Longitudinal anatomical and visual outcome of macular telangiectasia type 2 in Asian patients.

Limited information regarding the anatomical and visual prognosis of macular telangiectasia (MacTel) type 2 in the Asian population is currently available. Herein, we conducted a retrospective longitudinal analysis of Japanese patients diagnosed with MacTel type 2. Disease progression was evaluated using the Simple MacTel Classification developed by Chew EY et al. in 2023, and its association with visual changes was analyzed. Sixteen eyes of eight Japanese patients were included in the study, with an average follow-up period of 8.2 ± 3.9 years (range, 2.2-14.0). At the initial visit, 7 (44%) and 5 (31%) eyes were classified as Grade 2 (central ellipsoid zone break) and Grade 3 (noncentral pigment), respectively. The proportion of eyes that progressed by 1 or 2-steps in grade after 1, 3, 5, 8, and 12 years was 0%, 14%, 43%, 70%, and 100%, or 0%, 7%, 7%, 30%, and 75%, respectively. The visual acuity significantly deteriorated during the follow-up period, particularly in the two eyes with full-thickness macular holes (FTMH). Three out of 7 patients exhibited low serum serine concentrations, although no apparent correlation with anatomical or visual outcomes was observed. Overall, this cohort demonstrated chronic disease progression, both anatomically and functionally, in eyes with MacTel type 2, with FTMH potentially associated with greater visual loss.

Investigating the Usefulness of Fundus Autofluorescence in Retinitis Pigmentosa.

PURPOSE: To investigate the relationship between visual field (VF) loss and the rings of the ellipsoid zone (EZ) and autofluorescence (AF) in patients with retinitis pigmentosa (RP). DESIGN: Cross-sectional study. PARTICIPANTS: A total of 28 eyes of 15 patients with a clinical diagnosis of RP. METHODS: The VF was measured at a 1-degree interval on the horizontal or vertical lines, within the central 10 degrees from fixation, using MP-3 microperimetry (Nidek Co. Ltd., Aichi, Japan), and the sensitivity deviation from the normative database was calculated. OCT was performed, and the EZ was identified. Fundus AF was also measured, and the AF ring was identified. The VF test points were categorized according to their relationship with the EZ line and AF ring. The relationship between mean retinal sensitivity deviations on both the horizontal and vertical lines and the diameters of the EZ line and the AF ring in the corresponding directions was analyzed. The relationship between visual sensitivity deviation and the locations against EZ line and AF rings was also investigated. MAIN OUTCOME MEASURES: The relationship between the visual sensitivity deviation and the locations against EZ line and AF rings. RESULTS: The diameter of the AF ring had a significantly stronger relationship with mean retinal sensitivity deviation compared with the diameter of the EZ line in both the horizontal and vertical directions. Test point location inside/on/outside the AF ring was significantly related to retinal sensitivity deviation independent of the location inside/outside the EZ line (P < 0.001, linear mixed model). CONCLUSIONS: Visual sensitivity damage is significantly associated with the EZ lines and AF rings. The development of the AF ring is related to the decrease of visual function independent of and stronger than the EZ line.