Marfan syndrome

                                      

Marfan syndrome is a disorder of connective tissue, the tissue that strengthens the body's structures.
Disorders of connective tissue affect the skeletal system, cardiovascular system, eyes, and skin.
Eye care professionals should play a significant role in detecting Marfan syndrome. An eye care professional who suspects Marfan syndrome should urge the patient to see a medical geneticist and a cardiologist for a complete evaluation and a definitive diagnosis.
Several eye conditions seen in people with Marfan syndrome also occur in the general population. 
However, others, such as dislocated lenses, are rare in the general population but common in people with Marfan syndrome.

Early symptoms may indicate a retinal detachment:
Flashing lights
New floaters
A gray curtain moving across your field of vision
These symptoms do not always mean a retinal detachment is present.

One or more of the following conditions are common among persons with Marfan syndrome: high refractive error, flattened corneal curvature, dislocated lenses, early onset cataracts, glaucoma, retinal detachment and strabismus. 
Most people with Marfan syndrome are myopic (near-sighted) and have astigmatism (visual distortion). Approximately 65 percent of people with the disorder have dislocated lenses (ectopia lentis). The lens may be markedly off center or so subtly dislocated that an ophthalmologist might miss the dislocation without fully dilating the pupils.
A dislocated lens is often the first sign identified in a person with Marfan syndrome. While dislocated lenses may severely interfere with visual development and function, it is the accompanying cardiovascular manifestations of Marfan syndrome that may lead to severe morbidity. As a result, all people with lens dislocation should be evaluated further.
Lens dislocation is rarely, if ever, present at birth, and may be first noted at all ages, most commonly prior to 10 years of age.
People with Marfan syndrome should be taught to recognize the signs of retinal detachment and seek immediate consultation by an ophthalmologist..