Refractive Growth of the Crystalline Lens in the Infant Aphakia Treatment Study.

Objective: To compare the rate of refractive growth (RRG3) of the crystalline lens ("lens") versus the eye excluding the lens ("globe") for the fellow, noncataractous eyes of participants in the Infant Aphakia Treatment Study. Design: Retrospective cohort study. Subjects: A total of 114 children who had unilateral cataract surgery as infants were recruited. Biometric and refraction data were obtained from the normal eyes at surgery and at 1, 5, and 10 years. Subjects were included if complete data (axial length [AL], corneal power, and refraction) were available at surgery and at 10 years of age. Methods: At surgery and at 1, 5, and 10 years, AL, corneal power, and cycloplegic refraction were measured in the normal eyes. For each eye, the RRG3 was defined by linear regression of refraction at the intraocular lens (IOL) plane against log10 (age + 0.6 years). The RRG3 for the globe was based on IOL power for emmetropia; the RRG3 for the lens was based on IOL power calculated to give the observed refractions. Intraocular lens powers were calculated with the Holladay 1 formula. The means were compared with a paired 2-tailed t test, and linear regression was used to look for a correlation between RRG3 of the lens globe. Main Outcome Measures: The RRG3 of the lens and globe. Results: Complete data were available for 107 normal eyes. The mean RRG3 of the lenses was -12.0 ± 2.5 diopters (D) and the mean RRG3 of the globes was -14.1 ± 2.7 D (P < 0.001). The RRG3 of the lens correlated with the RRG3 of the globe (R 2  = 0.25, P < 0.001). Conclusions: The RRG3 was 2 D more negative in globes compared with lenses in normal eyes. Globes with a greater rate of growth tended to have lenses with a greater rate of growth.

Long-term strabismus outcomes after unilateral infantile cataract surgery in the Infant Aphakia Treatment Study.

PURPOSE: To characterize long-term strabismus outcomes in children in the Infant Aphakia Treatment Study (IATS). METHODS: This study was a secondary data analysis of long-term ocular alignment characteristics of children aged 10.5 years who had previously been enrolled in a randomized clinical trial evaluating aphakic management after unilateral cataract surgery between 1 and 6 months of age. RESULTS: In the IATS study, 96 of 109 children (88%) developed strabismus through age 10.5 years. Half of the 20 children who were orthophoric at distance through age 5 years maintained orthophoria at distance fixation at 10.5 years. Esotropia was the most common type of strabismus prior to age 5 years (56/109 [51%]), whereas exotropia (49/109 [45%]) was the most common type of strabismus at 10.5 years (esotropia, 21%; isolated hypertropia, 17%). Strabismus surgery had been performed on 52 children (48%), with 18 of these (35%) achieving microtropia <10Δ. Strabismus was equally prevalent in children randomized to contact lens care compared with those randomized to primary intraocular lens implantation (45/54 [83%] vs 45/55 [82%]; P = 0.8). Median visual acuity in the study eye was 0.56 logMAR (20/72) for children with orthotropia or microtropia <10Δ versus 1.30 logMAR (20/400) for strabismus ≥10Δ (P = 0.0003). CONCLUSIONS: Strabismus-in particular, exotropia-is common irrespective of aphakia management 10 years following infant monocular cataract surgery. The delayed emergence of exotropia with longer follow-up indicates a need for caution in managing early esotropia in these children. Children with better visual acuity at 10 years of age are more likely to have better ocular alignment.

Long-term Effect of Intraocular Lens vs Contact Lens Correction on Visual Acuity After Cataract Surgery During Infancy: A Randomized Clinical Trial.

Importance: Although intraocular lenses (IOLs) are often implanted in children, little is known whether primary IOL implantation or aphakia and contact lens correction results in better long-term visual outcomes after unilateral cataract surgery during infancy. Objective: To compare long-term visual outcomes with contact lens vs IOL correction following unilateral cataract surgery during infancy. Design, Setting, and Participants: This multicenter randomized clinical trial enrolled 114 infants with a unilateral congenital cataract who underwent cataract surgery with or without primary IOL implantation between 1 and 6 months of age. Data on long-term visual outcomes were collected when the children were age 10.5 years (July 14, 2015, to July 12, 2019) and analyzed from March 30 through August 6, 2019. Interventions: Intraocular lens implantation at the time of cataract surgery. Main Outcomes and Measures: Best-corrected visual acuity using the electronic Early Treatment Diabetic Retinopathy Study (E-ETDRS) testing protocol. Analysis was performed on an intention-to-treat basis. Results: Best-corrected visual acuity was measured at age 10.5 years for 110 of the 114 patients (96%) enrolled as infants. The participants included 58 girls (53%) and 52 boys (47%). Overall, 27 of the children (25%) had good (logMAR 0.30 [Snellen equivalent, 20/40] or better) visual acuity in the treated eye (12 [22%] in the IOL group and 15 [27%] in the aphakia group), but 50 children (44%) had a visual acuity of logMAR 1.00 (Snellen equivalent, 20/200) or worse (25 [44%] in the IOL group and 25 [44%] in the aphakia group). The median logMAR acuity in the treated eye was similar in children randomized to receive an IOL at the time of cataract extraction (0.89; interquartile range [IQR], 0.33-1.43 [Snellen equivalent, 20/159]) and those who remained aphakic (0.86; IQR, 0.30-1.46 [Snellen equivalent, 20/145]) (IQR, 0.30-1.46; P = .82). Although the overall difference in median visual acuity between the 2 groups was small, the estimate was imprecise (99% CI for the difference in medians was -0.54 to 0.47). Conclusions and Relevance: As in previous phases of the study, visual acuity outcomes were highly variable with only 27 children (25%) achieving excellent visual acuity in their treated eye and 50 children (44%) having poor vision in the treated eye. Implanting an IOL at the time of cataract extraction was neither beneficial nor detrimental to the visual outcome. Trial Registration: ClinicalTrials.gov Identifier: NCT00212134.

Strabismus surgery outcomes in the Infant Aphakia Treatment Study (IATS) at age 5 years.

PURPOSE: To report strabismus surgery frequency and outcomes after monocular infantile cataract surgery with or without IOL implantation. METHODS: The Infant Aphakia Treatment Study (IATS) is a randomized, multicenter clinical trial comparing treatment of aphakia with a primary IOL or contact lens in 114 infants with a unilateral congenital cataract. This report is a secondary outcome analysis of ocular motor data from IATS patients who underwent strabismus surgery prior to age 5 years. RESULTS: Strabismus surgery was performed in 45 (39%) patients (contact lens group [CL], 37%; IOL group, 42% [P = 0.70]). The indications for strabismus surgery were esotropia (62%), exotropia (33%), and hypertropia (4%). Infants who underwent cataract surgery at a younger age were less likely to undergo strabismus surgery (28-48 days, 12/50 [24%]; 49-210 days, 33/64 [52%]; P = 0.0037). Of the 42 patients who underwent strabismus surgery, 14 (33%) had a postoperative distance alignment within 8Δ of orthotropia at age 5 years. The 5-year visual acuity of children with strabismus was the same whether or not strabismus surgery had been performed (1.10 logMAR with surgery vs 1.00 without [P = 0.71]). CONCLUSIONS: In this study cohort, cataract surgery performed in the first 6 weeks of life was associated with a reduced frequency of strabismus surgery. Strabismus surgery outcomes in this population are guarded. Surgical improvement of strabismus does not appear to influence long-term visual acuity.

Sensorimotor outcomes by age 5 years after monocular cataract surgery in the Infant Aphakia Treatment Study (IATS).

PURPOSE: To evaluate sensorimotor outcomes among children in the Infant Aphakia Treatment Study (IATS). METHODS: Secondary outcome analysis was performed in this randomized, multicenter, clinical trial comparing treatment of unilateral aphakia with a primary intraocular lens (IOL) or contact lens (CL) correction. The alignment characteristics and sensory status of children through age 5 years were evaluated. RESULTS: In the IATS study, 91 of 112 children (81%) developed strabismus through age 5 years. Of 34 infants who were orthotropic at near 12 months after cataract surgery, at age 5 years 14 (41%) were orthotropic at distance, and 15 (44%) were orthotropic at near at age 5 years without strabismus surgery. Eight of 56 children (14%) in the CL group and 13 of 56 (23%) in the IOL group were orthotropic at distance (P = 0.33) at 5 years of age and had no history of strabismus surgery. Thirteen of 48 (27%) who underwent cataract surgery prior to 49 days of age compared to 8 of 64 (13%) who had surgery after 49 days were orthotropic (P = 0.085). Median visual acuity in the operative eye was 0.4 logMAR (20/50) for children with orthotropia or microtropia (<10(Δ)) versus 1.10 logMAR (20/252) for strabismus ≥10(Δ) (P = 0.0001). Stereopsis was detected in 12 of 21 children (57%) with orthotropia versus 16 of 89 (18%) children with strabismus (P = 0.0006). CONCLUSIONS: IOL placement does not reduce the development of strabismus after monocular congenital cataract surgery. Improved ocular alignment by age 5 years correlated strongly with improved visual acuity and stereopsis.

Cost of intraocular lens versus contact lens treatment after unilateral congenital cataract surgery in the infant aphakia treatment study at age 5 years.

PURPOSE: To analyze differences in the cost of treatment for infants randomized to primary intraocular lens (IOL) implantation versus optical correction with a contact lens (CL) after unilateral cataract surgery in the Infant Aphakia Treatment Study (IATS). DESIGN: Retrospective cost analysis of a prospective, randomized clinical trial based on Georgia Medicaid reimbursement data as well as actual costs of supplies used during the study, adjusted for inflation. PARTICIPANTS: The IATS is a multicenter (n = 12), randomized clinical trial comparing the optical treatment of aphakia with either primary IOL implantation (n = 57) or CL correction (n = 57) in 114 infants with unilateral congenital cataract. INTERVENTION: One hundred fourteen infants underwent unilateral cataract surgery and were either corrected optically by primary IOL implantation at the time of surgery or were corrected with a CL after surgery. MAIN OUTCOME MEASURES: The mean cost of cataract surgery and all additional surgeries, examinations, and supplies used up to 5 years of age. RESULTS: The 5-year treatment cost of an infant with a unilateral congenital cataract corrected optically with an IOL was $27 090 versus $25 331 for a patient treated with a CL after initial cataract surgery. The total cost of supplies was $3204 in the IOL group versus $7728 in the CL group. CONCLUSIONS: Unilateral cataract surgery in infancy coupled with primary IOL implantation is approximately 7% more expensive than aphakia and CL correction. Patient costs are more than double with CL versus IOL treatment.

Stereopsis results at 4.5 years of age in the infant aphakia treatment study.

PURPOSE: To determine whether stereopsis of infants treated for monocular cataracts varies with the type of optical correction used. DESIGN: Randomized prospective clinical trial. METHODS: The Infant Aphakia Treatment Study randomized 114 patients with unilateral cataracts at age 1-7 months to either primary intraocular lens (IOL) or contact lens correction. At 4.5 years of age a masked examiner assessed stereopsis on these patients using 3 different tests: (1) Frisby; (2) Randot Preschool; and (3) Titmus Fly. RESULTS: Twenty-eight patients (25%) had a positive response to at least 1 of the stereopsis tests. There was no statistically significant difference in stereopsis between the 2 treatment groups: Frisby (contact lens, 6 [11%]; IOL, 7 [13%]; P = .99), Randot (contact lens, 3 [6%]; IOL, 1 [2%]; P = .62), or Titmus (contact lens, 8 [15%]; IOL, 13 [23%]; P = .34). The median age at surgery for patients with stereopsis was younger than for those without stereopsis (1.2 vs 2.4 months; P = .002). The median visual acuity for patients with stereopsis was better than for those without stereopsis (20/40 vs 20/252; P = .0003). CONCLUSION: The type of optical correction did not influence stereopsis outcomes. However, 2 other factors did: age at surgery and visual acuity in the treated eye at age 4.5 years. Early surgery for unilateral congenital cataract and the presence of visual acuity better than or equal to 20/40 appear to be more important than the type of initial optical correction used for the development of stereopsis.

One-year strabismus outcomes in the Infant Aphakia Treatment Study.

OBJECTIVE: To evaluate the characteristics of strabismus in infants who underwent cataract surgery with and without intraocular lens (IOL) implantation. DESIGN: Secondary outcome analysis in a prospective, randomized clinical trial. PARTICIPANTS: The Infant Aphakia Treatment Study is a randomized, multicenter (n = 12), clinical trial comparing treatment of aphakia with a primary IOL or contact lens in 114 infants with a unilateral congenital cataract. INTERVENTION: Infants underwent cataract surgery with or without placement of an IOL. MAIN OUTCOME MEASURES: The proportion of patients in whom strabismus developed during the first 12 months of follow-up was calculated using the life-table method and was compared across treatment groups and age strata using a log-rank test. RESULTS: Strabismus developed within the first 12 months of follow-up in 38 pseudophakic infants (life-table estimate, 66.7%) and 42 infants (life-table estimate, 74.5%) treated with contact lenses (P = 0.59). The younger cohort (<49 days) at the time of surgery demonstrated less strabismus (29 of 50; life-table estimate, 58.0%) than the older cohort (≥ 49 days; 51 of 64; life-table estimate, 80.0%; P<0.01). CONCLUSIONS: Intraocular lens placement does not prevent the early development of strabismus after congenital cataract surgery. However, strabismus was less likely to develop in infants whose cataract was removed at an earlier age. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

A 10-year overview of double elevator muscle weakening procedures.

OBJECTIVE: To report the effect of weakening the superior rectus and inferior oblique (IO) muscles on ocular rotations. DESIGN: Observational case series. We reviewed a 10-year period of medical records of consecutive patients who underwent bilateral 5- to 11-mm (mean, 8.0 mm; SD, 1.1 mm) superior rectus muscle recessions combined with an IO muscle recession, myectomy, or anterior transposition. The effects on ocular rotations and eyelid position were recorded for the 37 patients (69 eyes) who were followed up for at least 6 months postoperatively. Nonparametric 1-way analysis of variance was used to compare results across the 3 procedures. The setting was a subspecialty practice at an academic institution. RESULTS: Supraduction deficiency was significantly associated with transposition of the IO muscle anterior to the inferior rectus muscle insertion compared with the standard IO muscle recession (P = .001), and IO muscle myectomy (P = .009). Y-pattern exotropia occurred more frequently after transposition of the IO muscle anterior to the inferior rectus muscle insertion than other weakening procedures (P<.001). CONCLUSION: Transposition of the IO muscle anterior to the inferior rectus muscle insertion, combined with ipsilateral superior rectus muscle recession, results in more supraduction deficiency and more frequent Y-pattern exotropia compared with standard IO muscle recession.

Cataract associated with type-1 diabetes mellitus in the pediatric population.

PURPOSE: To report 14 cases (28 eyes) of cataract associated with type-1 diabetes mellitus in young children and adolescents. METHODS: Retrospective review of the medical records of 14 patients from seven institutions. All patients under the age of 18 years who met the inclusion criteria of type-1 diabetes mellitus and cataract were included. RESULTS: Mean age at the time of diabetes diagnosis was 9.8 years (range, 0.5-14 years), and mean age at cataract diagnosis was 11.7 years (range, 5-16 years). Two patients presented with cataracts one month before the diagnosis of diabetes; seven after the diagnosis of diabetes; and in five patients the cataract was found at the time the diabetes was diagnosed by the pediatrician. Nineteen out of 23 operated eyes had a best corrected post operative visual acuity of 20/40 or better. Two patients (4 eyes) developed diabetic retinopathy postoperatively. CONCLUSIONS: Although cataract formation in type-1 diabetes is rare, it is treatable and potentially sight-saving in young children and adolescents.

Cataracts and glaucoma in patients with oculocerebrorenal syndrome.

BACKGROUND: Oculocerebrorenal syndrome is an X-linked recessive hereditary oculocerebrorenal disorder characterized by congenital cataract, mental retardation, and Fanconi syndrome of the proximal renal tubules. Other ocular findings include glaucoma, corneal opacity (keloid), enophthalmos, and hypotonia. OBJECTIVE: To describe the treatment of 7 patients (14 eyes) with bilateral cataracts associated with oculocerebrorenal syndrome. Method Retrospective review. RESULTS: Seven patients with oculocerebrorenal syndrome had visually significant bilateral cataracts detected on their first full ophthalmic examination. All underwent bilateral cataract surgery. The mean age (of 14 eyes) at cataract extraction was 1.25 (median, 1.1) months. Glaucoma diagnosis and treatment were more variable. The mean age (of 11 eyes) at glaucoma diagnosis was 24.1 (range, 0.2-70.0) months: the mean age was 0.2 month (of 4 eyes) when glaucoma was diagnosed before cataract extraction, and the mean age was 37.7 months (of 7 eyes) when glaucoma was diagnosed after cataract extraction. All eyes were followed up for a mean of 100.8 (range, 38-190) months. Main Outcome Measure Treatment of 7 patients (14 eyes) with bilateral cataracts associated with oculocerebrorenal syndrome. CONCLUSIONS: Early identification and surgical removal of cataracts is recommended in patients with oculocerebrorenal syndrome. Despite this, visual acuity results will only rarely be better than 20/70, and nystagmus is likely. Patients should be monitored closely and regularly for changes in intraocular pressure, optic nerve cupping, and refractive error to rule out the development of glaucoma.