Retinal pigment epithelial detachment in ABCA4-associated Stargardt's disease

The authors describe bilateral and sequential pigment epithelial detachment (PED) formation in a 16-year-old girl with ABCA4-associated Stargardt's disease (STGD1). On routine examination, a new large PED was observed in the left eye with late leakage evident on fluorescein angiography suggestive of possible occult choroidal neovascularization. The lesion was therefore treated with bevacizumab, but with no involutional lesion response. Months later, a similar lesion appeared in the right eye. The authors believe this to be the first reported case of acute bilateral and sequential PED development in a patient with ABCA4-associated STGD1. Optical coherence tomography-guided morphological analysis of lesion development in ABCA4 disease with documentation of PED formation may suggest a plausible role for disruption at the level of Bruch's membrane in the pathophysiology of STGD1.