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Mohamed A Ibrahim, Rupert Wolfgang Strauss, Xiangrong Kong, Ann-Margret Ervin, Alexander Ho, Janet S Sunness, Isabelle S Audo, David G Birch, Srinivas R Sadda, Millena G Bittencourt, Hendrik P Scholl; Scotopic and photopic macular functions as assessed with microperimetry (MP1) in patients with Stargardt disease type 1 – The SMART Study. Invest. Ophthalmol. Vis. Sci. 2016;57(12):2696.
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© ARVO (1962-2015); The Authors (2016-present)
To characterize scotopic and photopic macular function with MP1S in patients with Stargardt disease type 1 (STGD1) in the SMART Study (Scotopic Microperimetric Assessment of Rod Function in Stargardt Disease)
Patients with confirmed mutations in the ABCA4 gene and STGD1 phenotype were enrolled (sub-cohort of the ProgStar Study). One eye from every patient (best eye) underwent testing using a custom pattern on the MP1S system (Nidek Technologies), before (mesopic; m) and after (scotopic; s) dark-adaptation. Mesopic testing used a white stimulus; scotopic testing a blue stimulus with a combined neutral density filter to align sensitivity with the dynamic range of the MP1S and a 500nm short-pass filter. Tests were graded at the Doheny Image Reading Center. Mean sensitivity (MS) in the ring 4-10 degrees from the fovea was calculated for both the mesopic (mMS) and scotopic (sMS) tests. The distance of center of gravity of fixation points (preferred retinal locus or PRL) from the anatomical fovea was measured along with the 2SD bivariate contour ellipse area (BCEA). Descriptive statistics and correlation coefficients were used to assess the relationship between photopic and scotopic outcome variables.
136 patients from 7 centers were enrolled. 54% were female, 82% were white, mean age was 34.5 (SD±14) years, mean age of onset was 24.6 (SD±13.7) years, and mean disease duration (DD) was 10 (SD±7.3) years. The mean BCVA was 53.4 letters (SD±17.9). The mean mMS was 12.2 (SD±4.6) dB and the mean sMS was 11.1 (SD±5.0) dB with the one-log unit filter. mMS and sMS were highly correlated with Pearson correlation coefficient of 0.81 (p<0.0001). The mean mPRL was 4.9 (SD±3.9) degrees and the mean sPRL was 5.34 (SD±3.8) degrees. mPRL and sPRL were highly correlated with correlation coefficient of 0.77 (p<0.0001). The mean 2SD mBCEA was 26.8 (SD±34.7) deg2 and the mean sBCEA was 30.4 (SD±31) deg2. mBCEA and sBCEA were correlated with correlation coefficients of 0.68 (p<0.0001).
In our cohort of patients with STGD1, photopic and scotopic macular performance were highly correlated when assessed using mean macular sensitivity in the perifoveal area. There were no significant differences in the distance of PRL from fovea or the 2SD BCEA of fixation.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.
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