Friday Fact: Ectopia Lentis

We view life through a lens, a transparent structure that is integral to our interpretation of the world around us. In Marfan syndrome this lens can become displaced.

The lens bends and focuses light rays on the retina, adjusting its power to produce a sharp image. It is critical to sight and is located behind the iris.

Ectopia Lentis (EL) is an important sign of Marfan syndrome so anyone found to have EL by an ophthalmologist should be referred for further review with a geneticist to consider a diagnosis of Marfan syndrome.

What is Ectopia Lentis?

Displacement of the crystalline lens from its normal position within the eye. Normally, the lens is held in position by thread-like fibres called ‘zonules’, these can be dysfunctional in Marfan syndrome.

A ‘subluxed’ lens is partially displaced but still in the pupillary area.

A ‘luxated’ or ‘dislocated’ lens is completely displaced from its normal position in the eye.

What are the possible symptoms?

Short-sightedness (myopia)

Blurred vision

Increased pressure in the eye (glaucoma)

Diagnosis

Eye examination by an ophthalmologist including a test of vision and a slit lamp examination (to look at the structures inside the eye)

Treatment

This will depend upon the degree of lens displacement but could include glasses/contact lenses or surgery to remove and replace the dislocated lens.