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Related to ectopia lentis: Ectopia Lentis et Pupillae


A congenital or acquired positional abnormality of an organ or other part of thebody.
McGraw-Hill Dictionary of Scientific & Technical Terms, 6E, Copyright © 2003 by The McGraw-Hill Companies, Inc.
The following article is from The Great Soviet Encyclopedia (1979). It might be outdated or ideologically biased.



displacement of an organ into an adjacent body cavity or to the exterior. Ectopia may occur as a birth defect (anomaly of development) or as a result of injury to the walls of a cavity. Examples include ectopia of the urinary bladder to the exterior in case of partial closure of the anterior abdominal wall, ectopia of the spleen into the pleural cavity in case of hernia or traumatic rupture of the diaphragm, and hereditary ectopia lends, displacement of the crystalline lens of the eye.

Some types of ectopia are fatal, for example, ectopia cordis, congenital displacement of the heart outside the chest wall. Other types of ectopia can be corrected surgically.

The Great Soviet Encyclopedia, 3rd Edition (1970-1979). © 2010 The Gale Group, Inc. All rights reserved.
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Charteris, "Prevalence of ectopia lentis and retinal detachment in Marfan syndrome," Acta Ophthalmologica, vol.
The frequency increases to 8-38% in the presence of "ectopia lentis" (13, 14).
Different surgical techniques for the management of ectopia lentis have been reported such as lensectomy and anterior chamber IOL implantation, open loop3,4 or scleral fixation of IOL implant(SF-IOL).5 However, the risks include iritis, pigment dispersion, corec-topia, glaucoma, corneal endothelial loss, and retinal detachment.
Cardinal features of the disorder include tall stature, ectopia lentis, mitral-valve prolapsed, aortic-root dilatation, and aortic dissection (Collod-Beroud and Boileau, 2002).
Differential diagnoses of ectopia lentis also include trauma, syphilis, sulfite oxidase deficiency, and Weill--Marchesani syndrome.
* More weight is given to aortic root aneurysm / dissection and ectopia lentis. "In the absence of findings that are not expected in [Marfan syndrome], the combination of ectopia lentis and aortic root enlargement/dissection should be sufficient to make the diagnosis," the authors wrote, noting that all other cardiovascular and ocular manifestations of the condition, as well as findings in the skeleton, dura, skin, and lungs, "contribute to a systemic score that guides diagnosis when aortic disease is present but ectopia lentis is not."
Identification of FBN1 gene mutations in patients with ectopia lentis and marfanoid habitus.
By that time, other organ systems were known to be involved, as revealed by congenital displacement of the lens (ectopia lentis) and mitral valve disorders.
Homocystinuria causes mental retardation ectopia lentis osteoporosis, and thromboembolism.
One case is that of ectopia lentis as the result of familial traits alone, without involvement of other systems.
This study was undertaken to evaluate the refractive outcome and complication profile of sutureless, glueless, flapless, intrascleral fixation of PCIOLs in pediatric patient presenting with congenital ectopia lentis.
These included microcornea (2%), microcornea with iris coloboma (1%), sclerocornea (0.5%), iris coloboma (7%), aniridia (6.5%), ectopia lentis (4%), coloboma of lens (1%), anophthalmos (0.5%) and microphthalmos (1.5%).