Do not discount the diagnosis of VKH based on race: self-reported race and ethnicity of patients with Vogt-Koyanagi-Harada disease in a predominantly white population.

BACKGROUND: We examined the racial and ethnic distribution of patients with Vogt-Koyanagi-Harada disease (VKH) in a Midwestern US population through a retrospective chart review of patients with VKH seen in a tertiary referral centre between 2012 and 2017. All patients were diagnosed by one uveitis specialist (DAG). We identified 32 patients with VKH seen during this time period. The mean age at diagnosis was 37.7 ± 15.7 years, 7 were male, 25 female. Mean follow-up was 36.7 ± 21.7 months. Nine patients reported themselves as White non-Hispanic, (28.1%), 9 as Black/African-American (28.1%), 2 as Asian (6.3%) and 9 as Hispanic or Latino (28.1%). Three patients (9.4%) were of Middle-Eastern origin. The 2010 census results for race and ethnicity in the state of Illinois were: 71.5% White, 14.5% Black/African-American, 4.6% Asian, and 6.7% as Some Other Race. From the total population 15.8% reported themselves as Hispanic or Latino (of any race). CONCLUSIONS: VKH was much more frequent among white non-Hispanic patients (28.1%) and Black/African-American patients (28.1%) in our patient population than in previous reports from the US (3-14% and 4-23% respectively). While Hispanic patients in this series were over represented in the VKH population compared with the overall census data, the percentage of VKH patients in this series who were White non-Hispanic and Hispanic was the same. The diagnosis of VKH should be considered in any patient with the appropriate clinical features, regardless of race or ethnicity.

Diagnosing Vitreoretinal Lymphomas-An Analysis of the Sensitivity of Existing Tools.

Vitreoretinal lymphoma (VRL) is a rare ocular pathology that is notorious for mimicking chronic uveitis, which is a seemingly benign condition in comparison. The most common form of VRL is the diffuse large B-cell type, and there has been a high mortality rate. This dismal prognosis can be improved significantly if the disease is diagnosed early, but until now there is no consensus on an appropriate diagnostic algorithm. We conducted a retrospective search of PubMed Central® and analyzed results from thirty-three studies that were published between 2011-2021. The chosen studies incorporated some popular testing tools for VRL, and our analyses focused on comparing the average sensitivity of five diagnostic methods. The methods included cytology including ancillary immunohistochemistry, Myeloid Differentiation Factor 88 (MyD88) mutation analysis, polymerase chain reaction (PCR) for monoclonal rearrangements of immunoglobulin heavy chain (IgH) and T-cell Receptor (TCR) genes, flow cytometry, and IL10 and IL6 analysis. Across the varied diagnostic methods employed in thirty-three studies explored in this analysis, MyD88 mutation assay emerged as a strong contender given its sensitivity and low coefficient of variation. There is an imminent need for the introduction of newer assays that can further improve the sensitivity of identifying MyD88 mutation in cancer cells seen in the vitreous.

SEGMENTAL DIFFUSE VASCULAR LEAKAGE: A FLUORESCEIN ANGIOGRAPHIC FINDING IN PATIENTS WITH VON HIPPEL-LINDAU DISEASE.

PURPOSE: To describe a novel fluorescein angiographic finding in patients with von Hippel-Lindau disease. METHODS: Retrospective case series of patients diagnosed with retinal capillary hemangioblastoma (RCH) in the setting of von Hippel-Lindau. RESULTS: We identified six eyes of three patients with von Hippel-Lindau and leaky retinal vessels. All eyes showed segmental diffuse vascular leakage (SDVL) that was seen in the late phase of the angiogram and that originated from third order and more peripheral retinal veins and adjacent capillaries. These vessels did not drain from the RCHs. Segmental diffuse vascular leakage was mainly seen in the mid and far periphery. In some cases, it was located near the RCHs, while in other cases, it was remote. Segmental diffuse vascular leakage was also seen in one eye without RCHs. On follow-up, the extent and intensity of segmental diffuse vascular leakage did not change after the RCHs were treated with laser or cryotherapy. CONCLUSION: Diffuse vascular leakage from retinal venules around and away from RCHs in patients with von Hippel-Lindau disease is seen, but the clinical and prognostic importance of this finding is uncertain.

Vascular density in age-related macular degeneration after one year of antiVEGF treatment with treat-and-extend and fixed regimens.

Treatment of neovascular age-related macular degeneration (nAMD) with VEGF can be performed with several posologies. The purpose of our cross-sectional study was to analyze retinal vessel density by quantitative OCT-angiography (OCT-A) and to compare treat-and-extend (T&E) and fixed treatment protocols to a control group with dry AMD. Altogether 48 patients were enrolled: 13 eyes with T&E protocol ranibizumab treatment (group A) and 17 eyes with fixed regimen aflibercept therapy (group B), the control group comprised 18 eyes with dry AMD (group C). One year after the start of the treatment, quantitative OCT-A (AngioVue-Optovue, Fermont, USA) was performed: superficial and deep retinal vessel densities were analyzed in the foveal and parafoveal regions. Our results show, that the density of retinal superficial vasculature in the fovea was not different between the treatment groups (A: 25.9±9.1%; B: 24.3%±8.9), neither from group C (25.6±4.8%). Superficial parafoveal vascular density of the retina, however, was decreased in both treated groups (A: 46.7±9.1%, B: 42.9±6.1%, C: 49.7±4.9%). In the deep retinal plexus, vascular density was lower in both treatment groups compared to that of in controls in both the foveal and parafoveal area (A: 29.8±6.3%, B: 32.5±6.9%, C: 36.4±1.7% and A: 46.3±3.8%, B: 47.1±5.3%, C: 49.7±4.9%, foveal and parafoveal respectively). Our data suggest, that after one year of anti-VEGF treatment, reduced macular vessel density in three of the four examined vascular regions can be found independent of the treatment regimen.

Novel imaging modalities in patients with uveitis.

Imaging is one of the key elements in the diagnosis and management of ocular diseases. This is particularly true of retinal conditions. In this review we discuss novel imaging modalities such as optical coherence tomography angiography (OCTA), choroidal optical coherence tomography (OCT), anterior chamber OCT, as well as widefield imaging modalities and fundus autofluorescence. We describe the advantages and shortcomings of these imaging modalities in comparison to the more traditional ones, as well as some common pitfalls. We also provide examples of how these newer imaging methods can aid clinicians in their decision making, and provide novel insights into disease pathogenesis.

Vertical Hyperreflective Lesions on Optical Coherence Tomography in Vitreoretinal Lymphoma.

Importance: Vitreoretinal lymphoma is a diagnostic challenge and the pathophysiology is still unclear. Objective: To describe an imaging finding seen on optical coherence tomography (OCT) of patients with vitreoretinal lymphoma. Design, Setting, and Participants: This case series study was a retrospective medical record review of patients who received a diagnosis of vitreoretinal lymphoma at the Department of Ophthalmology at Northwestern University between July 2014 and January 2016. Main Outcomes and Measures: Optical coherence tomography findings in vitreoretinal lymphoma. Results: We identified 7 patients (4 women [57.1%]; mean [range] age, 62.4 [45-75] years; 12 eyes) with intraocular lymphoma involving the retina (5 patients [71.4%] with primary vitreoretinal or central nervous system lymphoma with ocular involvement, 1 patient [14.3%] with testicular lymphoma with secondary central nervous system lymphoma and vitreoretinal lymphoma, and 1 patient [14.3%] with secondary vitreoretinal lymphoma). We identified vertical hyperreflective lesions that showed moderate or high reflectivity and affected all layers of the neuroretina in 5 patients (7 of 12 eyes [58.3%]). These often preceded the development of subretinal pigment epithelial deposits and were often localized around second-order and third-order retinal vessels. In most cases, they resolved with minimal or no scarring after the initiation of chemotherapy. Conclusions and Relevance: Vertical hyperreflective lesions are a common physical finding on OCT in eyes with vitreoretinal lymphoma.

Effect of retinal photocoagulation on intraretinal lipid exudates in diabetic macular edema documented by optical coherence tomography.

PURPOSE: To study the changes in the distribution and morphologic features of intraretinal microexudates after macular photocoagulation. DESIGN: Prospective cohort study. PARTICIPANTS: Thirteen treatment-naïve patients with clinically significant macular edema in type 2 diabetes. METHODS: Patients were treated with focal macular photocoagulation. Changes in the localization of hyperreflective foci were analyzed by spectral domain (SD) optical coherence tomography (OCT) during follow-up at day 1, week 1, and months 1, 2, 3, and 4 in defined areas. Further, fundus photography and infrared imaging were performed at all visits and findings were correlated to OCT results. MAIN OUTCOME MEASURES: Changes in retinal morphologic features detected in OCT. RESULTS: A dynamic change in the distribution pattern of hyperreflective foci was observed over 4 months after the photocoagulation. With the decrease of retinal thickness, the dots either resolved completely or became confluent at the apical border of the outer nuclear layer, and finally formed ophthalmoscopically detectable hard exudates during extended follow-up. In the event of retinal thickening despite laser treatment, the hyperreflective dots maintained their previous distribution throughout all retinal layers. As a fourth response, dissemination of plaques of hard exudates into multiple, separate, hyperreflective foci were detected. CONCLUSIONS: Hyperreflective foci in the retina seem to represent precursors or components of hard exudates. Their specific localization depends greatly on the presence of microvascular extravasation and intraretinal fluid accumulation. Retinal photocoagulation has a major impact on retinal edema and subsequently on the distribution of intraretinal lipid deposits. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.