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Photo Essay |

Visualization of Lipofuscin Accumulation in Stargardt Macular Dystrophy by High-Resolution Fourier-Domain Optical Coherence Tomography

Christina Gerth, MD; Robert J. Zawadzki, PhD; Stacey S. Choi, PhD; John L. Keltner, MD; Susanna S. Park, MD, PhD; John S. Werner, PhD
Arch Ophthalmol. 2007;125(4):575. doi:10.1001/archopht.125.4.575.
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A 15-year-old patient was diagnosed with Stargardt macular dystrophy based on maculopathy (Figure 1) and abnormal multifocal electroretinographic responses. Retinal images were obtained with high-speed (up to 18 000 A-scans/s; 18 frames/s; 1000 lines/frame), high-resolution (axial ~ 4.5 μm; lateral 10-15 μm) Fourier-domain optical coherence tomography (FDOCT) (constructed at University of California, Davis).1,2 The FDOCT revealed well-demarcated oval to round “bumps” within the retinal pigment epithelial layer extending from subfoveal to 5° extrafoveal (Figure 2A). The outer nuclear layer was not detectable in this area when compared with an age-matched control (Figure 2B).

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Figure 1.

Fundus photograph and fluorescein angiography (FA) of the left eye. A, Perimacular yellowish flecks and blunting of the foveal reflex are seen (visual acuity: 20/150 with extrafoveal fixation). B, Early-phase FA shows a “dark choroid,” a sign of dye blockade due to lipofuscin.

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Figure 2.

Horizontal Fourier-domain optical coherence tomographic scans through the macula from the patient's left eye (A) and an age-matched control (B). A, Large arrowheads denote well-demarcated oval areas within the retinal pigment epithelium, the largest measuring 0.35 × 0.18 mm (small horizontal arrows). The outer nuclear layer is missing within the central scan area when compared with the control.

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