April 2011
Volume 52, Issue 14
Free
ARVO Annual Meeting Abstract  |   April 2011
Peripapillary Sparing And Macular Involvement In Stargardt Disease
Author Affiliations & Notes
  • Tomas R. Burke
    Ophthalmology, Columbia Coll Phys Surg,
    Columbia University, New York, New York
  • David W. Rhee
    Ophthalmology, Columbia Coll Phys Surg,
    Columbia University, New York, New York
  • Stephen H. Tsang
    Ophthalmology, Columbia Coll Phys Surg,
    Columbia Univ-Harkness Eye Inst, New York, New York
  • Theodore Smith
    Ophthalmology, Columbia Coll Phys Surg,
    Columbia University, New York, New York
  • Rando Allikmets
    Ophthalmology, Columbia Coll Phys Surg,
    Columbia University, New York, New York
  • Margaret A. Lazow
    Ophthalmology, Columbia Coll Phys Surg,
    Columbia University, New York, New York
  • Stanley Chang
    Dept of Psychology and Ophthalmology, Ophthalmology,
    Columbia Univ-Harkness Eye Inst, New York, New York
  • Donald C. Hood
    Dept of Psychology and Ophthalmology, Ophthalmology,
    Columbia University, New York, New York
  • Vivienne C. Greenstein
    Ophthalmology, Columbia Coll Phys Surg,
    Columbia University, New York, New York
  • Footnotes
    Commercial Relationships  Tomas R. Burke, None; David W. Rhee, None; Stephen H. Tsang, None; Theodore Smith, None; Rando Allikmets, None; Margaret A. Lazow, None; Stanley Chang, None; Donald C. Hood, Topcon, Inc (F, C); Vivienne C. Greenstein, None
  • Footnotes
    Support  The Eye Surgery Fund; NIH Grant EY02115, EY09076, EY015520, EY017404, EY13435, EY018213; Foundation Fightling Blindness; New York Community Trust and Research to Prevent Blindness
Investigative Ophthalmology & Visual Science April 2011, Vol.52, 5010. doi:
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      Tomas R. Burke, David W. Rhee, Stephen H. Tsang, Theodore Smith, Rando Allikmets, Margaret A. Lazow, Stanley Chang, Donald C. Hood, Vivienne C. Greenstein; Peripapillary Sparing And Macular Involvement In Stargardt Disease. Invest. Ophthalmol. Vis. Sci. 2011;52(14):5010.

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Abstract

Purpose: : To compare structure and function of the macula and peripapillary retina in Stargardt disease to see if there is relative sparing of the peripapillary area.

Methods: : Sixteen eyes of 16 patients with Stargardt disease and 12 eyes of 12 age-similar controls were studied. The ABCR 500 micro-array was used for genotyping. Patients were classified based on full-field electroretinogram (ERG) results.[1] Fundus autofluorescence (FAF) and spectral domain-optical coherence tomography (SD-OCT) (Heidelberg Spectralis HRA+OCT) horizontal line scans through the foveal and peripapillary areas were obtained. The thicknesses of the outer nuclear layer + outer plexiform layer (ONL+), outer segment (OS) and retinal pigment epithelium (RPE) through the foveal area and the peripapillary area from 1° to 4° temporal to the optic disc were measured using a computer aided manual segmentation technique.[2] Visual sensitivity in the central 10° and the peripapillary area was assessed with the Nidek MP-1 microperimeter. Total deviation plots were generated. Visual field sensitivities were compared to thicknesses of the underlying retinal layers.

Results: : All patients had at least 1 mutation detected in the ABCA4 gene. Thirteen patients had ERG group I and 3 had group II disease. For all patients SD-OCT line scans showed significant thinning of the ONL+ and significant thinning or absence of the OS layers. The ONL+ layer was significantly thinner even in regions with preserved inner segment/OS junctions. The RPE was significantly thinner in 10 patients. A greater correlation of visual sensitivity with OS (r2:0.66) compared to ONL+ thickness (r2:0.45) was demonstrated. At eccentricities >8° from the fovea the ONL+, OS and RPE thickness values were less affected in 10, 5 and 9 patients, respectively, at locations in the nasal compared with temporal retina. Of the 9 patients where the peripapillary area was studied from 1° to 4° from the optic disc, there was only 1 patient, with ERG group II disease, who showed significant thinning of the ONL+ and absence of the OS layer. This patient had abnormal peripapillary FAF.

Conclusions: : The SD-OCT and MP-1 results, consistent with previous reports using en-face imaging, showed relative sparing of the peripapillary area in Stargardt disease and provide quantitative markers of disease. 1. Lois et al.(2001) Arch Ophthalmol;119:359-69. 2. Hood et al.(2009) IOVS;50:2328-36.

Keywords: imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound) • retinal degenerations: hereditary • perimetry 
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