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Ferenc B. Sallo, Tunde Peto, Catherine Egan, Ute E. K. Wolf-Schnurrbusch, Traci E. Clemons, Mark C. Gillies, Daniel Pauleikhoff, Gary S. Rubin, Emily Y. Chew, Alan C. Bird, the MacTel Study Group; The IS/OS Junction Layer in the Natural History of Type 2 Idiopathic Macular Telangiectasia. Invest. Ophthalmol. Vis. Sci. 2012;53(12):7889-7895. doi: https://doi.org/10.1167/iovs.12-10765.
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To document the progression of a break in the photoreceptor inner segment/outer segment (IS/OS) junction layer and its functional correlates over time in the natural history of type 2 idiopathic macular telangiectasia (type 2 MacTel).
Patients with at least 1 year of follow-up were selected from the MacTel Study. En face images were created by manual segmentation of the IS/OS junctional line in volume scans acquired using a spatial-domain optical coherence tomography retinal imaging unit. Retinal sensitivity thresholds were determined using a retinal microperimeter unit. Aggregate retinal sensitivity loss within IS/OS lesions was calculated. Changes over time in an area of IS/OS defects and retinal sensitivity were analyzed.
Thirty-nine eyes of 23 patients (mean age: 62.3 ± 9.2 years) were analyzed. Mean follow-up time was 1.9 years (range: 1–3 years). Mean IS/OS break area at baseline was 0.575 mm2 (SE = 0.092, 95% confidence interval [CI]: 0.394–0.756 mm2). The cluster-adjusted mean annual progression rate in IS/OS break area was 0.140 mm2 (SE = 0.040, 95% CI: 0.062–0.218 mm2, P < 0.001). Mean aggregate retinal sensitivity loss was at baseline 28.56 dB (SE = 5.43, 95% CI: 17.32–39.80 dB, n = 28), a positive correlation with IS/OS lesion area was present (P < 0.001). The mean annual rate of change in aggregate sensitivity loss was 5.14 dB (SE = 1.51, 95% CI: 2.19–8.10 dB, P < 0.001, n = 37), a significant correlation with lesion area increase was found (P = 0.006).
Both IS/OS break area and rate of enlargement correlate with aggregate retinal sensitivity loss in type 2 MacTel. En face OCT imaging of the IS/OS layer provides a functionally relevant method for documenting disease progression in type 2 MacTel.
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