Abstract
Purpose :
To study corneal integrity and eye findings in a set of pediatric Marfan Syndrome (MFS) patients with and without a history of ectopia lentis.
Methods :
A prospective, clinical, comparative case study was conducted on 54 eyes of 27 children with MFS. 40 eyes had a history of ectopia lentis resulting in lensectomy, vitrectomy and complete capsulectomy. 14 eyes were phakic with no clinical signs of natural lens subluxation. At each study visit over the course of 3 years best-corrected visual acuity (BCVA), tonometry, refractive error (RE), central corneal thickness (CCT), biomicroscopic examination, axial length (AL), anterior chamber depth (ACD), endothelial cell density (ECD) and corneal curvature were monitored, then compared and correlations were established.
Results :
Marfan Syndrome patients with a history of ectopia lentis, on average, had a greater central corneal thickness, longer axial length, decreased cell density, flatter corneal curvature and higher corneal astigmatism. Neither group showed signs of corneal ectasia. Endothelial cell loss was significant in one patient that underwent several procedures following removal of her natural lens in both eyes at age 6.
Conclusions :
In pediatric patients with a history of Marfan Syndrome or an FBN1 mutation and ectopia lentis requiring lensectomy, vitrectomy and complete capsulectomy, the ensuing aphakia may be corrected with glasses or contact lenses. In patients that are intolerable of glasses or contact lens wear, intraocular lenses remain a viable option although several more years of research are needed to deem the safest lens implant. Because corneal integrity is superior in patients with a history of Marfan Syndrome or an FBN1 mutation and no history of ectopia lentis, correction of a myopic refractive error does not have to be limited to glasses or contact lens wear. Corneal refractive surgery can also be considered.
This abstract was presented at the 2023 ARVO Annual Meeting, held in New Orleans, LA, April 23-27, 2023.