Longitudinal Changes of Fixation Stability and Location Within 24 Months in Stargardt Disease: ProgStar Report No. 16

Affiliations

¹ From the Shiley Eye Institute and Jacobs Retina Center (E.M.S.), University of California, San Diego, La Jolla, California, USA; Institute of Molecular and Clinical Ophthalmology Basel (IOB) (E.M.S., M.E.G.V.C., H.P.N.S.), Basel, Switzerland.
² Moorfields Eye Hospital (R.W.S., K.F.), London, United Kingdom; Department of Ophthalmology (R.W.S.), Johannes Kepler University, Linz, Austria; Department of Ophthalmology (R.W.S.), Medical University, Graz, Austria.
³ Institute of Molecular and Clinical Ophthalmology Basel (IOB) (E.M.S., M.E.G.V.C., H.P.N.S.), Basel, Switzerland; Department of Clinical Research (M.E.G.V.C.), University of Basel, Switzerland.
⁴ Moorfields Eye Hospital (R.W.S., K.F.), London, United Kingdom; Laboratory of Visual Physiology, Division of Vision Research (K.F.), National Institute of Sensory Organs, National Hospital Organization Tokyo Medical Center, and Department of Ophthalmology (K.F.), Keio University, School of Medicine, Tokyo, Japan; University College London (UCL) Institute of Ophthalmology (K.F.), London, United Kingdom.
⁵ Retina Foundation of the Southwest (D.G.B.), Dallas, Texas, USA.
⁶ Scheie Eye Institute (A.V.C.), University of Pennsylvania, Philadelphia, Pennsylvania, USA.
⁷ Hoover Low Vision Rehabilitation Services (J.S.S.), Greater Baltimore Medical Center, Baltimore, Maryland, USA.
⁸ Universitäts-Augenklinik Tübingen (E.Z.), Eberhard Karls University of Tübingen, Tübingen, Germany.
⁹ Doheny Eye Institute (S.V.R.S.), and University of California, Los Angeles; Department of Ophthalmology, David Geffen School of Medicine (S.V.R.S.), Los Angeles, California, USA; Department of Ophthalmology (H.P.N.S.), University of Basel, Basel, Switzerland.
¹⁰ Institute of Molecular and Clinical Ophthalmology Basel (IOB) (E.M.S., M.E.G.V.C., H.P.N.S.), Basel, Switzerland; Department of Ophthalmology (H.P.N.S.), University of Basel, Basel, Switzerland. Electronic address: Hendrik.Scholl@usb.ch.

PMID: 34298008
DOI: 10.1016/j.ajo.2021.07.013

Free article

Multicenter Study

Longitudinal Changes of Fixation Stability and Location Within 24 Months in Stargardt Disease: ProgStar Report No. 16

Etienne M Schönbach et al. Am J Ophthalmol. 2022 Jan.

Free article

. 2022 Jan:233:78-89.

doi: 10.1016/j.ajo.2021.07.013. Epub 2021 Jul 21.

Affiliations

¹ From the Shiley Eye Institute and Jacobs Retina Center (E.M.S.), University of California, San Diego, La Jolla, California, USA; Institute of Molecular and Clinical Ophthalmology Basel (IOB) (E.M.S., M.E.G.V.C., H.P.N.S.), Basel, Switzerland.
² Moorfields Eye Hospital (R.W.S., K.F.), London, United Kingdom; Department of Ophthalmology (R.W.S.), Johannes Kepler University, Linz, Austria; Department of Ophthalmology (R.W.S.), Medical University, Graz, Austria.
³ Institute of Molecular and Clinical Ophthalmology Basel (IOB) (E.M.S., M.E.G.V.C., H.P.N.S.), Basel, Switzerland; Department of Clinical Research (M.E.G.V.C.), University of Basel, Switzerland.
⁴ Moorfields Eye Hospital (R.W.S., K.F.), London, United Kingdom; Laboratory of Visual Physiology, Division of Vision Research (K.F.), National Institute of Sensory Organs, National Hospital Organization Tokyo Medical Center, and Department of Ophthalmology (K.F.), Keio University, School of Medicine, Tokyo, Japan; University College London (UCL) Institute of Ophthalmology (K.F.), London, United Kingdom.
⁵ Retina Foundation of the Southwest (D.G.B.), Dallas, Texas, USA.
⁶ Scheie Eye Institute (A.V.C.), University of Pennsylvania, Philadelphia, Pennsylvania, USA.
⁷ Hoover Low Vision Rehabilitation Services (J.S.S.), Greater Baltimore Medical Center, Baltimore, Maryland, USA.
⁸ Universitäts-Augenklinik Tübingen (E.Z.), Eberhard Karls University of Tübingen, Tübingen, Germany.
⁹ Doheny Eye Institute (S.V.R.S.), and University of California, Los Angeles; Department of Ophthalmology, David Geffen School of Medicine (S.V.R.S.), Los Angeles, California, USA; Department of Ophthalmology (H.P.N.S.), University of Basel, Basel, Switzerland.
¹⁰ Institute of Molecular and Clinical Ophthalmology Basel (IOB) (E.M.S., M.E.G.V.C., H.P.N.S.), Basel, Switzerland; Department of Ophthalmology (H.P.N.S.), University of Basel, Basel, Switzerland. Electronic address: Hendrik.Scholl@usb.ch.

PMID: 34298008
DOI: 10.1016/j.ajo.2021.07.013

Abstract

Purpose: Stargardt disease type 1 (STGD1) is the most common macular dystrophy. The assessment of fixation describes an important dimension of visual function, but data on its progression over time are limited. We present longitudinal changes and investigate its usefulness for clinical trials.

Design: International, multicenter, prospective cohort study.

Methods: Included were 239 individuals with genetically confirmed STGD1 (one or more disease-causing ATP binding cassette subfamily A member 4 [ABCA4] variant). We determined the fixation stability (FS) using 1 SD of the bivariate contour ellipse area (1 SD-BCEA) and fixation location (FL) using the eccentricity of fixation from the fovea during five study visits every 6 months.

Results: At baseline, 239 patients (105 males [44%]) and 459 eyes, with a median age of 32 years, were included. The baseline mean logBCEA was 0.70 ± 1.41 log deg² and the mean FL was 6.25° ± 4.40°. Although the mean logBCEA did not monotonically increase from visit to visit, the overall yearly increase in the logBCEA was 0.124 log deg² (95% CI, 0.063-0.185 log deg²). The rate of change was not different between the 2 years but increased faster in eyes without flecks outside of the vascular arcades and depended on baseline logBCEA. FL did not change statistically significantly over time.

Conclusions: Fixation parameters are unlikely to be sensitive outcome measures for clinical trials in STGD1 but may provide useful ancillary information in selected cases to longitudinally describe and understand an eye's visual function.

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Longitudinal Changes of Fixation Stability and Location Within 24 Months in Stargardt Disease: ProgStar Report No. 16

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Longitudinal Changes of Fixation Stability and Location Within 24 Months in Stargardt Disease: ProgStar Report No. 16

Authors

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Abstract

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