June 2021
Volume 62, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2021
Changes of Fixation Stability and Fixation Location over 24 Months in Stargardt Disease: the ProgStar Study
Etienne Schönbach; Rupert Strauss; Marco Cattaneo; David G Birch; Artur V Cideciyan; Janet S Sunness; Kaoru Fujinami; Eberhart Zrenner; Srinivas R Sadda; Hendrik P Scholl
Author Affiliations & Notes
  • Etienne Schönbach
    Johns Hopkins Medicine Wilmer Eye Institute, Baltimore, Maryland, United States
    Case Western Reserve University, Cleveland, Ohio, United States
  • Rupert Strauss
    Department of Ophthalmology, Medical University of Graz, Austria, Graz, Austria
    Institute of Molecular and Clinical Ophthalmology Basel, Basel, Switzerland, Switzerland
  • Marco Cattaneo
    Institute of Molecular and Clinical Ophthalmology Basel, Basel, Switzerland, Switzerland
    Clinical Research, Universitat Basel, Basel, BS, Switzerland
  • David G Birch
    Retina Foundation of the Southwest, Dallas, Texas, United States
  • Artur V Cideciyan
    University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Janet S Sunness
    Greater Baltimore Medical Center, Baltimore, Maryland, United States
  • Kaoru Fujinami
    Moorfields Eye Hospital NHS Foundation Trust, London, London, United Kingdom
  • Eberhart Zrenner
    Eberhard Karls Universitat Tubingen, Tubingen, Baden-Württemberg, Germany
  • Srinivas R Sadda
    University of California Los Angeles, Los Angeles, California, United States
  • Hendrik P Scholl
    Institute for Molecular and Clinical Ophthalmology, BS, Switzerland
    Ophthalmology, Universitat Basel, Basel, BS, Switzerland
  • Footnotes
    Commercial Relationships   Etienne Schönbach, Foundation Fighting Blindness Clinical Research Institute (Columbia, MD) (F), German National Academy of Sciences Leopoldina, Grant Number LPDS 2015-14 (Halle, Germany) (F); Rupert Strauss, Austrian Science Fund (Vienna, Austria; FWF; Project number: J 3383-B23) (F), Foundation Fighting Blindness Clinical Research Institute (Columbia, MD) (F); Marco Cattaneo, None; David Birch, Foundation Fighting Blindness Clinical Research Institute (FFB CRI, Columbia, MD) (F); Artur Cideciyan, None; Janet Sunness, Acucela (Tokyo, Japan) (C), Apellis (Crestwood, KY) (C), Cell Cure’s OpRegen study (Jerusalem, Israel) (C), Genentech (San Francisco, CA) (C); Kaoru Fujinami, AGTC (Alachua, FL) (C), Astellas Pharma Inc (Tokyo, Japan) (C), Editas (Cambridge, MA) (C), Foundation Fighting Blindness Alan Laties Career Development Program (CF-CL-0416-0696-UCL), USA (F), Foundation Fighting Blindness Clinical Research Institute (Columbia, MD) (F), Grant-in-Aid for Scientists to support international collaborative studies of the Ministry of Education, Culture, Sports, Science and Technology, Japan (16KK01930002) (F), Grant-in-Aid for Young Scientists (A) of the Ministry of Education, Culture, Sports, Science and Technology, Japan (16H06269) (F), Great Britain Sasakawa Foundation Butterfield Awards, UK.. Dr. Birch is supported by NIH EY009076 (F), Health Labour Sciences Research Grant, The Ministry of Health Labour and Welfare, Japan (201711107A) (F), Iveric (New York, NY) (C), Janssen Pharmaceutica Japan (Tokyo, Japan) (C), Japan Agency for Medical Research and Development (18ek0109355h0001) (F), Kubota Pharmaceutical Holdings Co, Ltd, Acucela Inc (Tokyo, Japan) (C), Nacuity (Ft. Worth, TX) (C), National Hospital Organization Network Research Fund, Japan (H30-NHO-Sensory Organs-03) (F), Novartis Japan (Tokyo, Japan) (C), ProQR (Leiden, The Netherlands) (C), Sanofi Genzyme (Cambridge, MS) (C); Eberhart Zrenner, None; Srinivas Sadda, 4DMT (Emeryville, GA) (C), Allergan (Dublin, Republic of Ireland) (C), Amgen (Thosand Oaks, CA) (C), Carl Zeiss Meditec (F), Carl Zeiss Meditec (Dublin, CA) (S), CenterVue (Padova, Italy) (C), CenterVue (Padova, Italy) (S), Genentech (San Francisco, CA) (C), Heidelberg Engineering (Heidelberg, Germany) (C), Heidelberg Engineering (Heidelberg, Germany) (S), Merck (Kenilworth, NJ) (C), Nidek (Gamagori, Japan) (F), Nidek (Gamagori, Japan) (S), Novartis (Basel, Switzerland) (C), Optos (Dunfermline, UK), (C), Optos (Dunfermline, UK), (F), Regeneron (Tarrytown, New York) (C), Thrombogenics (Leuven, Belgium) (C), Topcon (Tokyo, Japan) (S); Hendrik Scholl, Astellas Institute for Regenerative Medicine (Chūō, Tokyo) (C), Belite Bio (San Diego, CA) (F), Boehringer Ingelheim Pharma GmbH & Co (Ingelheim am Rhein, Germany) (F), Dr. Scholl is co-director of the Institute of Molecular and Clinical Ophthalmology Basel (IOB) which is constituted as a non-profit foundation and receives funding from the University of Basel, the University Hospital Basel, Novartis, and the government of Basel-Stadt. These arrangements have been reviewed and approved by the University of Basel (Universitätsspital Basel, USB) in accordance with its conflict of interest policies. Grants at USB are negotiated and administered by the institution (USB) which receives them on its proper accounts. Individual investigators who participate in the sponsored project(s) are not directly compensated by the sponsor but may receive support from the institution for their project(s). (F), Foundation Fighting Blindness (Columbia, MD) (F), Gensight Biologics (Paris, France) (C), Gerson Lehrman Group (New York, NY) (F), Gyroscope Therapeutics Ltd. (San Francisco, CA) (C), Ionis Pharmaceuticals, Inc. (Carlsbad, CA) (C), IVERIC bio (Ophthotech Corporation) (New York, NY) (C), Janssen Research & Development, LLC (Johnson & Johnson) (Raritan, NJ) (C), Kinarus AG (Basel, Switzerland) (C), National Center of Competence in Research Molecular Systems Engineering (Switzerland) (F), Nordisk (FOCUS trial) (Bagsværd, Denmark) (F), Nordisk (FOCUS trial) (Bagsværd, Denmark) (C), Novartis Pharma AG (Basel, Switzerland) (C), Novartis Pharma AG (CORE) (Basel, Switzerland) (C), Pharma Research & Early Development (pRED) of F. Hoffmann-La Roche Ltd (Basel, Switzerland) (C), ReNeuron Group Plc/Ora Inc. (Pencoed, UK) (F), Swiss National Science Foundation (Bern, Switzerland) (F), Wellcome Trust (London, UK) (F)
  • Footnotes
    Support  Foundation Fighting Blindness, Leopoldina Acadamy Germany
Investigative Ophthalmology & Visual Science June 2021, Vol.62, 2167. doi:
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      Etienne Schönbach, Rupert Strauss, Marco Cattaneo, David G Birch, Artur V Cideciyan, Janet S Sunness, Kaoru Fujinami, Eberhart Zrenner, Srinivas R Sadda, Hendrik P Scholl; Changes of Fixation Stability and Fixation Location over 24 Months in Stargardt Disease: the ProgStar Study. Invest. Ophthalmol. Vis. Sci. 2021;62(8):2167.

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Abstract

Purpose : Stargardt Disease type 1 (STGD1) is the most common inherited macular degeneration. Several treatment approaches including pharmacotherapy, gene therapy, and stem cell therapy are in clinical trials. Fixation stability (FS) and location (FL) describe important dimensions of visual function but there is limited data on how they are affected by disease over time. Here, we present longitudinal changes of FS and FL over 24 months in STGD1 from the international, prospective, multicenter ProgStar study (NCT01977846).

Methods : Over 5 study visits every 6 months, patients with a molecular diagnosis of STGD1 completed separate fixation exams of roughly 30 seconds using the MP-1 Microperimeter (Nidek). FS was expressed through the 68.3 %-Bivariate Contour Ellipse Area (BCEA), FL through the distance of the barycenter of all fixation events from the fovea as determined on OCT images.

Results : At baseline, 239 patients (105 males, 44 %) and 459 eyes with a median age of 32 years (mean± SD, 33.8 ± 15.2 years) were included. The baseline mean log BCEA was 0.75 ± 1.29 log deg2 and the mean FL was 6.45 ± 4.52 deg. Although the mean log BCEA did not monotonically increase from visit to visit, the overall yearly increase in log BCEA was 0.124 log deg2 (99% CI, 0.063-0.185). It was not different during the first year compared to the second year. The increase was faster in eyes without flecks outside of the vascular arcades and depended on baseline BCEA. Looking at the subset of the better of two eyes resulted in a yearly increase in log BCEA of 0.138 log deg2 (99% CI, 0.045, 0.230) and confirmed the observed association with baseline BCEA.

Conclusions : Fixation parameters may serve as useful secondary endpoints to longitudinally describe visual dysfunction.

This is a 2021 ARVO Annual Meeting abstract.

 

These two fundus photos of left eyes show the Bivariate Contour Ellipse Area (BCEA): the inner circle encompasses 68.3% of all fixation events (represented through blue dots) and a larger area means that fixation is more unstable. The area of the inner circle is indicated in the top left in log deg2. The distance in degrees of the barycenter of all fixation events from the fovea in degrees (FL) is in the top right. The spaghetti plots show the evolution of the log BCEA in log deg2 (center) and the FL in degrees (right) over 24 months of follow-up of the whole cohort analyzed.
View OriginalDownload Slide

These two fundus photos of left eyes show the Bivariate Contour Ellipse Area (BCEA): the inner circle encompasses 68.3% of all fixation events (represented through blue dots) and a larger area means that fixation is more unstable. The area of the inner circle is indicated in the top left in log deg2. The distance in degrees of the barycenter of all fixation events from the fovea in degrees (FL) is in the top right. The spaghetti plots show the evolution of the log BCEA in log deg2 (center) and the FL in degrees (right) over 24 months of follow-up of the whole cohort analyzed.

These two fundus photos of left eyes show the Bivariate Contour Ellipse Area (BCEA): the inner circle encompasses 68.3% of all fixation events (represented through blue dots) and a larger area means that fixation is more unstable. The area of the inner circle is indicated in the top left in log deg2. The distance in degrees of the barycenter of all fixation events from the fovea in degrees (FL) is in the top right. The spaghetti plots show the evolution of the log BCEA in log deg2 (center) and the FL in degrees (right) over 24 months of follow-up of the whole cohort analyzed.

These two fundus photos of left eyes show the Bivariate Contour Ellipse Area (BCEA): the inner circle encompasses 68.3% of all fixation events (represented through blue dots) and a larger area means that fixation is more unstable. The area of the inner circle is indicated in the top left in log deg2. The distance in degrees of the barycenter of all fixation events from the fovea in degrees (FL) is in the top right. The spaghetti plots show the evolution of the log BCEA in log deg2 (center) and the FL in degrees (right) over 24 months of follow-up of the whole cohort analyzed.
View OriginalDownload Slide

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
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Copyright © 2024 Association for Research in Vision and Ophthalmology.
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