June 2013
Volume 54, Issue 15
ARVO Annual Meeting Abstract  |   June 2013
Hyperautofluorescent macular ring in a series of enhanced S-cone patients
Author Affiliations & Notes
  • Rony Gelman
    Ophthalmology, Doheny Eye Institute, Los Angeles, CA
    Ophthalmology, Columbia University, New York, NY
  • Jonathan Greenberg
    Ophthalmology, Columbia University, New York, NY
  • Tobias Duncker
    Ophthalmology, Columbia University, New York, NY
  • Lawrence Yannuzzi
    Vitreous Retina Macula Consultants of New York, New York, NY
    LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear, and Throat Institute, New York, NY
  • Stephen Tsang
    Ophthalmology, Columbia University, New York, NY
  • Footnotes
    Commercial Relationships Rony Gelman, None; Jonathan Greenberg, None; Tobias Duncker, None; Lawrence Yannuzzi, None; Stephen Tsang, None
  • Footnotes
    Support None
Investigative Ophthalmology & Visual Science June 2013, Vol.54, 2816. doi:
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      Rony Gelman, Jonathan Greenberg, Tobias Duncker, Lawrence Yannuzzi, Stephen Tsang; Hyperautofluorescent macular ring in a series of enhanced S-cone patients. Invest. Ophthalmol. Vis. Sci. 2013;54(15):2816.

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

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Purpose: To describe fundus autofluorescence (AF) and spectral-domain optical coherence tomography (SD-OCT) findings in a case series of enhanced S-cone syndrome (ESCS) patients, and to correlate AF and SD-OCT changes around the border of the hyperautofluorescent ring.

Methods: Observational case series of three patients with ESCS. Diagnoses were confirmed by full-field electroretinography (ERG). Patients were evaluated with AF imaging in combination with horizontal SD-OCT line scans through the fovea, at the posterior pole and at a temporal locus for optimal visualization of the border of the ring.

Results: On AF imaging, all eyes demonstrated a macula ring of high intensity autofluorescence. This demarcated the hypoautofluorescent periphery from the central macular area, which had relatively spared autofluorescence in 2 subjects, and mottled hyperautofluorescence in the third. The inner segment ellipsoid (ISe) line (previously the inner segment outer segment junction) became disrupted towards the border of the ring, where it was lost. The ISe line was also somewhat thinner and disorganized central to the ring and at the fovea in two patients, while the third showed a long zone of ISe disorganization central to the ring border.

Conclusions: We describe AF and corresponding SD-OCT features of the hyperautofluorescent macular ring in three patients with ESCS.

Keywords: 550 imaging/image analysis: clinical • 552 imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound) • 696 retinal degenerations: hereditary  

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