Abstract
Purpose::
To compare fundus autofluorescence of melanin elicited with near-infrared light (NIA, 787 nm) to fundus autofluorescence of lipofuscin (FAF, elicited with 488 nm) in patients with Stargardt disease.
Methods::
Eleven consecutive patients diagnosed with Stargardt disease based on clinical and electrophysiological (ISCEV ERG and/or mfERG) findings underwent FAF and NIA imaging using a confocal scanning laser ophthalmoscope (Heidelberg Retina Angiograph 2) with either a 300 or wide-angle field-of-view.
Results::
In 8/11 patients FAF and NIA alterations were limited to the posterior pole, whereas in the remaining 3 patients marked changes were present beyond the vessel arcades. FAF images showed areas of FAF loss in combination with spot-like areas of increased FAF in all patients. In contrast, areas of increased NIA were observed only in 1 patient. In all other patients, areas of reduced NIA were present. In 9/11 patients, areas of reduced NIA were more extensive compared to areas of reduced FAF indicating that these methods facilitate the observation of different aspects of retinal pigment epithelial (RPE) integrity. Peripapillary preservation of RPE was detected with FAF and NIA in 1 patient with progressed Stargardt disease.
Conclusions::
NIA imaging provides an additional tool for non-invasive in vivo visualization of RPE abnormalities. Different patterns of FAF and NIA indicate different pathophysiologic processes involving lipofuscin and melanin. Combined FAF and NIA imaging will provide further insight in the development of Stargardt disease, may serve as a method for the detection of early RPE alterations and could help to monitor future therapeutic interventions.
Keywords: retinal degenerations: hereditary • retinal pigment epithelium • macula/fovea