Ectopia Lentis Syndrome

Ectopia Lentis Syndrome is an inherited connective tissue disorder that shares some of the features of Marfan Syndrome, particularly with lens dislocation of the eyes, which can cause serious vision problems.

A person with features of Ectopia Lentis Syndrome should consult with a doctor who knows about connective tissue disorders. It is often the case that the doctor will be a medical geneticist. It is very important that an early and proper diagnosis be made.

A person may be diagnosed with Ectopia Lentis Syndrome if they exhibit these similar characteristics of Marfan Syndrome, but those with Ectopia Lentis Syndrome do not have heart and blood vessel issues that are prominent in those with Marfan Syndrome. However, in order to make a proper diagnosis, a person should undergo frequent echocardiograms, as this is often the only way to tell the difference in making an accurate diagnosis.

Another important issue is often present with the eyes. More specially, with regards to the lens. The lens is the transparent structure inside the eyes that focuses light rays onto the retina (the nerve layer that lines the back of the eye, senses light, and creates impulses that go through the optic nerve to the brain).

Lens dislocation is when the lens is a bit off-center to completely floating. It can only be confirmed by an eye doctor and a special test called a slit-lamp eye examination (only after fully dilating the pupils). Lens dislocation can lead to the more serious complication of retinal detachment (when the membrane in the back of the eye separates from its supporting layers).

Symptoms of lens dislocation may include:

– nearsightedness (myopia)
– astigmatism (blurred vision due to an irregularity in the curvature of the front surface of the eye, the cornea)
– fluctuating or blurred vision

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