Spatial correlation between hyperpigmentary changes on color fundus photography and hyperreflective foci on SDOCT in intermediate AMD.

PURPOSE: Macular hyperpigmentation is associated with progression from intermediate to advanced age-related macular degeneration (AMD). The purpose of this study was to accurately correlate hyperpigmentary changes with spectral domain optical coherence tomography (SDOCT) hyperreflective foci in eyes with non-advanced AMD. METHODS: A prospective cross-sectional analysis of 314 eyes (314 subjects) with intermediate AMD was performed in the multicenter Age-Related Eye Disease Study 2 (AREDS2) Ancillary SDOCT Study to correlate hyperpigmentary changes on color fundus photographs (CFP) with abnormal morphology on SDOCT. Spatial coregistration was performed with an automated algorithm in two nonoverlapping subsets of 20 study eyes, which permitted double-masked CFP and SDOCT grading by certified investigators. RESULTS: Macular CFP hyperpigmentation was significantly associated with SDOCT intraretinal hyperreflective foci in the 314 study eyes (P < 0.001). In a substudy of 40 eyes, automated intermodality spatial coregistration was successfully achieved in all 136 (100%) retinal regions selected for CFP and SDOCT grading. In one subset of 20 study eyes, 28 of 39 (71.8%) retinal CFP regions with hyperpigmentation were correlated with focal hyperreflectivity on SDOCT, versus seven of 39 (17.9%) control regions (P < 0.001). In another subset of 20 eyes, 21 of 29 (72.4%) SDOCT regions with hyperreflective foci were correlated with hyperpigmentary changes on CFP, versus two of 29 (6.9%) control regions (P < 0.001). CONCLUSIONS: A novel algorithm achieves automated intermodality spatial coregistration for masked grading of regions selected on CFP and SDOCT. In intermediate AMD, macular hyperpigmentation has high spatial correlation to SDOCT hyperreflective foci and often represents the same anatomical lesion. (ClinicalTrials.gov number, NCT00734487.).

Baseline characteristics of participants in the natural history study of macular telangiectasia (MacTel) MacTel Project Report No. 2.

PURPOSE: To describe the baseline characteristics of a large international cohort of patients with macular telangiectasia type 2 (MacTel Type 2) in anticipation of a longitudinal natural history study to evaluate structural and functional changes, identify potential risk factors and related outcomes. METHODS: Images including fundus photographs, fluorescein angiograms, optical coherence tomography and fundus autofluorescence were collected. A grading system for MacTel type 2 was developed by the central reading center to evaluate lesion characteristics. Relationships between lesion characteristics and visual acuity were evaluated. RESULTS: A total of 310 participants have been enrolled in the study. The mean time since diagnosis was 3 years (range 0 to 25 years). The mean age at the baseline examination was 61 +/- 9 years. The mean visual acuity in the better eye was approximately 20/32 Snellen equivalents and approximately 20/50 in the worse eye. The visual acuity in the better eye of half of the participants was 20/32 or better. We found some relationships between visual acuity and lesions characteristic of MacTel Type 2. CONCLUSIONS: This is the first large-scale study of patients with MacTel Type 2. More than half of the patients had 20/32 or better vision in their better eye, which is a sign that decreased function in these participants may not be reflected in central visual acuity. These findings highlight the limitation of using visual acuity measurements as a measure of function and as an outcome measure in potential clinical trials in patients with MacTel Type 2.

The National Eye Institute Visual Function Questionnaire in the Macular Telangiectasia (MacTel) Project.

PURPOSE: To describe vision-targeted health-related quality of life (HR-QOL), measured with the National Eye Institute Visual Functioning Questionnaire (NEI-VFQ-25) in a cohort of patients with macular telangiectasia (MacTel) type 2 and to evaluate the relationship between visual acuity and NEI-VFQ-25 scores. METHODS: This was an analysis of cross-sectional baseline data from a longitudinal natural history study. Patients with MacTel type 2 were enrolled in the Natural History Study of The Macular Telangiectasia Project (The MacTel Project). NEI-VFQ-25 were completed at enrollment. Linear correlation and regression analyses were used to relate baseline NEI-VFQ-25 overall and subscale scores to visual acuity. RESULTS: Participants reported lower vision-related functioning measured by the NEI-VFQ-25 in most of the domains measured by the NEI VFQ compared with that of a normal reference group (P < 0.001 for all domains except color vision). Visual acuity was found to be associated with the NEI-VFQ-25 in many of the domains measuring degree of difficulty with common visual activities. CONCLUSIONS: This is the first cross-sectional cohort study to assess vision targeted HR-QOL in patients with MacTel type 2. Patients with MacTel type 2 reported markedly reduced visual functioning compared to reports of a normal reference group. These findings provide support to the use of the NEI-VFQ-25 in patients with MacTel type 2 to measure the effect of disease and potential therapies on vision-targeted HR-QOL.