iCliniq logo

Ask a Doctor Online Now

HomeHealth articlesectopia lentisWhat Is Ectopia Lentis?

Ectopia Lentis - Causes, Diagnosis and Management

Verified dataVerified data
0

4 min read

Share

Ectopia lentis is a condition characterized by displacement of the crystalline lens. Read to know more details.

Written by

Dr. Anjali

Medically reviewed by

Dr. Prashant Koranmath

Published At October 6, 2022
Reviewed AtFebruary 8, 2024

Introduction

Ectopia lentis is a rare condition that is a hereditary or acquired condition characterized by a change in the positioning of the lens. The lens is luxated or dislocated when it lies entirely outside the depression that lines the crystalline lens. It can be free-floating in the vitreous or present in the anterior chamber, or lies directly on the retina. The lens is considered subluxated when partially displaced but remains within the lens space. This disorder can occur because of trauma or may be associated with ocular or systemic disease, or it can be transmitted from one parent to child or from both parents to their child. It has a male preference, and this condition is bilateral. It can occur at any age, from birth to adulthood. Ectopia lentis is also called a subluxation of the lens.

Berryat described the first case in 1749, and Stellwag coined ectopia lentis in 1856. Ectopia lentis can lead to major complications within the eye, including clouding of the lens, called glaucoma, retinal damage, and blindness. The lens is a clear structure present in front of the eye. It can cause marked visual disturbance. In addition, it is associated with connective tissue disorders. There is a disruption of the zonal fibers of the lens. The treatment requires excellent skill because the lens is displaced significantly.

What Are the Symptoms Associated With Ectopia Lentis?

  • Vision problems are associated with ectopia lentis.

  • Affected individuals may present with nearsightedness.

  • The blurring of vision.

  • Double vision (diplopia).

  • Dizziness.

  • Cataract (clouding of the lens).

  • Tearing of the lining of the eye.

  • Glaucoma (a group of eye disorders causing vision loss by affecting the optic nerve).

  • Retinal detachment can occur.

What Are the Causes Associated With Ectopia Lentis?

It is a sign of severe systemic disease; it is important to determine the etiology of this condition for appropriate patient management and treatment.

  1. Trauma - Ectopia lentis can occur because of trauma. It can present at birth or manifest at any age.

  2. Systemic Disease - Many systemic diseases characterize this disorder.

  3. Ocular Pathology - Many ocular pathologies are associated with this condition.

  4. Mutations - FBN1 (fibrillin) gene and ADAMTSL4 (a disintegrin and metalloproteinase with thrombospondin motifs) gene can cause ectopia lentis.

  5. Marfan Syndrome - It is majorly associated with Marfan syndrome and is seen in 80 % of the patients. Lens dislocation in Marfan syndrome is usually bilateral.

  6. Syphilis - It is seen in patients suffering from syphilis.

  7. Intraocular Tumors - Tumors can also predispose to such conditions.

  8. Homocystinuria - It is called an inherited disorder where the body is unable to process proteins.

  9. Sulfite Oxidase Deficiency - This deficiency is a rare inborn error of sulfur-containing amino acids.

  10. Weill–Marchesani Syndrome - Inherited connective tissue disorder affecting the lens of the eyes of the patients can predispose them to such conditions.

How to Diagnose Ectopia Lentis?

The clinical features can investigate the diagnosis of ectopia lentils. However, there are not as such special investigations required to diagnose ectopia Lentis.

  • Visual Acuity - Visual acuity is checked using a standardized Snellen visual acuity chart. This condition results in severe vision loss; this will help in determining the ectopia lentils.

  • Refraction and Retinoscopy - It will help determine the myopic and astigmatic correction needed while prescribing glasses.

  • Genetic Analysis - Genetic analysis is carried out to rule out hereditary conditions.

  • Systemic Evaluation - Systemic evaluation is carried out to determine other conditions.

  • Amplitude-Scan Ultrasonography - It is also done to rule out ectopia lentis.

  • Bright-Scan Ultrasonography - It is done to rule out retinal detachment.

What Is the Management of Ectopia Lentis?

  1. Most cases are managed without surgical intervention.

  2. Counseling should be done for patients where the progression of dislocation is there, and for that, surgical management should be carried out.

  3. Appropriate spectacle or lens correction will help to provide stable visual acuity.

  4. Medical management includes refractive management and also the management of systemic diseases associated with this condition.

  5. Lensectomy or vitrectomy is a procedure where vitreous gel is partially or fully removed with optical rehabilitation with a contact lens and is done in more severe cases.

  6. Surgery is indicated in lens-induced glaucoma, lens-induced uveitis, visual acuity not improving with refractive correction, and retinal detachment.

Follow-Up

  • All patients of ectopia lentis require detailed counseling regarding the pathology, long-term visual outcome, and prognosis of the condition.

  • The importance of regular follow-up and post-operative medication use should be emphasized.

  • The patient should also understand the complications associated with ectopia lentis.

  • The importance of family screening, genetic counseling, and treatment of family members must be made clear to the patient.

  • The patient should receive information regarding the familial pedigree chart (a chart that diagrams the inheritance of traits) and the role of consanguineous marriages (marriages in which individuals are closely related and genetic disorders are twice seen in such families) in familial ectopia lentis.

What Is the Differential Diagnosis Associated With Ectopia Lentis?

  • Traumatic Cataract: Lens clouding may occur because of penetrating or blunt trauma to the eyes.

  • Traumatic Zonular Dialysis: Trauma of zonular fibers.

  • Pseudoexfoliation Syndrome: An age-related disorder that affects tissues of the eyes.

  • Intraocular lens Tilt: Seen in 5 to 10 % of the individuals after lens implantation.

  • Intraocular Lens subluxation and Intraocular Lens Dislocation: Displacement of the lens.

  • Lens Subluxation: Occurs because of complete disruption of zonal fibers

  • Lens Dislocation: The displacement of the lens because of the rupture of fibers.

  • Nucleus Drop: Occurs because of the inflammation in the eyes and increased pressure in the eyes.

  • Intraocular Lens Drop: Intraocular lens moves out from the normal position.

What Are the Complications Associated With Ectopia Lentis?

  • Glaucoma: Increases pressure in the eyes.

  • Corneal Injury: It is termed an injury to the cornea.

  • Amblyopia: Also called lazy eye, and is the reduced vision in one eye.

  • Corneal Endothelial Damage: Causes corneal swelling and inflammation.

  • Corneal Decompensation: Mechanical injury of the cornea during surgery.

  • Chronic Iridocyclitis: Where the iris (a pigmented structure that regulates the amount of eyes entering the retina) is inflamed.

  • Pupillary Block Glaucoma: When the aqueous flow through the iris is blocked.

  • Phacoanaphylactic Glaucoma: Rarest type of glaucoma.

  • Angle Recession Glaucoma: Traumatic blow to the eyes.

  • Subluxated Lens and Dislocated lens: Displacement of the lens.

  • Retinal Detachment: The primary cause is aging.

Conclusion

Ectopia lentis is classified as syndromic when it is present with syndromes. This condition affects the positioning of the lens. Patients suffering from ectopia lentis need to be educated on the importance of follow-up with a physician to rule out complications associated with this disorder. Complications include early severe myopia, retinal detachment, early cataracts, glaucoma, and amblyopia. It is inherited as a genetic condition, or it is acquired by injury or trauma or any other underlying eye disorders such as glaucoma or cataracts. Most patients perform well, and also, it depends on the grading of lens dislocation, the age of onset of this disorder, and secondary complications associated with it.

Source Article IclonSourcesSource Article Arrow
Dr. Prashant Koranmath
Dr. Prashant Koranmath

Ophthalmology (Eye Care)

Tags:

ectopia lentis
Community Banner Mobile
By subscribing, I agree to iCliniq's Terms & Privacy Policy.

Source Article ArrowMost popular articles

Ask your health query to a doctor online

Ophthalmology (Eye Care)

*guaranteed answer within 4 hours

Disclaimer: No content published on this website is intended to be a substitute for professional medical diagnosis, advice or treatment by a trained physician. Seek advice from your physician or other qualified healthcare providers with questions you may have regarding your symptoms and medical condition for a complete medical diagnosis. Do not delay or disregard seeking professional medical advice because of something you have read on this website. Read our Editorial Process to know how we create content for health articles and queries.

This website uses cookies to ensure you get the best experience on our website. iCliniq privacy policy