PMID: 9541430Apr 16, 1998Paper

Congenital ectopia lentis. A Danish national survey

Acta Ophthalmologica Scandinavica
J Fuchs, T Rosenberg

Abstract

To elucidate demographic and nosologic characteristics of congenital ectopia lentis (ECL) in Denmark. A register of affected persons was established based on information provided from medical records and files in a nation-wide retrospective study, the Danish ECL-study. Three hundred and ninety-six cases (197 males, 199 females) with ECL were included in the study. By January 1st, 1993, the estimated prevalence rate of ECL was 6.4/100,000. The estimated average point prevalence rate at birth 1976-85 was 0.83/10,000 live born. Only in 69% of the cases (274/396) was a nosologic classification possible, based on preexisting information about familial occurrence, systemic and ocular findings: Marfan syndrome was found in 68.2% of these (187/274), ectopia lentis et pupillae in 21.2%, simple dominant ectopia lentis in 8.0%, homocystinuria in 1.1%, sulfite oxidase deficiency and Weill-Marchesani syndrome in 0.7% each. In the remaining 31% (122/396) a nosologic diagnosis could not be established. The majority of affected persons have congenital ectopia lentis as a manifestation of a systemic disease. It is therefore essential to evaluate ECL patients systemically with a general physical examination, a metabolic screening, and an echocar...Continue Reading

Citations

Jun 13, 2001·Acta Ophthalmologica Scandinavica·M CristofanilliG Scarsella
Dec 3, 2011·Eye·A ChandraD G Charteris
Nov 8, 2001·International Ophthalmology Clinics·N FaberowskiD S Walton
Jul 14, 2007·International Journal of Clinical Practice·A HasanA Child
Mar 26, 2008·Journal of Cataract and Refractive Surgery·Andrew C KopelEric R Holz
Dec 1, 2006·Survey of Ophthalmology·Arie Y NemetIrina S Barequet
May 24, 2014·Acta Ophthalmologica·Liv DrolsumSvein Ove Semb
Oct 18, 2007·Journal of Child Neurology·Chen HoffmannTammar Kushnir
Oct 19, 2017·Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen Gesellschaft·C DietzJ M Rohrbach
Jan 16, 2021·Therapeutic Advances in Ophthalmology·James E Davison
Sep 28, 2020·Progress in Retinal and Eye Research·Steven Bassnett
Feb 6, 2007·Journal of Pediatric Ophthalmology and Strabismus·Deependra Vikram SinghShailesh Gadagina
Jun 2, 2021·Translational Vision Science & Technology·Tianhui ChenYongxiang Jiang

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