Infrared scanning laser ophthalmoscope imaging of the macula and its correlation with functional loss and structural changes in patients with stargardt disease

Abstract

Purpose: To correlate the degree of functional loss with structural changes in patients with Stargardt disease.

Methods: Eighteen eyes of 10 patients with Stargardt disease were studied. Scanning laser ophthalmoscope infrared images were compared with corresponding spectral-domain optical coherence tomography scans. Additionally, scanning laser ophthalmoscope microperimetry was performed, and results were superimposed on scanning laser ophthalmoscope infrared images and in selected cases on fundus autofluorescence images.

Results: Seventeen of 18 eyes showed a distinct hyporeflective foveal and/or perifoveal area with distinct borders on scanning laser ophthalmoscope infrared images, which was less evident on funduscopy and incompletely depicted in fundus autofluorescence images. This hyporeflective zone corresponded to areas of significantly elevated psychophysical thresholds on microperimetry testing, in addition to thinning of the retinal pigment epithelium and disorganization or loss of the photoreceptor cell inner segment-outer segment junction and external-limiting membrane on spectral-domain optical coherence tomography.

Conclusion: Scanning laser ophthalmoscope infrared fundus images are useful for depicting retinal structural changes in patients with Stargardt disease. A spectral-domain optical coherence tomography/scanning laser ophthalmoscope microperimetry device allows for a direct correlation of structural abnormalities with functional defects that will likely be applicable for the determination of retinal areas for potential improvement of retinal function in these patients during future clinical trials and for the monitoring of the diseases' natural history.

FIGURE 1

(A, B, C) Composite of a color fundus photograph (gray scale), and SLO infrared and FAF images from patient 5 with a geographic, atrophic-appearing macular lesion. (D) An outline of the atrophic macular lesion on SLO infrared imaging of patient 5, superimposed on the FAF image of this patient. (E) Microperimetry responses superimposed on the SLO infrared image of patient 5.

FIGURE 2

(A, B, C): Composite of a color fundus photograph (gray scale) and SLO infrared and FAF images from patient 1 with a bull's eye-appearing macular lesion. (D and E) Microperimetry responses of patient 1, superimposed on an SLO infrared image (D), and on an FAF image (E).

FIGURE 3

(A, B, C) Three-dimensional images composed of an SD-OCT scan and a corresponding SLO infrared image of patient 5 with a geographic atrophic macular lesion (A), and patient 1 with a bull's eye-appearing macular lesion (B), and corresponding image from a control subject (C) respectively. Brackets with arrowheads enclose the atrophic macular lesion and white arrowhead shows the disruption of the IS-OS junction of the photoreceptors and ELM from patient 5 (Figure 3A). The white arrow on Figure 3B shows the increased thickness and brightness of the IS-OS junction of the photoreceptors in the foveal region of patient 1.