Fundus photographs of affected patients with North Carolina macular dystrophy, showing fine, confluent drusen in the macula (grade I), a subretinal scar due to a prior choroidal neovascularization (Grade II), and a macular caldera (Grade III). In Grade I lesion, autofluorescence shows marked hyperautofluorescence of the bright drusen-like elements. SD-OCT shows a normal anatomy of the inner retina; the photoreceptor-retinal pigment epithelium complex appears normal, and the drusen-like lesions are not detectable. In Grade II lesion, SD-OCT shows increased reflectivity consistent with subretinal fibrosis or gliosis. In the Grade III lesion, fundus autofluorescence demonstrates central hypoautofluorescence, surrounded by normal appearing retina tissue and preserved foveal tissue. There is a hyperautofluorescent perimeter surrounding the edges of the lesion, possibly indicative of metabolic byproduct deposition. SD-OCT demonstrates a normal hyperreflective IS/OS photoreceptor junction with abrupt attenuation of outer retinal structures and RPE absence within the lesion.
