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A. C. Kopel, P. E. Carvounis, E. R. Holz; Surgical Management of Ectopia Lentis in Children. Invest. Ophthalmol. Vis. Sci. 2007;48(13):2242.
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To compare outcomes and complications of pars plana lensectomy-vitrectomy for the management of ectopia lentis in children with or without an iris-fixated intraocular lens.
Retrospective, interventional, comparative series of consecutive patients at Baylor College of Medicine, operated on by a single-vitreoretinal surgeon comprising 22 eyes with ectopia lentis in 12 pediatric patients who underwent pars plana lensectomy-vitrectomy with or without intraocular lens insertion (iris fixated) between June 1998 and October 2006. The primary outcome measure was the proportion of eyes achieving visual acuity (Va) 20/40 or better, while secondary outcome measures included mean visual acuity and proportion of eyes developing a complication. The study was approved by our Institution’s Review Board.
Ten of 22 eyes (6 patients) were left aphakic (Group A) while 12 of 22 eyes (6 patients) received an iris-sutured intraocular lens (Group L). Mean age at surgery (group A: 7.5 years, group L: 5.5 years, p=0.35), mean follow-up (group A: 21.9 months, group L: 19.8 months, p=0.82) and preoperative best corrected logMAR visual acuity (group A: 0.83 (20/134), group L: 0.80 (20/117), p=0.81) were similar in the two groups. There was no statistically significant difference between the two groups in the proportion of eyes achieving visual acuity of 20/40 or better (Group A: 5/10, Group L: 10/12, p=0.17) or in the mean postoperative best-corrected logMAR visual acuity (Group A: 0.41 (20/52), Group L: 0.24 (20/35), p= 0.18). Complications included intraocular lens dislocation in 4/12 eyes from Group L (33%, 95% confidence interval: 11-65%). Eyes with dislocated lenses underwent retrieval with refixation of the intraocular lens and resulted in a mean Va 20/28. No retinal detachment has been observed to date.
In the intermediate term, pars plana lensectomy-vitrectomy appears to be a safe option for the management of ectopia lentis. Insertion of an iris-fixated intraocular lens was not associated with better visual outcomes and carries a significant risk of dislocation. Patients with bilateral ectopia lentis are therefore best left aphakic.
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