Table 9.
Summary of the typical findings identified on multimodal imaging in Stargardt disease.
Feature | Colour fundus imaging | Fluorescence angiography | ICGA | SW-AF imaging | NIR-AF imaging | Infrared image | Optical coherence tomography | Optical coherence tomography angiography | Adaptive optics |
---|---|---|---|---|---|---|---|---|---|
Flecks | Yellow-white flecks | Usually hypofluorescent flecks but can sometimes be hyperfluorescent | Hypofluorescent and do not always correspond to flecks seen in colour photographs | Typically hyperfluorescent and resorbed flecks can be hypofluorescent | Typically hypofluorescent | Typically, hyperreflective, while resorbing flecks are hyporeflective | hyper-reflective deposits | Hyperreflective deposits | Highly reflective structures that are not arranged in a contigious mosaic |
Atrophy | ‘Beaten bronze’ appearance. The atrophic area that can sometimes be associated with visible choroid and pigmentary deposition | Hyperfluorescent | Dark region surrounded by a ring of increased signal | Decreased AF signal | Decreased AF signal | Hyperreflective region that is usually surrounded by a darker less reflective ring | Disruption/ loss of the RPE layer (difficult to discern) and loss of the EZ | Disruption/loss of the RPE layer (difficult to discern) and loss of the EZ | Brightly oversaturated |
Other features identified specifically by modality | UWF imaging can show peripheral pigmented lesions in some patients | Dark choroid sign | Reticular pattern of decreased fluorescence | Flecks and atrophy usually more advanced than those seen in SW-AF | Thickening of the ELM in early disease | Absence of choriocapillaris in macular areas of EZ loss | Increased cone spacing Enlarged photoreceptors Increased cone: rod ratio |
AF, autofluorescence; ELM, external limiting membrane; EZ, ellipsoid zone; ICGA, indocyanine green angiography; NIR-AF, near infrared-wavelength autofluorescence; RPE, retinal pigment epithelium; SW-AF, short-wavelength autofluorescence; UWF, ultra-widefield.