What is ectopia lentis et Pupillae?

Background: Ectopia lentis et pupillae (ELeP) is a rare congenital inherited disorder characterized by lenticular and pupillary ectopia. Recent speculation on its pathogenesis is based on clinical observation and includes a neuroectodermal defect or persistence of fetal vasculature.

How does ectopia Lentis affect vision?

Vision problems are common in isolated ectopia lentis. Affected individuals often have nearsightedness (myopia) and can have an irregular curvature of the lens or a structure that covers the front of the eye (the cornea), which causes blurred vision (astigmatism).

Which is the most common cause of ectopia Lentis?

Systemic conditions commonly associated with ectopia lentis. 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.

How is ectopia Lentis diagnosis?

The ocular examination for ectopia lentis should consist of checking visual acuity, an external ocular exam, a slit lamp exam, retinoscopy and refraction, and a dilated fundus examination. Corneal diameter should be measured, as megalocornea is associated with Marfan syndrome.

How is ectopia Lentis treated?

Techniques include:

Lensectomy/vitrectomy with aphakic contact lens or spectacles.
Iris-fixated intraocular lens.
Scleral-sutured posterior intraocular lens.
Implantation of in-the-bag intraocular lens with a capsular tension ring.
Femtosecond laser technology can aid in the surgical management of subluxated cataracts.

What is familial ectopia Lentis?

Ectopia lentis et pupillae – ectopia lentis et pupillae is a rare congenital autosomal recessive (AR) disorder in which there is asymmetric eccentric pupils that are displaced in the opposite direction of a lens dislocation. The pupils are characteristically oval and ectopic, and often dilate poorly.

Can a lens move after cataract surgery?

What is intraocular lens dislocation? Intraocular lens (IOL) dislocation is a very rare condition that affects patients who have undergone cataract surgery and consists of the displacement of the implanted lens towards the vitreous cavity of the eye.

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.

Can you see with a dislocated lens?

The most common symptom of a dislocated intraocular lens implant is sudden, painless blurring of vision in one eye. The vision tends to be very blurry, but not blacked-out. Sometimes, the lens implant can be seen resting on the surface of the retina when laying on the back.

What is mild cerebellar ectopia?

Cerebellar tonsillar ectopia may be considered a mild form of a Chiari malformation, depending on the severity of visible symptoms. Frequent symptoms that are associated with mild cerebellar tonsillar ectopia include headaches, pain around the neck area, problems balancing and unclear vision.

What is the meaning of ‘ectopia’?

Definitions for ectopia ɛkˈtoʊ pi ə ec·topia. Here are all the possible meanings and translations of the word ectopia. The condition such that an organ or other body part has an abnormal position. Origin: [NL., fr. Gr. ‘ek out + place.] In medicine, an ectopia is a displacement or malposition of an organ or other body part.

What is ectopia lentis?

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 is ectopia lentis, isolated?

Isolated ectopia lentis is a condition that affects the eyes, specifically the positioning of the lens. The lens is a clear structure at the front of the eye that helps focus light. In people with isolated ectopia lentis, the lens in one or both eyes is not centrally positioned as it should be but is off-center (displaced).