Deep Scleral Exposure: A Degenerative Outcome of End-Stage Stargardt Disease

Widespread scleral exposure is a clinical outcome in a subset of STGD1 following a long duration of disease progression (∼50 years). The blonde fundus in such cases may exhibit phenotypic overlap and shared therapeutic implications with other aggressive chorioretinal dystrophies such as end-stage choroideremia, gyrate atrophy, or RPE65-Leber congenital amaurosis.