Longitudinal Changes of Fixation Location and Stability Within 12 Months in Stargardt Disease: ProgStar Report No. 12.
- Schönbach EM Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, USA; Department of Ophthalmology and Visual Sciences, Case Western Reserve University, Cleveland, Ohio, USA.
- Strauss RW Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, USA; Department of Ophthalmology, Medical University, Graz, Austria; Moorfields Eye Hospital, London, UK; Department of Ophthalmology, Johannes Kepler University, Linz, Austria.
- Kong X Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, USA; Department of Biostatistics and Epidemiology, University of Massachusetts-Amherst, Amherst, Massachusetts, USA.
- Muñoz B Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, USA.
- Ibrahim MA Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, USA.
- Sunness JS Hoover Low Vision Rehabilitation Services, Greater Baltimore Medical Center, Baltimore, Maryland, USA.
- Birch DG Retina Foundation of the Southwest, Dallas, Texas, USA.
- Hahn GA Center for Ophthalmology, Eberhard Karls Universität, Tübingen, Germany.
- Nasser F Center for Ophthalmology, Eberhard Karls Universität, Tübingen, Germany.
- Zrenner E Center for Ophthalmology, Eberhard Karls Universität, Tübingen, Germany.
- Sadda SR Doheny Eye Institute, UCLA David Geffen School of Medicine, Los Angeles, California, USA.
- West SK Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, USA.
- Scholl HPN Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, USA; Institute of Molecular and Clinical Ophthalmology Basel (IOB), Basel, Switzerland; Department of Ophthalmology, University of Basel, Basel, Switzerland. Electronic address: Hendrik.Scholl@usb.ch.
- 2018-06-12
Published in:
- American journal of ophthalmology. - 2018
Adolescent
Adult
Aged
Child
Female
Fixation, Ocular
Follow-Up Studies
Humans
Macular Degeneration
Male
Middle Aged
Prospective Studies
Retina
Stargardt Disease
Visual Acuity
Visual Field Tests
Visual Fields
Young Adult
English
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.
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.
- Language
-
- English
- Open access status
- green
- Identifiers
-
- DOI 10.1016/j.ajo.2018.06.003
- PMID 29890160
- Persistent URL
- https://sonar.ch/global/documents/8804
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