Stargardt disease is a rare, genetic presentation that affects the macula in children. These patients experience vision loss due to an abnormal accumulation of lipofuscin.¹ However, the disease can be extremely heterogeneous and has variable macular and peripheral retinal involvement.¹ An Italian research team published a recent investigation attempting to correlate peripheral ultra-widefield (UWF) involvement with macular alterations, as assessed by structural OCT and OCT angiography (OCT-A), to identify different phenotypes of Stargardt disease.²

Using UWF autofluorescence, they found three distinct Stargardt phenotypes, each with variable OCT and OCT-A impairment. The team looked at the cases of 140 male patients: 70 with Stargardt and 70 controls. They performed a complete ophthalmologic assessment and multimodal imaging, including OCT, OCT-A, fundus autofluorescence and UWF imaging.²

They subdivided the patients with Stargardt disease into three types based on peripheral involvement and analyzed their OCT and OCT-A quantitative parameters. Type III made up 17% of the Stargardt group and had significantly worse visual function, OCT and OCT-A imaging parameters than types I and II.²