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Familial Ectopia Lentis

In some families, the skeletal features of the Marfan syndrome are inherited together with lens dislocation. This is called familial ectopia lentis.

What is ectopia lentis?
Ectopia lentis is associated with the eye and skeletal features of the Marfan syndrome and can only be differentiated from "emerging" Marfan syndrome with ongoing clinical follow-up, including frequent echocardiograms.

What is lens dislocation?
The lens is the transparent structure inside the eye 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 a slit-lamp eye examination by an ophthalmologist after fully dilating the pupil.  Lens dislocation may lead to the more serious complication of retinal detachment.

What are the symptoms of lens dislocation?
Symptoms of lens dislocation 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

How is lens dislocation treated?
Lens dislocation may be treated by:

  • Blurred vision caused by lens dislocation can be corrected with glasses or contact lenses (special flat contacts may be required for proper correction).
  • The use of aphakic (refracting around the lens) glasses in combination with Atropine sulfate 1% ophthalmic drops to enlarge the pupil serve as an effective alternative to surgery.
  • Lens removal is not suggested until it is essential.