in lower rates of radiation toxicity and favorable visual outcomes compared to Collaborative Ocular Melanoma Study plaques, while maintaining adequate local tumor control. METHODS AND MATERIALS: Retrospective review of 133 patients treated for choroidal melanoma with 125I brachytherapy, using PS software and EP plaques, from 1990 through 2015. A dose of 85 Gy at a rate of 0.6 Gy/h was prescribed to the tumor apex (with a typical margin of 2 mm) over 7 days. Primary outcomes were local tumor recurrence, globe salvage, and metastasis. Secondary outcomes were changes in visual acuity and radiation complications. RESULTS: With median followup of 42 months, 5-year Kaplan-Meier estimated rates for tumor control, globe salvage, and metastatic-free survival were 98.3%, 96.4%, and 88.2%, respectively. Median doses to the macula and optic nerve were 39.9 Gy and 30.0 Gy, respectively. Forty-three percent of patients developed radiation retinopathy, and 20% developed optic neuropathy; 39% lost ≥6 Snellen lines of vision. CONCLUSIONS: The PS/EP system is designed to improve the accuracy and conformality of the radiation dose, creating a steep dose gradient outside the melanoma to decrease radiation to surrounding ocular structures. We report favorable rates of local tumor control, globe salvage, metastases, and radiation complications when compared to the Collaborative Ocular Melanoma Study and other studies. Overall, the PS/EP system results in excellent tumor control and appears to optimize long-term visual and radiation-related outcomes after brachytherapy.

Orbital Paraganglioma: A Systematic Review.

Purpose The purpose of this study was to perform a systematic review of the literature on orbital paragangliomas, evaluating clinical presentation, diagnosis, management patterns, and prognosis. Methods The systematic review was conducted based on the principles described in the Preferred Reporting Items for Systematic Reviews and Meta-Analysis. PubMed, Cochrane databases, Embase, and Web of Science were searched for articles related to orbital paragangliomas. Inclusion criteria included English language articles with original reports on human subjects. Data on clinical presentation, diagnosis, treatment, and prognosis were collected. Results Twenty-seven articles met inclusion criteria (28 total patients). The mean patient age was 37.1 years (range, 3-75 years); 13 (46.4%) patients were male. The most common presenting symptoms were proptosis (89.2%), visual acuity changes (67.9%), and extraocular muscle restriction (64.2%). Lesions were most commonly intraconal (92.9%). Single modality therapy was employed in 19 patients (67.9%), including excision in 12 patients (42.9%) and exenteration in 5 patients (17.9%). Ten patients (35.7%) developed recurrence, and there was a 92.9% survival rate (mean follow-up, 29 months). Conclusion Orbital paragangliomas are rare tumors with an excellent prognosis. These lesions commonly present with proptosis, and are primarily managed surgically with simple excision, although exenteration and adjuvant radiation may be necessary for invasive tumors. This series is the largest and most comprehensive systematic review of orbital paragangliomas conducted to date.