Journal of Pediatric Ophthalmology & Strabismus • Vol. 52, No. 5, 2015 269 Review Article New Management Strategies for Ectopia Lentis Melissa A. Simon, MD; Catherine A. Origlieri, MD; Anthony M. Dinallo, MD, MPH; Brian J. Forbes, MD, PhD; Rudolph S. Wagner, MD; Suqin Guo, MD ABSTRACT Ectopia lentis refers to displacement of the crystalline lens in the setting of various systemic and metabolic disorders. A literature review was conducted to inves- tigate the management of non-traumatic ectopia len- tis in the pediatric population, particularly focusing on surgical intervention. Both limbal and pars plana approaches for lensectomy are well established in the literature. Surgical options for intraocular lens implanta- tion in pediatric eyes with ectopia lentis include anterior chamber intraocular lenses and iris-fixated or scleral-fix- ated posterior chamber intraocular lenses. Recently, the use of capsular tension rings has also been described with promising results. Visual rehabilitation and treat- ment of amblyopia are essential for patients within the amblyogenic age group following surgical intervention. [J Pediatr Ophthalmol Strabismus. 2015;52(5):269-281.] INTRODUCTION Ectopia lentis is a rare condition associated with displacement of the crystalline lens of the eye. The lens is defined as subluxed when it is displaced but still contained within the pupillary space, whereas a luxated lens is completely detached from the cili- ary body and can be loose within either the anterior chamber or the vitreous cavity. 1,2 Displacement of the lens may induce refractive changes such as astig- matism, myopia, or aphakic hyperopia. Patients may also experience diplopia, particularly if the equator of the lens crosses the visual axis. 1 The underlying pathophysiology of ectopia lentis involves the disruption or dysfunction of the zonular fibers. 1 Nearly 50% of all cases of lens dislo- cation are caused by trauma, although ectopia lentis may occur in predisposed eyes even in the absence of trauma because this condition frequently occurs in the setting of systemic disease or metabolic disorder. 3 Isolated ectopia lentis, also called simple ectopia lentis, is an autosomal dominant disorder. This con- dition may present at birth or later in life, and the lens generally displaces upward and temporally. An- other heritable condition is ectopia lentis et pupil- lae, in which both the lens and pupil are displaced, usually in opposite directions. This disorder is auto- somal recessive and generally bilateral. 1 Microsphe- rophakia, an additional heritable disorder often as- sociated with Weill-Marchesani syndrome, is often bilateral and characterized by a small and spherical lens. Patients with microspherophakia typically have abnormalities in the zonular apparatus, accounting for the high incidence of lens dislocation in these eyes. 4 Although there are several systemic disorders associated with ectopia lentis, Marfan syndrome, Weill–Marchesani syndrome, and homocystinuria account for more than 75% of reported non- traumatic lens displacements. Marfan syndrome is From The Institute of Ophthalmology and Visual Science, New Jersey Medical School, Rutgers University, Newark, New Jersey (MAS, RSW, SG); Children’s National Health System, Washington, DC (CAO); Monmouth Medical Center, Long Branch, New Jersey (AMD); and Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania (BJF). Submitted: August 29, 2014; Accepted: May 5, 2015 The authors have no financial or proprietary interest in the materials presented herein. Drs. Simon and Origlieri contributed equally to this work and should be considered as equal first authors. Dr. Wagner did not participate in the editorial review of this manuscript. Correspondence: Suqin Guo, MD, The Institute of Ophthalmology and Visual Science, New Jersey Medical School, Rutgers University, 90 Bergen Street, DOC 6100, Newark, NJ 07101. E-mail: guos1@umdnj.edu doi: 10.3928/01913913-20150714-02