June 2021
Volume 62, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2021
A Prospective Longitudinal Four-Year Analysis of Ellipsoid Zone Loss in Stargardt Disease
Author Affiliations & Notes
  • Michelle A. Chen
    National Eye Institute, Bethesda, Maryland, United States
    Howard University College of Medicine, Washington, District of Columbia, United States
  • Wadih M Zein
    National Eye Institute, Bethesda, Maryland, United States
  • Laryssa Huryn
    National Eye Institute, Bethesda, Maryland, United States
  • Robert B Hufnagel
    National Eye Institute, Bethesda, Maryland, United States
  • Amy Turriff
    National Eye Institute, Bethesda, Maryland, United States
  • Catherine A Cukras
    National Eye Institute, Bethesda, Maryland, United States
  • Brian Patrick Brooks
    National Eye Institute, Bethesda, Maryland, United States
  • Brett G Jeffrey
    National Eye Institute, Bethesda, Maryland, United States
  • Footnotes
    Commercial Relationships   Michelle Chen, None; Wadih Zein, None; Laryssa Huryn, None; Robert Hufnagel, None; Amy Turriff, None; Catherine Cukras, None; Brian Brooks, None; Brett Jeffrey, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2021, Vol.62, 1532. doi:
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      Michelle A. Chen, Wadih M Zein, Laryssa Huryn, Robert B Hufnagel, Amy Turriff, Catherine A Cukras, Brian Patrick Brooks, Brett G Jeffrey; A Prospective Longitudinal Four-Year Analysis of Ellipsoid Zone Loss in Stargardt Disease. Invest. Ophthalmol. Vis. Sci. 2021;62(8):1532.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : To quantify the rate of ellipsoid zone (EZ) loss over a four-year period in patients with Stargardt disease

Methods : 16 patients with Stargardt disease with two or more variants in in ABCA4 were followed prospectively at baseline, 6, 12, 24, 36, and 48 months with Spectral-domain OCT (SD-OCT) volume scans spanning a 30o x 15o rectangle centered on the fovea (37 B scans) The length of central discontinuity of the inner segment/outer segment band (EZ band) was measured manually on the Heidelberg for each SD-OCT B-scan and area of EZ band loss (mm2) was calculated using the Riemann sum. Using a radius linear model, the square-root transformed area of EZ band loss (mm) was calculated.

Results : Twenty-five eyes from sixteen patients were included in this 4-year prospective longitudinal study. The median age of patients at the baseline visit was 34 years (range 12-63) with median best-corrected visual acuity of 20/125 (range 20/32-20/200). The mean (±SD) EZ band disruption at the baseline visit was 2.33± 0.63 mm. The length of EZ disruption was highly correlated between right and left eyes (P<0.0001; R=0.97) and was correlated with age (P=0.025; R=0.56) but not visual acuity. The median rate of growth in EZ band disruption was 0.061 mm and ranged from minimal change (0.019 mm/yr) to high rates of growth (0.267mm/yr) but was linear for each eye measured. The rate of growth in EZ band loss was also highly correlated between eyes (P=0.0004; R=0.85) but was not correlated with age, visual acuity or size of EZ band disruption at baseline.

Conclusions : Measuring the rate of EZ disruption provides a direct structural measure that is related to loss of photoreceptors. While the rate of EZ loss varied between patients, all patients demonstrated linear growth over a four-year period. This information is highly relevant to clinical trial planning and sample size calculations. The high correlation in rate of loss EZ band loss between the two eyes suggest that one eye could serve as a control in therapeutic trials aimed at treating a single eye.

This is a 2021 ARVO Annual Meeting abstract.

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