TY - JOUR
T1 - Longitudinal Changes of Fixation Location and Stability Within 12 Months in Stargardt Disease
T2 - ProgStar Report No. 12
AU - ProgStar Study Group
AU - Schönbach, Etienne M.
AU - Strauss, Rupert W.
AU - Kong, Xiangrong
AU - Muñoz, Beatriz
AU - Ibrahim, Mohamed A.
AU - Sunness, Janet S.
AU - Birch, David G.
AU - Hahn, Gesa Astrid
AU - Nasser, Fadi
AU - Zrenner, Eberhart
AU - Sadda, Srini Vas R.
AU - West, Sheila K.
AU - Scholl, Hendrik P.N.
AU - Scholl, Hendrik P.N.
AU - Strauss, Rupert W.
AU - Wolfson, Yulia
AU - Bittencourt, Millena
AU - Shah, Syed Mahmood
AU - Ahmed, Mohamed
AU - Schönbach, Etienne
AU - Fujinami, Kaoru
AU - Traboulsi, Elias
AU - Ehlers, Justis
AU - Marino, Meghan
AU - Crowe, Susan
AU - Briggs, Rachael
AU - Borer, Angela
AU - Pinter, Anne
AU - Fecko, Tami
AU - Burgnoni, Nikki
AU - Sunness, Janet S.
AU - Applegate, Carol
AU - Russell, Leslie
AU - Michaelides, Michel
AU - Esposti, Simona Degli
AU - Moore, Anthony
AU - Webster, Andrew
AU - Connor, Sophie
AU - Barnfield, Jade
AU - Salchi, Zaid
AU - Alfageme, Clara
AU - McCudden, Victoria
AU - Pefkianaki, Maria
AU - Aboshiha, Jonathan
AU - Liew, Gerald
AU - Holder, Graham
AU - Robson, Anthony
AU - King, Alexa
AU - Wojciechowski, Robert
AU - Ervin, Ann Margret
N1 - Publisher Copyright:
© 2018 Elsevier Inc.
PY - 2018/9
Y1 - 2018/9
N2 - Purpose: To investigate the natural history of Stargardt disease (STGD1) using fixation location and fixation stability. Design: Multicenter, international, prospective cohort study. Methods: Fixation testing was performed using the Nidek MP-1 microperimeter as part of the prospective, multicenter, natural history study on the Progression of Stargardt disease (ProgStar). A total of 238 patients with ABCA4-related STGD1 were enrolled at baseline (bilateral enrollment in 86.6%) and underwent repeat testing at months 6 and 12. Results: Outcome measures included the distance of the preferred retinal locus from the fovea (PRL) and the bivariate contour ellipse area (BCEA). After 12 months of follow-up, the change in the eccentricity of the PRL from the anatomic fovea was −0.0014 degrees (95% confidence interval [CI], −0.27 degrees, 0.27 degrees; P =.99). The deterioration in the stability of fixation as expressed by a larger BCEA encompassing 1 standard deviation of all fixation points was 1.21 degrees squared (deg 2 ) (95% CI, −1.23 deg 2 , 3.65 deg 2 ; P =.33). Eyes with increases and decreases in PRL eccentricity and/or BCEA values were observed. Conclusions: Our observations point to the complexity of fixation parameters. The association of increasingly eccentric and unstable fixation with longer disease duration that is typically found in cross-sectional studies may be countered within individual patients by poorly understood processes like neuronal adaptation. Nevertheless, fixation parameters may serve as useful secondary outcome parameters in selected cases and for counseling patients to explain changes to their visual functionality.
AB - Purpose: To investigate the natural history of Stargardt disease (STGD1) using fixation location and fixation stability. Design: Multicenter, international, prospective cohort study. Methods: Fixation testing was performed using the Nidek MP-1 microperimeter as part of the prospective, multicenter, natural history study on the Progression of Stargardt disease (ProgStar). A total of 238 patients with ABCA4-related STGD1 were enrolled at baseline (bilateral enrollment in 86.6%) and underwent repeat testing at months 6 and 12. Results: Outcome measures included the distance of the preferred retinal locus from the fovea (PRL) and the bivariate contour ellipse area (BCEA). After 12 months of follow-up, the change in the eccentricity of the PRL from the anatomic fovea was −0.0014 degrees (95% confidence interval [CI], −0.27 degrees, 0.27 degrees; P =.99). The deterioration in the stability of fixation as expressed by a larger BCEA encompassing 1 standard deviation of all fixation points was 1.21 degrees squared (deg 2 ) (95% CI, −1.23 deg 2 , 3.65 deg 2 ; P =.33). Eyes with increases and decreases in PRL eccentricity and/or BCEA values were observed. Conclusions: Our observations point to the complexity of fixation parameters. The association of increasingly eccentric and unstable fixation with longer disease duration that is typically found in cross-sectional studies may be countered within individual patients by poorly understood processes like neuronal adaptation. Nevertheless, fixation parameters may serve as useful secondary outcome parameters in selected cases and for counseling patients to explain changes to their visual functionality.
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U2 - 10.1016/j.ajo.2018.06.003
DO - 10.1016/j.ajo.2018.06.003
M3 - Article
C2 - 29890160
AN - SCOPUS:85049539585
SN - 0002-9394
VL - 193
SP - 54
EP - 61
JO - American journal of ophthalmology
JF - American journal of ophthalmology
ER -