North Carolina macular dystrophy: Clinicopathologic correlation

PURPOSE: To describe the clinical and histopathologic findings of a 72-year -old female with North Carolina macular dystrophy. METHODS: Observational case report with histopathologic correlation. Clinic al examination includes slit-lamp biomicroscopy, indirect ophthalmoscopy, c olor fundus photography, and focal electroretinography. Histopathologic exa mination of the enucleated left eye performed with light microscopy. RESULTS: Light microscopy demonstrated a discrete macular lesion characteri zed by focal absence of photoreceptor cells and retinal pigment epithelium with attenuation of the Bruch membrane and focal atrophy of the chorio, cap illaris. Adjacent to the macular lesion, some lipofus, cin was identified i n the retinal pigment epithelium. CONCLUSION: North Carolina macular dystrophy has both clinical and microsco pic appearances of a well, demarcated lesion confined to the macula, which involves the retina, pigment epithelium, and choriocapillaris.