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