Ultrawidefield Autofluoresence in Abca4 Stargardt Disease

Abstract

Ultrawidefield fundus autofluorescence reveals peripheral changes in the majority of patients with Stargardt disease. Peripheral FAF changes may have implications for diagnosis, prognosis, and management of individual patients with Stargardt disease.

Peripheral Visual Fields in ABCA4 Stargardt Disease and Correlation With Disease Extent on Ultra-widefield Fundus Autofluorescence

“…Cases with extensive widespread atrophy with concomitant foveal sparing have also been described, though less frequently. 18,27,28 Foveal sparing was not evaluated in this cohort.…”

“…The images were categorized into the UWF-FAF types previously described: type I represented by central atrophy with or without flecks limited to the posterior pole (55-degree); type II represented by central atrophy with flecks only extending beyond the posterior pole; and type III represented by central atrophy, varying degrees of flecks, and the presence of atrophy in the periphery. 18 The latter was divided into three subtypes of peripheral atrophy patterns: type IIIa, with mild to moderate atrophy (punctate) extending beyond the equator; type IIIb, with severe atrophy extending to the equator; and type IIIc, with severe atrophy extending beyond to the equator. 18 …”

“…18 The latter was divided into three subtypes of peripheral atrophy patterns: type IIIa, with mild to moderate atrophy (punctate) extending beyond the equator; type IIIb, with severe atrophy extending to the equator; and type IIIc, with severe atrophy extending beyond to the equator. 18 …”

“…suggested that more widespread disease, represented by type III, was associated with cone and rod loss on electroretinogram (ERG); however, this study had limited functional data. 18 Others have shown that patients with more functional loss on ERG at their initial assessment tended to evolve with a faster rate of atrophy enlargement detected on FAF over time and faster central scotoma progression on kinetic perimetry. 14,20 Understanding the relationship between the spatial extent of FAF abnormalities and functional outcomes may allow us to use FAF to monitor disease progression and predict visual prognosis.…”

“…used ultra-widefield FAF (UWF-FAF) to describe peripheral changes that would not have been detected using 30 or 50-degree FAF and proposed a new classification based on this method: type I, consisting of lesions confined to the macula without peripheral changes; type II, consisting of central atrophy with peripheral flecks only; and type III, consisting of central atrophy, with varying degrees of flecks and atrophy in the periphery. 18 …”