Ectopia Lentis Treatment & Management

  • Author: Charles W Eifrig, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Jul 22, 2011
 

Medical Care

Without an antecedent history of trauma, patients with ectopia lentis may possess a systemic disease with potentially deleterious effects; therefore, comanagement with the patient's pediatrician or internist is essential. Dietary restriction may be partially effective in patients with homocystinuria. Repair of an impending dissecting aortic aneurysm in Marfan syndrome may be life saving. If a hereditary condition is discovered, appropriate genetic counseling should be given. Moreover, all relatives with potential risk should be examined.

Treatment of glaucoma is dependent on the etiologic mechanism.

  • In pupillary block (eg, patients who have Weil-Marchesani with microspherophakia), laser peripheral iridotomy or iridectomy should be performed, and intraocular pressure elevation should be treated medically. Prophylactic laser iridotomy in patients with microspherophakia is beneficial.
  • Treatment of a lens dislodged into the anterior chamber is initially pharmacological with mydriasis/cycloplegia (to permit posterior migration of the lens behind the iris) in conjunction with ocular massage through a closed lid to promote this posterior migration. Surgical treatment will then be needed to prevent further complications.
  • Treatment of a dislocated lens in the vitreous is surgical; however, many vitreoretinal surgeons may advocate observation if no visual disturbance or impending retinal complication is apparent.
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Surgical Care

Lens surgery in ectopia lentis is technically challenging, and the numerous techniques and strategies are beyond the scope of this article.[12]

  • Indication for lensectomy
    • Lens in the anterior chamber
    • Lens-induced uveitis
    • Lens-induced glaucoma
    • Lenticular opacity with poor visual function
    • Anisometropia or refractive error not amenable to optical correction (eg, in a child to prevent amblyopia)
    • Impending dislocation of the lens
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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, 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
  1. Jarrett WH II. Dislocation of the lens. A study of 166 hospitalized cases. Arch Ophthalmol. Sep 1967;78(3):289-96. [Medline].

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

  3. Nelson LB, Maumenee IH. Ectopia lentis. Surv Ophthalmol. Nov-Dec 1982;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. In: Principles and Practice of Ophthalmology. 2000:2225-2239.

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

  7. 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].

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

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

  10. 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].

  11. Wentzloff JN, Kaldawy RM, Chen TC. Weill-Marchesani syndrome. J Pediatr Ophthalmol Strabismus. May-Jun 2006;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. May 2007;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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