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Marfan Vision Complications

 
  • In about half of people with Marfan's syndrome, the lens in your eye moves or falls into an abnormal position (called a lens dislocation). It can be present at birth or it may develop when you are a child or a teenager.
  • Part of the lining of your eye (the retina) may tear or peel away from the back of your eye. This is called a retinal detachment.
  • Other possible eye problems include short-sightedness (myopia), clouding of the lens within your eye (a cataract) and an increase in pressure within your eye (glaucoma).
  • Eye problems in people with Marfan's syndrome may cause problems with vision if they are not treated early enough.

Eye Tests

           Eye exams that may be needed include:

  • Slit-lamp exam. This test checks for lens dislocation, cataracts or a detached retina. Your eyes will need to be completely dilated with drops for this exam.
  • Eye pressure test. To check for glaucoma, your eye doctor may measure the pressure inside your eyeball by touching it with a special tool. Numbing eyedrops are usually used before this test.




Eyes

Marfan syndrome can affect the eyes in many ways. For this reason, people with Marfan syndrome should see an ophthalmologist (a medical doctor who takes care of the eyes) to find out if they have any eye problems and learn how to care for their eyes.

Except for dislocated lenses, all of the eye problems in Marfan syndrome also occur in the general population. As a result, doctors do not always realize these eye problems are related to Marfan syndrome. It is important to know that even though these problems occur in the general population, they are much more common in people who have Marfan syndrome.

Some features of the Marfan eye can cause problems with seeing clearly (vision problems). These include:

Dislocated lensesAbout 6 in 10 people with Marfan syndrome have dislocated lenses in one or both eyes. This means the lens, located at the front of the eye, has slipped out of place because the connective tissue that holds the lens in place (called zonules) is weak. When this happens, the lens can slip in any direction—up, down, to the side, or back. It can slip a little or completely out of place, and anywhere in between. With the lens out of place, the eye can’t focus properly and vision is blurry.

Symptoms of lens dislocation depend on severity and may include mild to severe nearsightedness, blurred vision, and fluctuating vision. Lens dislocation can only be confirmed by an ophthalmologist using a slit-lamp eye examination after fully dilating the pupil.

For most people, dislocated lenses occur before age 20, although lenses can dislocate at any age. Babies and children can have dislocated lenses. Dislocated lenses are rare in the general population, so people with dislocated lenses should be tested for Marfan syndrome if there is not another known cause.

The Eye 


Some people with Marfan syndrome have problems with the eyes, such as:

  • Nearsightedness
  • Glaucoma (high pressure within the eye) at a young age
  • Cataracts (the eye’s lens becomes cloudy)
  • A shift in one or both lenses of the eye
  • A detached retina in the eye.
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Eyes (continued)

Retinal detachmentRetinal detachment is a separation of the light-sensitive membrane in the back of the eye (the retina) from its supporting layers. Symptoms that may indicate a retinal detachment include:

  • Flashing lights
  • New floaters
  • A gray curtain moving across your field of vision
These symptoms do not always mean a retinal detachment is present, but you should see your eye doctor immediately if any of these signs occur.

Head trauma can cause retinal detachment in anyone, and those who are highly myopic (near-sighted) are always at risk for retinal detachment. For people with Marfan syndrome, however, retinal detachment can happen spontaneously. See your doctor immediately if you see flashing lights, new floaters or a gray curtain moving across your field of vision.

Severe myopiaNearsightedness—when objects in the distance are blurred.

AstigmatismBlurred vision caused by an irregular curve of either the lens or the cornea. The cornea is the layer of tissue covering the very front of the eye.

AmblyopiaWhen one eye does not develop normal vision during early childhood. Sometimes called “lazy eye.”

StrabismusWhen the eyes point in different directions. Sometimes the eyes turn inward resulting in “crossed eyes,” but the eyes can also turn outward or downward.

GlaucomaA disease of the eye caused by increased pressure inside the eye. Untreated glaucoma can cause blindness.

Pre-senile cataractsClouding of the eye lens before age 60. Cataracts are common in older people who do not have Marfan syndrome, but people with Marfan syndrome can get cataracts at younger ages—even before age 40.

There are some other eye features that often occur in people who have Marfan syndrome. These features do not usually cause vision problems, but they can help doctors decide whether or not a person has Marfan syndrome. They include:

  • Flattened curve of the cornea. This feature may make it more difficult to fit contact lenses.
  • Larger than normal corneas.
  • Difficulty in completely dilating (opening) the pupils when the doctor does an eye exam.
  • Obvious sunken eyeballs (enophthalmos)

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