Objective Characterization of the structural and functional progression of ocular von Hippel-Lindau (VHL) disease and analysis of patient factors influencing disease progression. Design Retrospective analysis of a case series from a longitudinal observational study. Participants Two hundred and forty-nine participants with clinically-defined systemic VHL disease and greater than two years of ophthalmic follow-up. Methods Standardized scoring of ocular phenotype and systemic characteristics was performed at each study visit and analyzed longitudinally to determine progression of ocular VHL disease. Main Outcome Measures Measures evaluated include: visual acuity, features of ocular VHL disease (presence, location, number, and extent of retinal capillary hemangioblastomas [RCHs]), germline mutation in the VHL gene, demographics (age, sex, age of onset of ocular disease), and patient characteristics (smoking status, body mass index). Results A majority of participants demonstrated relative anatomical and functional stability in ocular VHL disease status over a mean follow-up period of 8.2±4.0 years. About three-quarters (73%) of participants without ocular VHL disease at baseline remained disease-free at the end of follow-up. Among eyes with ocular VHL disease at baseline, 88% did not develop RCH in a new retinal location, 70% remained stable in RCH number, and 79% remained stable in the extent of RCH involvement. Mean visual acuity for all study eyes (n = 498) decreased by 5.1±0.6 letters across follow-up, with 16.1% decreasing by more than 10 letters. Among eyes affected at baseline, greater vision loss was associated with the presence of juxtapapillary RCHs, development of RCH in a new location, and increase in peripheral RCH number and extent. Younger baseline age, younger age at onset of ocular VHL disease, involvement of fellow eye with ocular VHL disease, and missense or protein-truncating germline mutations were significantly associated with increased anatomical involvement and functional deterioration. Conclusions Patients with ocular VHL disease maintain relative anatomical and functional stability, with only a minority demonstrating marked anatomical progression and marked vision loss. Systemic and ocular risk factors for anatomical progression and vision loss can help practitioners identify patients with a higher risk profile for counseling, closer follow-up, and proactive treatment.
Purpose To qualitatively and quantitatively compare Optos© fundus camera fluorescein angiographic images of retinal vascular leakage with 9-field montage Topcon© fluorescein angiography (FA) images in patients with uveitis. We hypothesized that Optos images reveal more leakage in uveitis patients. Design Retrospective, observational case series. Methods Images of all uveitis patients imaged with same-sitting Optos FA and 9-field montage FA during a 9 month period at a single institution (52 eyes of 31 patients) were graded for the total area of retinal vascular leakage. The main outcome measure was area of fluorescein leakage. Results The area of apparent FA leakage was greater in Optos images than in 9-field montage images (median 22.5 mm2 vs. 4.8 mm2, P<0.0001). Twenty-two of 49 (45%) eyes with gradable photos had at least 25% more leakage on the Optos image than on the montage image. Two (4.1%) had at least 25% less leakage on Optos, and 25 (51%) were similar between the two modalities. Two eyes had no apparent retinal vascular leakage on 9-field montage but were found to have apparent leakage on Optos images. Twenty-three of the 49 eyes had posterior pole leakage, and of these 17 (73.9%) showed more posterior pole leakage on the Optos image. A single 200 degree Optos FA image captured a mean 1.50x the area captured by montage photography. Conclusion More retinal vascular pathology, both in the periphery and the posterior pole, is seen with Optos FA in uveitis patients when compared with 9-field montage. The clinical implications of Optos FA findings have yet to be determined.
Purpose To evaluate whether dietary intake of lutein/zeaxanthin and B vitamins is associated with cataract prevalence and incidence. Design Clinic-based, baseline cross-sectional and prospective cohort study designs. Participants 3115 (6129 eyes) persons enrolled in the Age-Related Eye Disease Study, aged 55 to 80 years, followed for mean of 9.6 years. Methods Participants completed baseline food frequency questionnaires. Baseline and annual lens photographs were graded centrally. Multivariable models controlling for previously identified risk factors for cataracts were used to measure the association of cataracts with reported dietary intake, using the lowest quintile as reference. Main Outcome Measures Cataract surgery, cataract status (type and severity) at baseline, development of cataracts. Results At baseline, increased dietary riboflavin and B12 were inversely associated with nuclear and cortical lens opacities. In comparisons of persons with and without cataract, persons with the highest riboflavin intake vs. those with the lowest intake had the following associations: odds ratio (OR): 0.78, 95% confidence interval (CI): 0.63–0.97 for mild nuclear, OR: 0.62, 95% CI: 0.43–0.90 for moderate nuclear, and OR: 0.80, 95% CI: 0.65–0.99 for mild cortical cataracts. For B12, the results were: OR: 0.78, 95% CI: 0.63–0.96 for mild nuclear, OR: 0.62, 95% CI: 0.43–0.88 for moderate nuclear, and OR: 0.77, 95% CI: 0.63–0.95 for mild cortical cataracts. Highest dietary B6 intake was associated with a decreased risk of developing moderate nuclear lens opacity compared with the lowest quintile, OR: 0.67, 95% CI: 0.45–0.99. Highest dietary intake levels of niacin and B12 were associated with a decreased risk of development of mild nuclear or mild cortical cataracts in participants not taking Centrum® multivitamin. For participants taking Centrum® during the study, highest intake of dietary folate was associated with an increased risk of development of mild posterior subcapsular lens opacity. No statistically significant associations were found between lutein/zeaxanthin intake and presence at baseline or development of nuclear or cortical lens opacity outcomes. Conclusions Findings from our study are consistent with earlier studies suggesting that dietary intake of B vitamins may affect the occurrence of age-related lens opacities. Further investigations are warranted.
Purpose This study evaluated a novel tool known as the motion diamond stimulus (MDS), which utilizes contrast-generated illusory motion in dynamic test regions to determine contrast sensitivity (CS). Methods Patients with treated unilateral retinal vein occlusions (RVOs) underwent three assessments: the MDS, the Pelli-Robson (PR), and the National Eye Institute's Visual Function Questionnaire (VFQ-25). The MDS assessment produced two data end points, α and β. The α value represents the overall contrast threshold level and the β value serves to quantify the adaptability of the visual contrast system. The CS parameters from the MDS and log CS PR output values were used to compare RVO eyes ( n = 20) to control eyes ( n = 20). Results The study participants had a mean composite VFQ-25 score of 89.5 ± 10.4 on the VFQ-25. A significant difference was observed between the RVO eyes and the control eyes in PR log CS scores ( P value = 0.0001) and in MDS α value ( P value = 0.01). No difference in MDS β value was found between the study groups ( P value = 0.39). Conclusions The results for the MDS assessment's α parameter corroborated the PR scores, suggesting contrast sensitivity threshold impairment in patients with RVO. No significant difference in β value was observed, suggesting that adaptability of the visual system is maintained in treated RVO eyes. Translational Relevance Currently, visual complaints cannot be entirely identified by Snellen visual acuity alone. The MDS offers potentially a more complete look at visual function, by including contrast sensitivity and may be able to quantify changes otherwise overlooked in retinal disease progression.
Purpose To investigate pigment clumping in idiopathic macular telangiectasia type 2 (IMT2) for its incidence, development, and progression during the course of the disease. Methods Patients with a diagnosis of IMT2 and greater than 12 months of follow-up were reviewed retrospectively. Measurements of the area of pigment clumping were performed and correlated with visual acuity and findings on spectral domain optical coherence tomography (OCT) and microperimetry (MP1). Results Fifty-three eyes in 27 patients with a mean follow-up of 42.5±14.2 months (range 12–79 months) were included. At study baseline, 16 eyes (30%) had evidence of pigment clumping without associated neovascular changes. During follow-up, 8/33 (24%) additional study eyes without prior pigment clumping developed it in stage 3 (Gass-Blodi classification) disease. Pigment clumping increased in overall area as a function of follow-up time. Pigment clumping was associated with increased intraretinal reflectivity on OCT and development of scotomas on microperimetry. Conclusions Pigment clumping commonly develops in stage 3 IMT2 disease, enlarges in area continuously over time, and is associated with declining visual function. Longitudinal measurements of the total area of pigment clumping may be helpful in following disease progression and may constitute a useful outcome measure for interventional clinical studies.
Purpose The purpose of this one-year prospective study was to investigate how induction/pro re nata (PRN) ranibizumab intravitreal treatment of eyes with neovascular age related macular degeneration affects the anatomy of choroidal neovascularization (CNV) and the overlying outer retinal tissue. Methods High speed indocyanine green angiography (HS-ICG) measurements provided quantification of the CNV size in 60 patients followed for one year. Minimum intensity projection optical coherence tomography (minIP OCT), a novel algorithm assessing minimum optical intensity between the internal limiting membrane and retinal pigment epithelium, measured the area of outer retinal disruption overlying the CNV. Fluorescein angiography (FA) was also assessed to evaluate late retinal leakage. Results After one year, mean area of CNV measured with ICG decreased by 5.8%. MinIP OCT mean area of outer retinal disruption overlying the CNV decreased by 4.2%. Mean area of FA leakage decreased by 6.3%. Both the area of outer retinal disruption measured with minIP OCT and the area of leakage on FA typically exceeded the area of CNV on ICG at baseline and one year. Conclusion CNV treated with induction/PRN intravitreal ranibizumab for one year essentially remained static. MinIP OCT suggests that the area of outer retinal disruption overlying the CNV may be greater than the CNV itself and often correlates with the leakage area on fluorescein angiography. Additionally, there was minimal change in the area of outer retinal disruption on MinIP OCT even when fluid resolved. Measurements of the extent of CNV lesions based on ICG and minIP OCT may provide useful outcome variables to help assess the CNV complex longitudinally and warrant further validation.
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