What causes ectopia lentis?
Ectopia lentis is caused by trauma, pseudoexfoliation syndrome, syphilis, Marfan’s syndrome, Weill–Marchesani syndrome, Stickler’s syndrome, Ehlers–Danlos syndrome, homocystinuria, hyperlysinemia, sulfite oxidase deficiency, aniridia, congenital glaucoma, megalocornea, hereditary ectopia lentis, and ectopia lentis et …
What causes ectopia lentis in Marfan syndrome?
It is thought that the increased incidence of ectopia lentis with Marfan syndrome is due to altered fibrillin microfibrils leading to incompetent zonular fibers and structural abnormalities of the lens capsule.
What does ectopia lentis mean?
Ectopia lentis is defined as displacement or malposition of the crystalline lens of the eye. [1, 2, 3] Berryat described the first reported case of lens dislocation in 1749, and Stellwag subsequently coined the term ectopia lentis in 1856 (describing a patient with congenital lens dislocation).
What causes bilateral lens dislocation?
Lens dislocation could be a result of a high-energy trauma, hereditary ocular diseases, or parts of systemic diseases, such as Marfan syndrome and homocystinuria .
Can an optometrist diagnose ectopia lentis?
There is a dominant and recessive form of ectopia lentis. An ophthalmologist can often distinguish between dominant and recessive ectopia lentis based upon family history and physical findings. However, gene testing is available for both dominant and recessive ectopia lentis syndrome.
What causes Marfan syndrome?
Marfan syndrome is caused by a mutation in a gene called FBN1. The mutation limits the body’s ability to make proteins needed to build connective tissue. One in four people with Marfan syndrome develops the condition for unknown reasons. A person with Marfan syndrome has a 1 in 2 chance of passing it on to their child.
How do you diagnose Marfan syndrome?
If your doctor suspects Marfan syndrome, one of the first tests he or she may recommend is an echocardiogram. This test uses sound waves to capture real-time images of your heart in motion. It checks the condition of your heart valves and the size of your aorta.
What is the difference between subluxation and dislocation of lens?
In lens subluxation, zonular filaments are broken, and the lens is no longer held securely in place but remains in the pupillary aperture. Lens dislocation occurs following complete disruption of the zonular filaments and displacement of the lens from the pupil. Trauma is the leading cause of lens dislocation.
What is ectopia lentis?
Ectopia lentis is defined as displacement or malposition of the crystalline lens of the eye. Berryat described the first reported case of lens dislocation in 1749, and Stellwag subsequently coined the term ectopia lentis in 1856 (describing a patient with congenital lens dislocation).
What are the possible complications of ectopia lentis?
Ectopia lentis is potentially visually debilitating. Visual acuity varies with the degree of malpositioning of the lens. Amblyopia is a common cause of decreased vision in congenital ectopia lentis and is preventable and treatable.
What is the prevalence of Marfan syndrome in the US?
Marfan syndrome is the most frequent cause of heritable ectopia lentis. The syndrome is transmitted as an autosomal dominant trait with variable expressivity and has a prevalence of approximately 5 per 100,000.