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The Progression of Stargardt Disease Using Volumetric Hill of Vision Analyses Over 24 Months: ProgStar Report No.15.
Schönbach, Etienne M; Janeschitz-Kriegl, Lucas; Strauss, Rupert W; Cattaneo, Marco E G V; Fujinami, Kaoru; Birch, David G; Cideciyan, Artur V; Sunness, Janet S; Weleber, Richard G; Ip, Michael S; Sadda, SriniVas R; Scholl, Hendrik P N.
Affiliation
  • Schönbach EM; From the Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, USA; University Hospitals Eye Institute, Case Western Reserve University, Cleveland, Ohio, USA.
  • Janeschitz-Kriegl L; Institute of Molecular and Clinical Ophthalmology Basel (IOB), Basel, Switzerland; Departments of Ophthalmology, University of Basel, Basel, Switzerland.
  • Strauss RW; From the Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, USA; Moorfields Eye Hospital, London, United Kingdom; Department of Ophthalmology, Johannes Kepler University, Linz, Austria; Department of Ophthalmology, Medical University, Graz, Austria; UCL Institute of Ophthalmology,
  • Cattaneo MEGV; Clinical Research, University of Basel, Basel, Switzerland.
  • Fujinami K; Moorfields Eye Hospital, London, United Kingdom; UCL Institute of Ophthalmology, London, United Kingdom; Laboratory of Visual Physiology, Division of Vision Research, National Institute of Sensory Organs, National Hospital Organization Tokyo Medical Center, Tokyo, Japan; Department of Ophthalmology,
  • Birch DG; Retina Foundation of the Southwest, Dallas, Texas, USA.
  • Cideciyan AV; Scheie Eye Institute, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
  • Sunness JS; Hoover Low Vision Rehabilitation Services, Greater Baltimore Medical Center, Baltimore, Maryland, USA.
  • Weleber RG; Casey Eye Institute, Oregon Retinal Degeneration Center, Oregon Health & Science University, Portland, Oregon, USA.
  • Ip MS; Doheny Eye Institute, Los Angeles, California, USA; UCLA David Geffen School of Medicine, Los Angeles, California, USA.
  • Sadda SR; Doheny Eye Institute, Los Angeles, California, USA; UCLA David Geffen School of Medicine, Los Angeles, California, USA.
  • Scholl HPN; Institute of Molecular and Clinical Ophthalmology Basel (IOB), Basel, Switzerland; Departments of Ophthalmology, University of Basel, Basel, Switzerland. Electronic address: Hendrik.Scholl@iob.ch.
Am J Ophthalmol ; 230: 123-133, 2021 10.
Article in En | MEDLINE | ID: mdl-33951446
ABSTRACT

PURPOSE:

To report the yearly rate of change in macular function in patients with Stargardt disease type 1 (STGD1) over 24 months and to establish a new volumetric visual function index for use in clinical trials investigating the efficacy on retinal sensitivity.

METHODS:

Design:

International, multicenter, prospective cohort study with 5 study visits every 6 months over 24 months.

PARTICIPANTS:

A total of 233 individuals with genetically confirmed STGD1 (≥1 disease-causing ABCA4 variant). MAIN OUTCOME

MEASURES:

The total volume (VTOT) beneath the sensitivity surface of a 3-D model of the hill of vision and mean sensitivity (MS) derived from mesopic microperimetry performed with a white stimulus. Changes of VTOT over time and its correlation with the ABCA4 genotype and baseline features.

RESULTS:

At baseline, 440 eyes (233 patients) with a mean (SD) age of 33.7 (15.0) years, mean (SD) visual acuity of 46.08 (16.03) ETDRS letters were analyzed with an average VTOT of 0.91 decibel-steradian (dB-sr) and an MS of 10.73 dB. The overall mean rate of decrease in sensitivity [95% confidence interval] was 0.077 [0.064, 0.090] dB-sr/y for VTOT and 0.87 [0.72, 1.02] dB/year for MS. The progression rate of VTOT depended on baseline visual function (0.029 dB-sr/year for low and 0.120 dB-sr/year for high baseline VTOT; P < .001) and exhibited a difference in the first vs second year of follow-up (0.065 dB-sr/year vs 0.089 dB-sr/year, respectively; P < .001). The absence of pigmentary abnormalities of the retinal pigment epithelium at baseline was found to be associated with a faster progression rate (P < .001), whereas a significant association with the genotype was not detected (P = .7).

CONCLUSION:

In STGD1, both microperimetric outcomes demonstrate statistically significant and clinically meaningful changes after relatively short follow-up periods. Volumetric modeling may be useful in future interventional clinical trials that aim to improve retinal sensitivity or to slow down its decline and for structure-function correlations.
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Retina / Visual Fields Type of study: Clinical_trials / Observational_studies Limits: Adult / Humans Language: En Journal: Am J Ophthalmol Year: 2021 Document type: Article Affiliation country:
Fulltext
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Retina / Visual Fields Type of study: Clinical_trials / Observational_studies Limits: Adult / Humans Language: En Journal: Am J Ophthalmol Year: 2021 Document type: Article Affiliation country: