Longitudinal Changes of Fixation Location and Stability Within 12 Months in Stargardt Disease: ProgStar Report No. 12

ProgStar Study Group

Research output: Contribution to journalArticle

Abstract

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 (deg2) (95% CI, −1.23 deg2, 3.65 deg2; 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.

Original languageEnglish (US)
Pages (from-to)54-61
Number of pages8
JournalAmerican Journal of Ophthalmology
Volume193
DOIs
StatePublished - Sep 1 2018

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Disease Progression
Natural History
Confidence Intervals
Counseling
Cohort Studies
Cross-Sectional Studies
Outcome Assessment (Health Care)
Prospective Studies
Stargardt disease 1

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Longitudinal Changes of Fixation Location and Stability Within 12 Months in Stargardt Disease : ProgStar Report No. 12. / ProgStar Study Group.

In: American Journal of Ophthalmology, Vol. 193, 01.09.2018, p. 54-61.

Research output: Contribution to journalArticle

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title = "Longitudinal Changes of Fixation Location and Stability Within 12 Months in Stargardt Disease: ProgStar Report No. 12",
abstract = "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 (deg2) (95{\%} CI, −1.23 deg2, 3.65 deg2; 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.",
author = "{ProgStar Study Group} and Sch{\"o}nbach, {Etienne M.} and Strauss, {Rupert W.} and Xiangrong Kong and Beatriz Mu{\~n}oz and Ibrahim, {Mohamed A.} and Sunness, {Janet S.} and Birch, {David G.} and Hahn, {Gesa Astrid} and Fadi Nasser and Eberhart Zrenner and Sadda, {Srini Vas R.} and West, {Sheila K.} and Scholl, {Hendrik P.N.} and Scholl, {Hendrik P.N.} and Strauss, {Rupert W.} and Yulia Wolfson and Millena Bittencourt and Shah, {Syed Mahmood} and Mohamed Ahmed and Etienne Sch{\"o}nbach and Kaoru Fujinami and Elias Traboulsi and Justis Ehlers and Meghan Marino and Susan Crowe and Rachael Briggs and Angela Borer and Anne Pinter and Tami Fecko and Nikki Burgnoni and Sunness, {Janet S.} and Carol Applegate and Leslie Russell and Michel Michaelides and Esposti, {Simona Degli} and Anthony Moore and Andrew Webster and Sophie Connor and Jade Barnfield and Zaid Salchi and Clara Alfageme and Victoria McCudden and Maria Pefkianaki and Jonathan Aboshiha and Gerald Liew and Graham Holder and Anthony Robson and Alexa King and Robert Wojciechowski and Ervin, {Ann Margret}",
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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

PY - 2018/9/1

Y1 - 2018/9/1

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 (deg2) (95% CI, −1.23 deg2, 3.65 deg2; 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 (deg2) (95% CI, −1.23 deg2, 3.65 deg2; 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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DO - 10.1016/j.ajo.2018.06.003

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JO - American Journal of Ophthalmology

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