Ectopia Lentis

Author: Charles W Eifrig, MD; Chief Editor: Hampton Roy Sr, MD more...

Updated: Jul 22, 2011

What would you like to print?

Background
Pathophysiology
Epidemiology
Show All

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 (cataract).

Ectopia lentis. Dislocated lens into the vitreous secondary to trauma.

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.

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.

Proceed to Clinical Presentation

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, and Retina Society

Disclosure: Nothing to disclose.

Specialty Editor Board

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, and Texas Medical Association

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD Associate Professor 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, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

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, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association

Disclosure: Nothing to disclose.

Lance L Brown, OD, MD Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

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, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Acknowledgments

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

References

Jarrett WH II. Dislocation of the lens. A study of 166 hospitalized cases. Arch Ophthalmol. Sep 1967;78(3):289-96. [Medline].
Nirankari MS, Chaddah MR. Displaced lens. Am J Ophthalmol. Jun 1967;63(6):1719-23. [Medline].
Nelson LB, Maumenee IH. Ectopia lentis. Surv Ophthalmol. Nov-Dec 1982;27(3):143-60. [Medline].
Clark CC. Ectopia lentis: a pathologic and clinical study. Arch Ophthalmol. 1939;21:124-153.
Albert DM, Jakobiec FA. Pathology of the lens. In: Principles and Practice of Ophthalmology. 2000:2225-2239.
Nelson L. Ectopia lentis in childhood. J Pediatr Ophthalmol Strabismus. Jan-Feb 2008;45(1):12. [Medline].
Omulecki W, Wilczynski M, Gerkowicz M. Management of bilateral ectopia lentis et pupillae syndrome. Ophthalmic Surg Lasers Imaging. Jan-Feb 2006;37(1):68-71. [Medline].
Parrish RK II. Anatomy, physiology, and pathology of the crystalline lens. In: Bascom Palmer Eye Institute's Atlas of Ophthalmology. 1999:241.
Duane T. Cataracts and systemic disease. In: Duane's Clinical Ophthalmology. 5. 1999:13-14.
Ganesh A, Smith C, Chan W, et al. Immunohistochemical evaluation of conjunctival fibrillin-1 in Marfan syndrome. Arch Ophthalmol. Feb 2006;124(2):205-9. [Medline].
Wentzloff JN, Kaldawy RM, Chen TC. Weill-Marchesani syndrome. J Pediatr Ophthalmol Strabismus. May-Jun 2006;43(3):192. [Medline].
Konradsen T, Kugelberg M, Zetterström C. Visual outcomes and complications in surgery for ectopia lentis in children. J Cataract Refract Surg. May 2007;33(5):819-24. [Medline].

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.

View Table List

Read more about Ectopia Lentis on Medscape

About Medscape Reference | Editorial & Advisory Board

[ CLOSE WINDOW ]

About Medscape Reference

Medscape Reference is the most authoritative and accessible point-of-care medical reference for physicians and health care professionals. All content is available free of charge, both online and via mobile devices.

Medscape Reference articles represent the expertise and practical knowledge of top physicians and pharmacists from leading academic medical centers in the US and worldwide. In addition, our rigorous literature survey process allows us to rapidly integrate new practice-changing information into the relevant topics by systematically reviewing the major medical and pharmacy journals, news announcements, and important practice guidelines.

Medscape Reference provides comprehensive coverage across more than 30 medical specialties and is composed of the following areas:

Diseases and Conditions

More than 6,300 evidence-based and physician-reviewed disease and condition articles are organized to rapidly and comprehensively answer clinical questions, as well as to provide in-depth information in support of diagnosis, treatment, and other clinical decision-making. Topics are richly illustrated with more than 30,000 clinical photos and radiographic images.

Procedures

More than 650 clinical procedure articles provide clear, step-by-step instructions, and include instructional videos and images to allow clinicians to master the newest techniques or to improve their skills in procedures they have previously performed.

Drugs

More than 2,100 monographs, including prescription and over-the-counter drugs, plus over 5,000 corresponding brand-name drugs, herbals, and supplements, are provided. Drug images and pricing are also included. In addition, a Drug Interaction Checker provides rapid access to tens of thousands of interactions between brand and generic drugs, over-the-counter drugs, and herbals and supplements.

Anatomy

More than 70 anatomy articles feature clinical images and diagrams of the major human body systems and organs. The articles assist with the understanding of the anatomy involved in treating specific conditions and performing procedures. They can also facilitate physician-patient discussions.

Special Features
Best Evidence

This email newsletter features current and clinically relevant journal citations that are selected via our rigorous literature survey review process. These same citations are incorporated into the relevant Medscape Reference articles. This newsletter serves as a quick and easily accessible way to stay up to date with clinically relevant journal articles that impact clinical practice.

Case Studies

These challenging and classic case presentations provide a quick way for practicing physicians to sharpen their diagnostic and patient-management skills. Each case starts with how the patient presented in the clinic and then using a question and answer format, progresses through diagnosis and on to treatment and includes follow up information about outcome for the patient.

Image collections

Image collections are a visually-engaging presentation of both common and uncommon diseases, case presentations, and current controversies in medicine. They are presented in an image rich, slideshow format and are designed to challenge and expand physicians' knowledge of clinically important topics.

Medscape is the leading online destination for healthcare professionals for clinical information. In addition to clinical reference tools, Medscape also offers:

Medscape News Learn more
Medscape Education Learn more