March 2012
Volume 53, Issue 14
ARVO Annual Meeting Abstract  |   March 2012
Refractive changes in children with Marfan Syndrome
Author Affiliations & Notes
  • Luisa M. Hopker
    Ophthalmology, UT Southwestern, Dallas, Texas
  • Serena X. Wang
    Ophthalmology, UT Southwestern, Dallas, Texas
  • David R. Weakley
    Ophthalmology, UT Southwestern, Dallas, Texas
  • Footnotes
    Commercial Relationships  Luisa M. Hopker, None; Serena X. Wang, None; David R. Weakley, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science March 2012, Vol.53, 6774. doi:
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      Luisa M. Hopker, Serena X. Wang, David R. Weakley; Refractive changes in children with Marfan Syndrome. Invest. Ophthalmol. Vis. Sci. 2012;53(14):6774.

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      © ARVO (1962-2015); The Authors (2016-present)

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Purpose: : To evaluate refractive changes, visual acuity, and frequency of lens subluxation and lensectomy in children with Marfan syndrome.

Methods: : Retrospective review of all Marfan syndrome patients seen by the ophthalmology service from 2000-2011. The mean spherical equivalent (SE), mean astigmatic error (AST) and best-corrected visual acuity (BCVA) were recorded at initial and final visits. Presence of lens subluxation and surgical intervention was evaluated.

Results: : Eight-three patients were seen during the study period and 120 eyes of 62 patients had available data and were included. Forty eyes (33%), had subluxed lenses and 22/40 eyes (55%), underwent lensectomy. For the non-subluxed eyes, mean age was 9.8 yrs (0.9-18.0), incidence of myopia was 58%, (mean SE -1.53 D, range-23.0 to +4.0), and mean BCVA was 0.08 LogMar. For subluxed lens eyes, mean age was 11.3 yrs (0.3-17) , incidence of myopia was 100% (mean SE was -8.37) and mean BCVA was 0.6 LogMar. Age, refractive status and BCVA were significantly different between these two groups (p=0.008, p<0.0001 and p<0.0001, respectively).Of patients with subluxed lenses without lensectomy, mean age was 7.7yrs (3-17.2), mean SE was -6.46D, mean AST was 3.65D and mean BCVA 0.49 LogMar. With mean follow-up of 4yrs (1-8.6), myopia increased significantly (mean -8.51 D range -3.75 to -18; p=0.01; R2=0.4), but not astigmatism ( AST= 3.79D range 0 to 10; p=0.11). Final BCVA was unchanged (0.49 LogMar).Patients undergoing lensectomy (available data of 13 eyes), mean age at surgery was 5.3yrs (0.3-10.7) with a mean pre-operative SE of -6.46D, mean AST of 5.15D and mean BCVA 0.85 LogMar. These patient did not differ significantly in mean age (p=0.12), SE (p=0.21) or AST (p=0.11) from non-operated patients. Post-operatively, mean SE was +11.34D and mean BCVA was 0.24 LogMar with mean follow up of 3.4yrs (0.1-5.8), BCVA improved from 0.85 LogMar to 0.24 after surgery (p=0.0004). One eye developed a retinal detachment.

Conclusions: : In our study the incidence of lens subluxation in children with Marfan Syndrome was 33%, and approximately half of these patients required surgery. There was a progressive myopic shift in those patients who did not undergo surgery. In patients who underwent lensectomy, BCVA improved significantly with surgery.

Keywords: refractive error development • clinical (human) or epidemiologic studies: outcomes/complications • visual acuity 

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