AIM: To analyze clinical features of Stargardt disease by fundus autofluorescence(FAF)and observe microstructural changes of the retina by frequency-domain optical coherence tomography(Spectralis OCT)to explore the clinical value of combined FAF and OCT in diagnosis of Stargardt's disease.

METHODS:Non-interventional observation study. Confocal scanning laser ophthalmoscope(Heidelberg, Germany Company)was applied on 9 patients(18 eyes)with Stargardt disease for blue autofluorescence(BL-FAF, excitation 488nm, filter >500nm), Spectralis OCT examination, fundus photography, mfERG examination and fundus fluorescein angiography(FFA)examination. Seven cases also received indocyanine green angiography(ICGA)inspection. The study analyzed distribution characteristics of Stargardt's disease under FAF examination and microstructural changes of the retina under Spectralis OCT.

RESULTS:The BL-FAF of the oval macular lesions presented low fluorescence and visible edge demonstrated varying thickness and strong fluorescence. OCT showed retinal pigment epithelial lesions(RPE), photoreceptors(PR)atrophy and neurosensory meager. Under FFA examination, scattered point-like fluorescence could be seen in the early stage, and macular atrophy tumor size appears consistent with the bull's-eye fluorescence could be seen in the late stage. The disease demonstrated choriocapillaris atrophy in the late stage under ICGA examination. New vessels could be seen in rare cases.

CONCLUSION: FAF with Spectralis OCT and mf-ERG is an effective non-invasive examination methods for diagnosis of Stargardt disease. Changes of its clinical characteristics might suggest degeneration of both RPE and PR and various pathological and physiological changes of lipofuscin.