Purpose: To retrospectively analyze the surgical outcomes of congenital/developmental cataracts in Japan. Methods: A mail questionnaire was sent to facilities engaged in surgical treatment of congenital cataracts. Results: Twenty-nine facilities reported on 809 eyes of 508 patients with congenital/developmental cataracts. Three hundred fifty-nine eyes underwent intraocular lens implantation (pseudophakia group), while 450 eyes were left aphakic (aphakia group). The average age at surgery was significantly higher in the pseudophakia group (70 ± 53 months) than in the aphakia group (14 ± 24 months) (P < 0.0001). A significantly larger proportion of patients with unilateral cataract (52.0 %) received IOL implantation than did those with bilateral cataracts (42.6 %) (P = 0.0224). The prevalence of associated ocular disorders, such as nystagmus, microcornea, nanophthalmos, and persistent fetal vasculature, was significantly higher in the aphakia group than in the pseudophakia group. Postoperatively, glaucoma developed more frequently in the aphakia group (5.8 %) than in the pseudophakia group (0.7 %) (P = 0.0003). Posterior capsule opacification developed more frequently in the pseudophakic eyes even when both posterior capsulotomy and anterior vitrectomy were performed, especially in patients aged 1 year or younger. Postoperative visual acuity was significantly better in the pseudophakia group than in the aphakia group, both in the unilateral and in the bilateral cases. Conclusions: Surgeons tended to select IOL implantation, rather than to leave the eye aphakic, in patients who were older at the time of surgery, had fewer coexisting ocular disorders, and suffered from unilateral cataract. Under such circumstances, pseudophakic eyes obtained significantly better postoperative visual acuity than did aphakic eyes.
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