Figure 2.

Clinical features of ABCA4-associated retinopathies. A, D, G, and H: Fundus photography. B and E: Optical coherence tomography (OCT). C, F, and I: Electroretinograms (ERGs). A, B, and C: The ophthalmologic examination of patient H147 showed typical features of STDG. Atrophy of photoreceptors and RPE in the macula is shown on fundus photography and OCT. D, E, and F: Patient H145 showed CRD with macular degeneration and reduced cone response. The white arrows indicate choroidal excavation and defect of the Bruch’s membrane. G and H: Fundus examination of patient H91 at 10 and 19 years of age, respectively. I: Both the cone and the rod responses on the ERG at the age of 10 years were strikingly decreased, suggesting typical features of RP.