Isolated ectopia lentis

Isolated ectopia lentis is a condition that affects the positioning of the lens in the eyes. The lens is a clear structure at the front of the eye that helps focus light. In people with isolated ectopia lentis, the lens in one or both eyes is out of place (ectopic) instead of centrally positioned. Isolated ectopia lentis is often present at birth but may become apparent during childhood. The lens may drift further out of place over time.

Vision problems are common in people with isolated ectopia lentis. These problems often include nearsightedness (myopia) and double vision (diplopia). The lens or the structure that covers the front of the eye (the cornea) can have an irregular curvature, which causes blurred vision (astigmatism). Affected individuals may also develop clouding of the lenses (cataracts) or increased pressure in the eyes (glaucoma) at an earlier age than other adults. A small number of people with isolated ectopia lentis experience tearing in the lining at the back of the eye (retinal detachment), which can lead to further vision problems and blindness.

In individuals with isolated ectopia lentis, each eye can be affected differently. In addition, the eye problems vary among affected individuals, even those within the same family.

Ectopia lentis is classified as isolated when it occurs without signs and symptoms that affect other body systems. Ectopia lentis can also be classified as syndromic when it is part of a syndrome that affects multiple parts of the body. Ectopia lentis is a common feature of genetic syndromes such as Marfan syndrome and Weill-Marchesani syndrome.

Ectopia lentis can also occur in individuals who experience injury or trauma to the head or eye. Traumatic ectopia lentis is not genetic, can occur at any age, and is the most common cause of ectopia lentis.