North Carolina macular dystrophy (NCMD) is an inherited eye disorder that affects the development of the macula, the small, but important part of the eye located in the center of the retina. The macula allows a person to see fine details and do tasks that require central vision, such as reading and driving. It is also important for seeing colors. The severity of changes in the development of the macula varies, causing some people to have little or no vision loss, while others may have severe vision loss. NCMD is considered non-progressive, which means most researchers believe the vision loss does not change after birth. Others believe it may progress slowly through age twelve. However, vision loss may increase if complications develop, such as new, abnormal blood vessels growing under the retina (choroidal neovascularization).

NCMD is caused by changes (mutations) in a region of chromosome 6 (MCDR1 locus), as well as in a region of chromosome 5 (MCDR3 locus). Although there is no cure for NCMD, treatment may include low vision aids such as glasses with high powered lenses, large print reading material, and computer software that can turn text into speech.

• Grade 1: Yellow-white spots or lesions (drusen) on the macula (usually causing no vision loss or mild blurriness of fine details)
• Grade 2: Clustered and joined yellow-white spots (drusen) on the macula with or without changes within the retinal pigment epithelium (RPE) and formation of scar tissue. The RPE is the colored part of the retina, and although it is not involved in vision, it helps the retinal cells involved in vision get the nourishment needed to stay healthy.
• Grade 3: Thin outer, white coat of the eye (staphyloma) or loss of macular tissue (coloboma) with damage and loss of tissue in the layer of nerves and blood vessels under the retina (choroid) and retina (chorioretinal atrophy). Vision loss is usually more severe with these findings.