Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 
  • Users Online: 44535
  • Home
  • Print this page
  • Email this page

   Table of Contents      
OPHTHALMIC IMAGE
Year : 2020  |  Volume : 68  |  Issue : 10  |  Page : 2255

Bilateral ectopia lentis in opposite quadrants in a child with Marfan syndrome


Department of Ophthalmology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India

Date of Web Publication23-Sep-2020

Correspondence Address:
Dr. Ramanuj Samanta
Department of Ophthalmology, All India Institute of Medical Sciences, ishikesh, Uttarakhand
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_301_20

Rights and Permissions

How to cite this article:
Sood G, Udenia H, Samanta R, Agrawal A, Ghawghawe H. Bilateral ectopia lentis in opposite quadrants in a child with Marfan syndrome. Indian J Ophthalmol 2020;68:2255

How to cite this URL:
Sood G, Udenia H, Samanta R, Agrawal A, Ghawghawe H. Bilateral ectopia lentis in opposite quadrants in a child with Marfan syndrome. Indian J Ophthalmol [serial online] 2020 [cited 2020 Oct 31];68:2255. Available from: https://www.ijo.in/text.asp?2020/68/10/2255/295734



A 15-year-old girl presented for evaluation of right eye exotropia since childhood. Ocular examination revealed best-corrected visual acuity of 20/40 in both eyes (refractive correction of -12DS/-3DC × 40° in right and -11DS/-2.5DC×130° in left eye); alternate divergent strabismus (40°) with left eye predominant fixation and ectopia lentis in opposite quadrants [Figure 1]a and [Figure 1]b. Fundus showed tessellated appearance with lattice degeneration. Physical examination revealed increased arm span to height ratio (1.052), decreased body upper to lower segment ratio (0.77), pectus carinatum and other clinical features [Figure 1]c, [Figure 1]d, [Figure 1]e, [Figure 1]f, [Figure 1]g compatible with Marfan Syndrome (MFS). Echocardiography revealed aortic root dilatation. She was diagnosed as ectopia lentis associated with MFS.[1],[2] Due to good visual acuity, she was kept under close ophthalmological follow-up and referred to cardiology for immediate management. The index report demonstrates an unusual ocular presentation (opposite lens subluxation in same individual) in a patient of MFS, which is rarely reported in literature.
Figure 1: (a and b) Anterior segment photograph demonstrating infero-temporal lens subluxation in right eye (a) and superior lens subluxation in left eye (b). (c-g) Clinical images showing right eye exotropia, bilateral enophthalmos, and malar hypoplasia (c); high arched palate (d), arachnodactyly (e), positive wrist sign (f), and thumb sign (g)

Click here to view


Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Loeys BL, Dietz HC, Braverman AC, Callewaert BL, De Backer J, Devereux RB, et al. The revised Ghent nosology for the Marfan syndrome. J Med Genet 2010;47:476-85.  Back to cited text no. 1
    
2.
Nemet AY, AssiaEI, Apple DJ, Barequet IS. Current concepts of ocular manifestations in Marfan syndrome. Surv Ophthalmol 2006;51:561-75.  Back to cited text no. 2
    


    Figures

  [Figure 1]



 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
References
Article Figures

 Article Access Statistics
    Viewed153    
    Printed0    
    Emailed0    
    PDF Downloaded26    
    Comments [Add]    

Recommend this journal