Congenital stromal corneal dystrophy

Congenital stromal corneal dystrophy is an inherited eye disorder that primarily affects the clear layer that covers the front surface of the eye (cornea). Affected individuals have multiple corneal opacities, which are damaged areas that cause the cornea to appear cloudy or opaque rather than transparent. These corneal opacities form in the thickest layer of the cornea (stroma) and are typically present in both eyes (bilateral) from birth or soon thereafter. The corneas of affected individuals may also be thicker than normal and have an irregular surface. These corneal changes lead to a loss of visual acuity, which can impair a person’s ability to see clearly. Without treatment, visual acuity can worsen over time.

Congenital stromal corneal dystrophy is often associated with additional eye abnormalities, including eyes that do not look in the same direction (strabismus) and "lazy eye" (amblyopia). Less commonly, affected individuals may have involuntary eye movements (nystagmus) or increased sensitivity to light (photophobia).