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Ectopia Lentis

  • Author: Charles W Eifrig, MD; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Mar 10, 2015
 

Background

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).

The lens is considered dislocated or luxated when it lies completely outside the lens patellar fossa, in the anterior chamber, free-floating in the vitreous, or directly on the retina. The lens is described as subluxed when it is partially displaced but contained within the lens space. In the absence of trauma, ectopia lentis should evoke suspicion for concomitant hereditary systemic disease or associated ocular disorders.[4, 5]

Ectopia lentis. Dislocated traumatic lens (catarac Ectopia lentis. Dislocated traumatic lens (cataract).
Ectopia lentis. Dislocated lens into the vitreous Ectopia lentis. Dislocated lens into the vitreous secondary to trauma.
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Pathophysiology

Disruption or dysfunction of the zonular fibers of the lens, regardless of cause (trauma or heritable condition), is the underlying pathophysiology of ectopia lentis. The degree of zonular impairment determines the degree of lens displacement.

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Epidemiology

Frequency

United States

Ectopia lentis is a rare condition. Incidence in the general population is unknown. The most common cause of ectopia lentis is trauma, which accounts for nearly one half of all cases of lens dislocation.

Mortality/Morbidity

Ectopia lentis may cause marked visual disturbance, which varies with the degree of lens displacement and the underlying etiologic abnormality.

Sex

Males appear more prone to ocular trauma than females; therefore, a male preponderance has been reported. Male and female frequency varies with the etiology of the lens displacement.

Age

Ectopia lentis can occur at any age.[6] It may be present at birth, or it may manifest late in life.

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Contributor Information and Disclosures
Author

Charles W Eifrig, MD Vitreoretinal Surgeon, Retina Associates of Orange County

Charles W Eifrig, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, American Society of Retina Specialists, Retina Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

J James Rowsey, MD Former Director of Corneal Services, St Luke's Cataract and Laser Institute

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Sigma Xi, Southern Medical Association, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Richard W Allinson, MD Associate Professor, Department of Ophthalmology, Texas A&M University Health Science Center; Senior Staff Ophthalmologist, Scott and White Clinic

Richard W Allinson, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous coauthor, David E Eifrig, MD, to the development and writing of this article.

References
  1. Jarrett WH II. Dislocation of the lens. A study of 166 hospitalized cases. Arch Ophthalmol. 1967 Sep. 78(3):289-96. [Medline].

  2. Nirankari MS, Chaddah MR. Displaced lens. Am J Ophthalmol. 1967 Jun. 63(6):1719-23. [Medline].

  3. Nelson LB, Maumenee IH. Ectopia lentis. Surv Ophthalmol. 1982 Nov-Dec. 27(3):143-60. [Medline].

  4. Clark CC. Ectopia lentis: a pathologic and clinical study. Arch Ophthalmol. 1939. 21:124-153.

  5. Albert DM, Jakobiec FA. Pathology of the lens. Principles and Practice of Ophthalmology. 2000. 2225-2239.

  6. Nelson L. Ectopia lentis in childhood. J Pediatr Ophthalmol Strabismus. 2008 Jan-Feb. 45(1):12. [Medline].

  7. Omulecki W, Wilczynski M, Gerkowicz M. Management of bilateral ectopia lentis et pupillae syndrome. Ophthalmic Surg Lasers Imaging. 2006 Jan-Feb. 37(1):68-71. [Medline].

  8. Parrish RK II. Anatomy, physiology, and pathology of the crystalline lens. Bascom Palmer Eye Institute's Atlas of Ophthalmology. 1999. 241.

  9. Duane T. Cataracts and systemic disease. Duane's Clinical Ophthalmology. 1999. 5: 13-14.

  10. Ganesh A, Smith C, Chan W, et al. Immunohistochemical evaluation of conjunctival fibrillin-1 in Marfan syndrome. Arch Ophthalmol. 2006 Feb. 124(2):205-9. [Medline].

  11. Wentzloff JN, Kaldawy RM, Chen TC. Weill-Marchesani syndrome. J Pediatr Ophthalmol Strabismus. 2006 May-Jun. 43(3):192. [Medline].

  12. Konradsen T, Kugelberg M, Zetterström C. Visual outcomes and complications in surgery for ectopia lentis in children. J Cataract Refract Surg. 2007 May. 33(5):819-24. [Medline].

 
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Ectopia lentis. Dislocated traumatic lens (cataract).
Ectopia lentis. Dislocated lens into the vitreous secondary to trauma.
Ectopia lentis. Supertemporal dislocation of a lens in the right eye of a patient with Marfan syndrome. Note the attached zonular fibers.
Ectopia lentis. Microspherophakia and spontaneous inferior dislocation of a lens in a patient with Weil-Marchesani syndrome.
 
 
 
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