Surgery of the hereditary subluxated lens in children

To describe the criteria and techniques for the surgery of hereditary subluxated lens in children and analyze the visual and surgical outcome after an extended period of follow-up.

From 1982 to 1994, 65 children who had subluxation of their lens were followed in the authors' clinic. Thirty-seven children (59 eyes) underwent surgery (27 eyes had Marfan syndrome; 23 eyes had essential dislocation; and 9 eyes had a diagnosis of homocystinuria, aniridia, microphthalmia, or Weill-Marchesani syndrome). The indications for surgery were best-corrected visual acuity of less than 20/70, forward sub-luxation of the lens to the anterior chamber, monocular diplopia, or rapidly progressing posterior subluxation of the lens. All 54 eyes with subluxation of the crystalline lens behind the iris underwent pars plana lensectomy combined with anterior vitrectomy. In five eyes with total forward dislocation of the lens in the anterior chamber, a limbal approach for removal of the lens and protruding vitreous was used.

Postoperative follow-up ranged from 12 to 144 months (average, 55 months). After surgery, 522 eyes (88%) of the entire group achieved an improvement (2 lines or more on the Snellen chart) in best-corrected visual acuity. Seven eyes (12%) did not show improvement and remained with the same visual acuity as before the surgery. Retinal detachment was detected in one eye 2 years after surgery and was the only major postoperative complications observed in these patients.

These results and the unique continuous and long-term follow-up on our patients demonstrate that lensectomy of ectopia lentis combined with anterior vitrectomy using a closed-system technique is a beneficial and relatively safe procedure.