TITLE

Visual Outcome of Three Generations in a Turkish Family With Marfan Syndrome

AUTHOR(S)
Ugurbas, Silay Canturk
PUB. DATE
November 2010
SOURCE
Journal of Pediatric Ophthalmology & Strabismus;Nov/Dec2010, Vol. 47 Issue 6, p373
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Purpose: The long-term outcome and postoperative visual acuity in a single family with ectopia lentis secondary to Marfan syndrome is presented. Methods: Thirteen family members, ages ranging from 3 to 47 years, were evaluated from 2006 to 2008. Ten members were found to have primary or secondary visual problems secondary to ectopia lentis.Results: Evaluation of 10 members with subluxated lenses in three generations showed that the leading cause of loss of vision (< 20/200) was amblyopia (n = 5), followed by surgical complications (n = 2) and retinal detachment (n = 1). Moderate amblyopia was observed in 8 additional eyes. Of those with long axial length (> 25 mm, n = 10), visual acuity ranged from 20/400 to 20/70 and no eyes tested better than 20/40. In eight eyes with an axial length of less than 25 mm, visual acuity ranged between counting fingers and 20/30. In this group, 50% had a visual outcome of 20/40 or better. Two eyes had no light perception and phthisis secondary to surgical complications. Of the 18 eyes in which axial length data were available, more eyes in the axial myopia group had unfavorable visual outcome (P = .04, Wilcoxon signed rank test). Conclusion: Amblyopia due to delayed surgical intervention was the leading cause of visual loss in three generations of this family. Axial high myopia was more frequent in deeply amblyopic family members.
ACCESSION #
55545507

 

Related Articles

  • Long-Term Results of Lensectomy in Children With Ectopia Lentis. So Young Kim; Ho Kyung Choung; Seong Joon Kim; Young Suk Yu // Journal of Pediatric Ophthalmology & Strabismus;Jan/Feb2008, Vol. 45 Issue 1, p13 

    Purpose: To report the clinical features and long-term visual results in children with ectopia lentis after lensectomy. Methods: A retrospective analysis was performed with the records of 78 eyes of 42 patients operated on and observed for at least 3 years. Results: Mean age at diagnosis was 4.6...

  • Artisan Aphakic Intraocular Lens Implantation in Cases of Subluxated Crystalline Lenses Due to Marfan Syndrome. Aspiotis, Miltiadis; Asproudis, Ioannis; Stefaniotou, Maria; Gorezis, Spiridon; Psilas, Konstantinos // Journal of Refractive Surgery;Jan2006, Vol. 22 Issue 1, p99 

    PURPOSE: To assess implantation of an Artisan aphakic intraocular lens (IOL) in cases with subluxated lenses due to Marfan syndrome. METHODS: Retrospective study of a small case series comprised of seven eyes (two children and three adults) with subluxated lenses due to Marfan syndrome that...

  • Hyperopic LASIK may help children who have anisometropic amblyopia. Hasson, Matt // Ocular Surgery News Europe/Asia-Pacific Edition;Oct2008, Vol. 19 Issue 10, p5 

    The artice discusses hyperopic LASIK as a treatment to improve visual acuity of children with anisometropic amblyopia who could not tolerate glasses and contact lenses. According to the article, both photorefractive keratectomy and LASIK have both shown promising results in treating mypoic...

  • A 15-Year-Old Boy With Exotropia.  // Journal of Pediatric Ophthalmology & Strabismus;Jan/Feb2008, Vol. 45 Issue 1, p7 

    The article presents the case of a 15-year-old boy who has amblyopia and a large angle exotropia. The patient has undergone a 6-mm left media rectus recession and 8-mm left lateral rectus resection when he was four years old according to an out-of-state medical records provided by his...

  • Efficacy of amblyopia therapy initiated after 9 years of age. Park, K. H.; Hwang, J.-M.; Ahn, J. K. // Eye;Jun2004, Vol. 18 Issue 6, p571 

    AIMS/PURPOSE: To evaluate the efficacy of occlusion therapy initiated after 9 years of age. METHODS: A total of 16 amblyopes of 9 years or older (range, 9.0-14.5 years; mean, 10.5 years) with a difference in visual acuity of over two lines between the eyes alter 4 weeks of first full-time...

  • Multifocal IOL affords long-term VA. Lindsay, Rebecca A.; Packer, Mark // Ophthalmology Times;5/15/2011, Vol. 36 Issue 10, p10 

    The article presents a case study of a 16-year-old female who was referred for worsening myopic astigmatism due to simple ectopia lentis. To correct her astigmatism, a decision to proceed with aspheric diffractive multifocal intraocular lense (IOL) implantation with scleral fixation was made....

  • The influence of surgery and intraocular lens implantation timing on visual outcome in traumatic cataract. Rumelt, Shimon; Rehany, Uri // Graefe's Archive of Clinical & Experimental Ophthalmology;Sep2010, Vol. 248 Issue 9, p1293 

    To compare the visual outcome of primary versus secondary traumatic cataract extraction and primary versus secondary intraocular lens (IOL) implantation. The medical charts of consecutive patients who developed cataract following open and closed-globe injuries and were referred to our institute...

  • Improved Visual Acuity after Frontalis Sling Surgery for Simple Congenital Ptosis. Rodríguez, Gustavo; Agramonte, Ileana; Rojas, Irene; de Jesús Méndez, Teresita; Vilar, Susana; Dorrego, Milagros // MEDICC Review;Jan2011, Vol. 13 Issue 1, p23 

    INTRODUCTION Congenital ptosis is malpositioning of the eyelids that, when moderate or severe, can negatively affect visual development during its critical period, resulting in amblyopia: diminished visual acuity with no apparent organic cause. Early diagnosis and timely treatment are essential...

  • The improving outcomes in intermittent exotropia study: outcomes at 2 years after diagnosis in an observational cohort. Buck, Deborah; Powell, Christine J.; Rahi, Jugnoo; Cumberland, Phillippa; Tiffin, Peter; Taylor, Robert; Sloper, John; Davis, Helen; Dawson, Emma; Clarke, Michael P. // BMC Ophthalmology;2012, Vol. 12 Issue 1, p1 

    Background: The purpose of this study was to investigate current patterns of management and outcomes of intermittent distance exotropia [X(T)] in the UK. Methods: This was an observational cohort study which recruited 460 children aged < 12 years with previously untreated X(T). Eligible subjects...

Share

Read the Article

Courtesy of VIRGINIA BEACH PUBLIC LIBRARY AND SYSTEM

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics