Hereditary subluxated lenses: visual performances and long-term follow-up after surgery.

PURPOSE: To evaluate the visual functions of children with hereditary dislocated lenses after within-the-bag lensectomy surgery and to report the results of 18 eyes with a follow-up interval of 11 to 19 years.

DESIGN

Prospective, noncomparative, interventional case series.

PARTICIPANTS

Ninety-seven children (age range, 6 months-16 years) were examined. Fifty-seven patients had poor functional vision. Surgery was performed in 97 eyes.

METHODS

Fifty-nine eyes (37 patients) were operated on from 1980 through 1994, and 38 eyes (20 patients) underwent surgery from 1994 through 2001. The indications for surgery included a best-corrected visual acuity of less than 20/60, progressive subluxation of the lens with bisection of the pupil, imminent total dislocation, or cataractous changes. Within-the-bag lensectomy combined with a limited anterior vitrectomy was carried out either through the pars plana or the limbus. The resulting aphakia was corrected with contact lenses, glasses, or both and the children were followed up regularly.

MAIN OUTCOME MEASURES: Best-corrected visual acuity, intraocular pressure, and complications after surgery.

RESULTS

Preoperative mean visual acuity for all operated eyes was 0.17 +/- 0.1 (standard deviation). The postoperative mean visual acuity of the 59 eyes operated from 1980 through 1994 was 0.60 +/- 0.25. For the group of 38 eyes operated on from 1994 through 2001, the postoperative visual acuity was 0.63 +/- 0.22. In most eyes, vision achieved shortly after the surgery remained stable or improved on a follow-up of up to 19 years after surgery. Repeated refraction of the operated eyes showed a trend toward a decrease of hypermetropia. No major complications occurred during or shortly after surgery. Ten children (18 eyes) followed up regularly for more than 11 years and up to 19 years after surgery demonstrated a stable visual acuity with minor fluctuations and an intraocular pressure within the normal range in all operated eyes.

CONCLUSIONS

Within-the-bag lensectomy is a safe and rewarding procedure for the handling of dislocated lenses in children.