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To describe a video-documented assessment of cataract type in the eyes of patients with monocular infantile cataract who were enrolled in the Infant Aphakia Treatment Study.The Infant Aphakia Treatment Study is a randomized clinical trial in which the investigators compared intraocular lens (IOL) versus contact lens correction in 114 infants, aged 28 days to7 months. A total of 83 videos were available for morphological analysis of cataract. Three examiners reviewed all surgical recordings and agreed on the cataract characteristics by using a score sheet to record the lens layer or configuration of the opacity.Nuclear cataract was present in 45 of 83 eyes (54%). Posterior capsule plaque was observed in 73 eyes (88%). All eyes with fetal nuclear cataract had associated posterior capsule plaque. Cortical cataract without nuclear involvement was seen in 21 eyes (25%). Posterior bowing of the posterior capsule was noted in 4 eyes (5%). Evidence of persistent fetal vasculature (PFV) was present in 18 eyes (22%). PFV was the only finding in 5 eyes but was also seen in combination with nuclear (7 eyes) and cortical cataracts (6 eyes). The entire lens was white in 3 eyes (4%), whereas the lens was partially resorbed in 7 (8%) eyes. Anterior capsule fibrosis was noted in 5 eyes with advanced cataract (1 with total cataract, 4 with partially resorbed lens).Nuclear opacities were common, but many different cataract types presented in infancy. PFV occurred in isolation or in association with cataract. Posterior capsule plaque was frequently noted, especially when a nuclear cataract was present.

Background: It generally is accepted that cataract surgery during the first 6 weeks of life is associated with the best visual outcomes for children with dense unilateral congenital cataracts. The latent period for children with dense bilateral congenital cataracts has not been clearly defined. Methods: The best-corrected visual acuity (BCVA) at 4 to 6 years of age was collected retrospectively on a series of 43 children with dense bilateral congenital cataracts from 4 institutions. All of the children underwent a lensectomy, posterior capsulotomy, and anterior vitrectomy at 36 weeks of age or younger. Results: Cataract surgery was performed at a mean age of 11.5 weeks for the better-seeing eye. BCVA was assessed when the children were a mean of 5.3 years of age. The visual acuities of 26 (60%) were 20/40 or better, 12 (28%) were 20/50 to 20/80, and 5 (12%) were 20/100 or worse. There was a trend for worse BCVA with increasing age at the time of surgery (r = 0.28, P = 0.07). We noted that a BCVA of 20/100 or worse occurred only among eyes undergoing surgery when infants were older than 10 weeks (≤10 weeks: 0/21 = 0% vs. >10 weeks: 5/22 = 23%, P = 0.049). Children with preoperative nystagmus had worse visual outcomes; only 38% of children with preoperative nystagmus achieved a BCVA of 20/40 or better compared with 74% of children without preoperative nystagmus (P = 0.03). Conclusion: Previous reports have proposed that cataract surgery during the first 5 to 8 weeks of life is associated with better visual outcomes in children with dense bilateral congenital cataracts. Our results would suggest that good visual outcomes can be achieved beyond this age, but the incidence of poor visual outcomes increases if cataract surgery is delayed beyond 10 weeks of age. The absence of preoperative nystagmus is a better predictor of a good visual outcome than the age at surgery.

Introduction Upgaze paralysis due to supranuclear defects can cause significant visual symptoms. There are only a few reports on the treatment of this problem in the literature. Patients with Parinaud's syndrome may also have retraction nystagmus and convergence on attempted upgaze that further complicates the treatment. Methods We performed a retrospective review of 48 patients with Parinaud's syndrome. Eleven patients were not able to elevate to the primary position and underwent surgery for the upgaze defect. Surgical treatments included transpositions in 2 patients, inferior rectus recessions in 8, which was combined with a superior rectus resection in one. Results Preoperative duction measurements demonstrated an average limitation on upgaze to −19° (−10 to −30) below midline with zero representing midline. Postoperative measurements showed marked improvement to +19 degrees above midline (15 to 30). Inferior rectus recessions were as effective as transpositions in restoring function. In addition, there was a marked reduction in the retraction nystagmus and upgaze convergence as well. Conclusions Visually significant upgaze limitation can be relieved with bilateral inferior rectus recessions in Parinaud's syndrome. Retraction nystagmus and covergence movements are also markedly improved.

We present the case of a 9-year-old patient with corneal decompensation that was treated with Descemet stripping automated endothelial keratoplasty (DSAEK) and followed for 18 months. Although the procedure has been used successfully in adult populations, to our knowledge, this is the first report of DSAEK with long-term follow-up in a child. The outcome suggests that endothelial keratoplasty may be a good alternative to penetrating keratoplasty in children. Endothelial keratoplasty is a new form of corneal transplantation that promises faster visual recovery compared with penetrating keratoplasty. With endothelial keratoplasty, the corneal architecture is preserved; therefore, large spherical and astigmatic errors are not induced, and stable refraction is achieved earlier in the course of therapy.

Purpose: The purpose of this study was to determine whether the high rates of ocular problems described in previous retrospective reports of individuals with fragile X syndrome were present in a prospective sample of young boys. Fragile X syndrome is currently considered the leading hereditary cause of mental retardation, with prevalence estimates of 1:2500 to 1:5000 males. Methods: Forty-eight boys with fragile X syndrome between the ages of 2.5 and 11 years were evaluated for ocular abnormalities. They received complete ophthalmic evaluations including assessment of visual acuity, cycloplegic refraction, ocular motility assessment, and dilated fundus examination. Results: Approximately 25% of the children had clinically significant ocular findings that included refractive errors (17%, primarily hyperopia and astigmatism) and strabismus (8%). Of the 42 children with quantifiable visual acuities, only 1 child, with diagnoses of nystagmus and hyperopia, had a Snellen visual acuity that was not within normal limits for his age. Three of the 5 children with gross measures of visual acuity had clinically significant findings: 2 had hyperopia greater than 3.5 diopters and 1 had esotropia. The other 2 children with gross measures of acuity and the 1 child without visual acuity assessment had cycloplegic refractions of +1.25 to +1.5 and ocular motility appeared normal. Conclusions: These results suggest that previous reports of high rates of vision problems, particularly strabismus, in boys with fragile X syndrome may have resulted from selection bias. Although we did observe a higher prevalence of strabismus than that found in the general population (8% vs 0.5% to 1%), the proportion of children having strabismus in our sample was much smaller than that reported in other studies of children with fragile X syndrome (30% to 40%). However, 17% of the sample did have significant refractive errors. In addition to evaluating the ocular motility of children with fragile X syndrome, cycloplegic refraction should also be performed to determine whether refractive problems are present.