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North Carolina macular dystrophy (NCMD) is an autosomal dominantly inherited condition with 3 phenotypic grades.1 Grade 1 has yellow or white drusenlike deposits. Grade 2 features confluent drusen, pigmentary changes, retinal pigment epithelial (RPE) atrophy, or subretinal scarring. Grade 3 is described as a macular staphyloma, coloboma, or caldera with hyperpigmentation and subretinal fibrosis. Different grades and expressivity may be seen within one family, and the etiology is unknown.2
Schoenberger SD, Agarwal A. Intrachoroidal Cavitation in North Carolina Macular Dystrophy. JAMA Ophthalmol. 2013;131(8):1073-1076. doi:10.1001/jamaophthalmol.2013.1598