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OBJECTIVE: Our goal was to report the presentation, complications, and treatment strategies for infants with congenital dacryocystocele. PATIENTS AND METHODS: We performed a retrospective chart review of all patients presenting with dacryocystoceles to Children's Hospital Boston between the years of 1997 and 2006. This study was approved by the institutional review board. RESULTS: Forty-two patients (46 eyes) were identified (18 male, 24 female), presenting at a median age of 7 days of life. Twenty-eight (65%) patients presented with cellulitis or dacryocystitis and required systemic antibiotics. Four (9.5%) patients presented with respiratory compromise. Resolution occurred with conservative treatment for 10 eyes, but 36 (78%) required surgical intervention. Seventeen (37%) eyes received probing in the office or nursery, and 13 (76%) were successful. Probing in the operating room was required for 21 patients, 8 of whom required marsupialization of an intranasal cyst. CONCLUSIONS: Although congenital dacryocystoceles may resolve with conservative measures, many become infected and require systemic antibiotic treatment, and most require surgical intervention. Referral in the early neonatal period can aid in timely intervention before complications such as infection occur. [ABSTRACT FROM AUTHOR]

Purpose: To demonstrate the association between neighborhood quality, using the Child Opportunity Index (COI), and the visual outcomes in children with unilateral pediatric cataract., Methods: We retrospectively reviewed the medical records of patients ≤18 years of age who presented at Boston Children's Hospital between 2000 and 2022 with unilateral cataracts and underwent cataract extraction. The collected data included patient demographics, residential addresses, the reason for the initial presentation, the age at presentation, the morphology of the cataract, and the final visual acuity. The COI was used as a metric of neighborhood quality. We analyzed the association between the COI scores and the final visual acuity., Results: We included a total of 80 patients in the study. The mean age at presentation was 2.59 ± 2.97 years. The most common reason for the initial presentation was a failed vision screen (27.5%), followed by an abnormal red reflex (25.0%). Posterior cataract was the most common type of cataract (66.3%). In the adjusted analysis, we found that a lower overall COI (β = -0.17; 95% CI, -0.31 to -0.02; P = 0.02) was associated with higher logMAR visual acuity (ie, worse visual acuity) at the final follow-up visit. Lower education and health and environment COI subdomains were associated with higher logMAR visual acuity at the final follow-up visit (P = 0.01 and P = 0.02, resp.)., Conclusions: Neighborhood environment quality is a potentially important variable for predicting visual outcomes in pediatric cataracts. Lower COI scores were associated with worse visual outcomes., (Copyright © 2025 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Purpose: To assess the utility of the first or second examinations for retinopathy of prematurity (ROP) in a medium-risk cohort of infants and to propose an optimization to the current ROP screening guidelines., Design: Retrospective consecutive study., Subjects: Infants screened for ROP between January 2017 and August 2023 at three different tertiary-level care neonatal intensive care units., Methods: The analysis focused on patients who did not meet criteria for micro or nanoprematurity (those born at ≥27 weeks and weighing ≥800 grams)., Main Outcome Measures: The primary outcomes included the rates of ROP and treatment-warranted ROP (TW-ROP), the presence of TW-ROP at the first or second inpatient examinations, the number of inpatient examinations performed before the first ROP diagnosis, and the overall number of inpatient examinations performed., Results: A total of 2,004 neonates were screened for ROP, of which 1,125 (56.1%) met the inclusion criteria. Of those patients, 237 (21.1%) had ROP. Eleven infants (1.0%) required treatment for active disease. The median postmenstrual age (PMA) at first ROP diagnosis was 35.3 weeks (IQR, 33.7-37 weeks; range, 30.3-46.7 weeks). The median PMA at stage 3 diagnosis was 39.3 weeks (IQR, 38.3-41.2 weeks; range, 35.1-44.4 weeks). The median PMA at first treatment was 39.6 weeks (IQR, 35.8-43.3 weeks; range, 35.3-49.6 weeks). The median number of inpatient examinations was 2.0 (IQR, 1-4 exams) for traditional screening, 1.0 (IQR, 1-3) after eliminating the first ROP inpatient examination, and 1.0 (IQR, 1-2) after eliminating the first and second ROP examinations (p<0.001). No patients were diagnosed with stage ≥3 nor met type 1 ROP treatment criteria at the first or second inpatient examination (100% sensitivity for TW-ROP). In this cohort, starting exams at 34 weeks PMA would be estimated to save 30.6% of the inpatient examinations., Conclusions: In infants not meeting criteria for micro and/or nanoprematurity, there was no type 1 ROP at either of the first two inpatient examinations. We propose an amendment algorithm (FIRST-ROP) in which ROP exams start at 34 weeks PMA for neonates born at ≥27 weeks gestational age and ≥800 grams., (Copyright © 2025. Published by Elsevier Inc.)

Purpose: We aim to validate the previously published TWO-ROP algorithm on an external data set., Design: Retrospective consecutive study., Subjects: Infants screened for retinopathy of prematurity (ROP) between January 2013 and August 2023 at a tertiary referral multi-site., Methods: Infants with higher birth weight (BW) and greater gestational age (GA) were included and stratified into 3 groups as follows: group 1 (BW <1500 g, GA ≥30 weeks), group 2 (BW ≥1500 g, GA <30 weeks), and group 3 (BW ≥1500 g, GA ≥30 weeks)., Main Outcome Measures: The rate of ROP, treatment-warranted ROP (TW-ROP), and number of inpatient examinations were evaluated in the 3 groups., Results: In total, 1095 (33.8%) patients met the inclusion criteria. The number of patients in groups 1, 2, and 3 was 837 (76.4%), 72 (6.6%), and 186 (17.0%), respectively. Retinopathy of prematurity was detected in 120 (11.0%) patients; the rate was 9.8% in group 1, 20.8% in group 2, and 12.4% in group 3 (P = 0.013). The overall mean number of inpatient examinations for patients undergoing traditional, TWO-ROP 36-week, and TWO-ROP 40-week screening systems was 1.95, 1.43, and 0.99, respectively (P < 0.001). Stage 3 was found in 9 eyes of 5 patients (0.5%, all zone II). Three eyes of 2 patients (0.2%) had plus disease. Two patients had bilateral laser treatment at 44 and 39.4 weeks postconceptional age (PCA); 3 out of 4 of these eyes met type 1 treatment criteria. Overall, the ROP screening burden saved was 9.0% and 16.7% for the TWO-ROP 36-week and 40-week systems, respectively. The sensitivity for TW-ROP was 100% for TWO-ROP 36-week system and 99.4% for TWO-ROP 40-week system., Conclusion: The TWO-ROP algorithm can reduce the number of inpatient examinations while maintaining safety. To ensure timely management, we recommend that the single first ROP examination occur at 38 to 39 weeks PCA., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2024. Published by Elsevier Inc.)

Purpose: To report refractive change at age 5 years in pseudophakic eyes operated before 2 years of age., Design: Retrospective case series at 10 Infant Aphakia Treatment Study (IATS) sites., Participants: Children who underwent cataract surgery with primary intraocular lens placement during the IATS enrollment years, including infants 1 to <7 months of age with bilateral cataract and all children 7-24 months of age, regardless of laterality., Methods: Change in spherical equivalent refractive error (diopters[D]) was calculated from 1-month postoperatively to 5 years of age and compared for unilateral and bilateral (first eye only) cases, and for 1 to <7 vs 7-24 months at surgery., Main Outcome Measures: Refractive change (D) from surgery to age 5 years., Results: 96 children were included: 50 unilateral (surgery 7-24 months of age); 46 bilateral (n=20, surgery at 1 to <7 months of age; n=26, at 7-24 months). Median refractive change was significantly greater for bilateral pseudophakic eyes operated at 1 to <7 months (7.50 D, range 2.5 to 15 D) vs. 7-24 months (1.94 D, range -1.88 to 7.75 D; P<0.001). For children aged 7-24 months at lensectomy, median change was similar between unilateral (3.25D, range -1.75 to 13.5 D) vs. bilateral cases (1.94D, range 1.88 to 7.75 D; P=0.053). By age 5 years, none of the pseudophakic eyes 1 to <7 months at surgery had <2.5D myopic shift, but 55% of bilateral and 25% of unilateral cases 7-24 months at surgery had < 2.5D myopic shift., Conclusions: Greater magnitude and variability in refractive change was found in pseudophakic eyes operated at 1 to <7 months and for unilateral cases, which should be considered when choosing IOL power and initial post-operative target refraction for infants and toddlers., (Copyright © 2024. Published by Elsevier Inc.)

Purpose: To identify which features of Sturge-Weber syndrome (SWS) were most associated with glaucoma onset, severity, and treatment failure at a tertiary care center., Design: Retrospective cross-sectional study., Subjects: Children who had SWS with and without glaucoma., Methods: Electronic health records were reviewed for all children with SWS presenting between 2014 and 2020. Examination and imaging findings from dermatology, neurology, and ophthalmology were collected. Logistic regression was used to identify factors associated with glaucoma-related outcomes., Main Outcome Measures: Primary outcomes included glaucoma development, progression to surgery, and treatment failure. Failure was defined as having a final intraocular pressure >21 mmHg, devastating complication, or ≤20/200 vision., Results: Twenty-three of 44 SWS patients (52.3%) developed glaucoma, and 6 of 23 patients (26.1%) had both eyes affected. Sixteen of 29 eyes (55.2%) required surgery, and 29.6% overall met our failure criteria (mean follow-up: 5.1 ± 4.3 years). Glaucoma diagnosis was associated with bilateral port-wine birthmarks (PWBs; odds ratio [OR] 5.9; 95% confidence interval [CI] 1.3-43.2), PWB with any lower eyelid involvement (OR 9.7, 95% CI 2.6-44.5), and choroidal hemangiomas (OR 3.8, 95% CI 1.1-13.8), but was not associated with upper eyelid or leptomeningeal angiomas, seizures, prior hemispherectomy, or pulsed-dye laser. Eyes that progressed to surgery were more likely to have PWB affecting the lower eyelid (OR 33.7, 95% CI 4.5-728.0). No clinical or demographic factors were associated with treatment failure. In most cases, angle surgery failed (72.7%) but was a temporizing measure before subconjunctival filtering surgery., Conclusions: Lower eyelid and choroidal angiomas were associated with glaucoma diagnosis, suggesting a spatial relationship with SWS findings. However, leptomeningeal angiomas were not associated, possibly because these are further from the eye., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2024 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Background/aims: This was a cross-sectional comparative study. We evaluated the ability of three large language models (LLMs) (ChatGPT-3.5, ChatGPT-4, and Google Bard) to generate novel patient education materials (PEMs) and improve the readability of existing PEMs on paediatric cataract., Methods: We compared LLMs' responses to three prompts. Prompt A requested they write a handout on paediatric cataract that was 'easily understandable by an average American.' Prompt B modified prompt A and requested the handout be written at a 'sixth-grade reading level, using the Simple Measure of Gobbledygook (SMOG) readability formula.' Prompt C rewrote existing PEMs on paediatric cataract 'to a sixth-grade reading level using the SMOG readability formula'. Responses were compared on their quality (DISCERN; 1 (low quality) to 5 (high quality)), understandability and actionability (Patient Education Materials Assessment Tool (≥70%: understandable, ≥70%: actionable)), accuracy (Likert misinformation; 1 (no misinformation) to 5 (high misinformation) and readability (SMOG, Flesch-Kincaid Grade Level (FKGL); grade level <7: highly readable)., Results: All LLM-generated responses were of high-quality (median DISCERN ≥4), understandability (≥70%), and accuracy (Likert=1). All LLM-generated responses were not actionable (<70%). ChatGPT-3.5 and ChatGPT-4 prompt B responses were more readable than prompt A responses (p<0.001). ChatGPT-4 generated more readable responses (lower SMOG and FKGL scores; 5.59±0.5 and 4.31±0.7, respectively) than the other two LLMs (p<0.001) and consistently rewrote them to or below the specified sixth-grade reading level (SMOG: 5.14±0.3)., Conclusion: LLMs, particularly ChatGPT-4, proved valuable in generating high-quality, readable, accurate PEMs and in improving the readability of existing materials on paediatric cataract., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Prcis: Transscleral cyclophotocoagulation (TS-CPC) and endoscopic cyclophotocoagulation (ECP) were effective in reducing intraocular pressure (IOP) and glaucoma medications in childhood glaucoma., Objective: To report the outcomes of continuous wave TS-CPC and ECP in childhood glaucoma., Materials and Methods: We performed a systematic search of relevant databases. We collected data on age, follow-up duration, type of glaucoma, previous surgical interventions, preoperative and postoperative IOP, preoperative and postoperative number of glaucoma medications, adverse events, number of sessions, and success rates at different time points. The main outcome measures are the amount of IOP and glaucoma medication reduction., Results: We included 17 studies studying 526 patients (658 eyes); 11 evaluated the effectiveness of TS-CPC (268 patients, 337 eyes), 5 evaluated ECP (159 patients, 197 eyes), and one study compared both techniques (56 patients, 72 eyes for TS-CPC vs 43 patients, 52 eyes for ECP). The median duration of follow-up was 28 months in the TS-CPC group and 34.4 months in the ECP group. The mean number of treatment sessions was 1.7 in the TS-CPC and 1.3 in the ECP. In the TS-CPC group, the mean IOP was significantly reduced from 31.2 ± 8 to 20.8 ± 8 mm Hg at the last follow-up ( P < 0.001). The mean number of glaucoma medications was reduced from 2.3 ± 1.3 to 2.2 ± 1.3 ( P = 0.37). In the ECP group, there was also a significant reduction in the mean IOP from 32.9 ± 8 mm Hg with a mean of 1.7 ± 0.7 glaucoma medications to 22.6 ± 9.8 mm Hg ( P < 0.0001) on 1.2 ± 1.1 medications ( P = 0.009) at the last follow-up., Conclusion: Both TS-CPC and ECP were effective in reducing the IOP and glaucoma medications in childhood glaucoma. Multiple treatment sessions were required., Competing Interests: Disclosure: A.S.K. received grant support from Abbvie, New Jersey Health Foundation, and a fund for the NJ Blind. He is a consultant in Alcon, Glaukos. Also, he is the Speaker Bureau in Abbvie, Bausch & Lomb. The Bascom Palmer Eye Institute is supported by NIH Center Core Grant P30EY014801 and a Research to Prevent Blindness Unrestricted Grant. R.K.L. is supported by the Walter G. Ross Foundation. The remaining authors declare no conflict of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: To report timing of diagnosis and treatment of glaucoma following cataract surgery (GFCS) in a large cohort of infants undergoing cataract surgery at a tertiary care center., Study Design: Cross-sectional study., Participants: All consecutive infants that underwent cataract surgery over a 30-year period from January 1991 to December 2021 were included if they had at least 1 year follow-up., Methods: The data collection included age at time of cataract surgery, presence of associated ocular or systemic conditions, age at diagnosis of GFCS, and treatment required to control GFCS. Glaucoma diagnosis required intraocular pressure (IOP) > 21 mmHg on > 2 visits with glaucomatous optic nerve head changes and/or visual field changes, or in young children, other anatomic changes such as corneal enlargement or haze or accelerated axial elongation and myopic shift., Main Outcome Measures: The incidence of GFCS was calculated. Linear regression was performed to assess the effect of age at time of cataract surgery. Analysis of risk factors and treatment modalities was performed using univariate and multivariate analysis., Results: Three hundred eighty-three eyes (260 patients) were analyzed. Median age at surgery was 52 days and median follow-up, 8 years. Glaucoma following cataract surgery was noted in 27% (104/383 eyes; median age at surgery, 45 days; median follow-up, 13 years.) Young age at surgery (< 3 months) was the greatest risk factor (P = 0.001) but the incidence was similar for infants operated in the first, second, or third month of life (25%, 36%, 40%, respectively, P = 0.4). Microcornea (41%, P < 0.0001), poorly dilating pupils (25%, P = 0.001), persistent fetal vasculature (PFV, 13%; P = 0.8), or anterior segment dysgenesis (3%, P = 0.02) were considered as additional risk factors. Surgical intervention was needed for 73% (24/33) eyes with early-onset GFCS compared with 14% (10/71) eyes with later-later onset GFCS (P < 0.0001). Medical treatment was effective in 86% with later-onset GFCS (P = 0.006)., Conclusions: The incidence of GFCS was 27%, and timing of diagnosis occurred in a bimodal fashion. Early-onset GFCS usually requires surgical intervention; medical treatment is effective for later-onset GFCS. Cataract surgery within the first 3 months of life, microcornea, and poorly dilating pupils were major risk factors., Financial Disclosure(s): The authors have no proprietary or commercial interest in any materials discussed in this article., (Copyright © 2023 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To examine the relationship between the Child Opportunity Index (COI) and severity of retinoblastoma at presentation., Design: Cross-sectional study., Methods: Children (age <18 years) treated for retinoblastoma at a tertiary care center between January 2000 and May 2023 were included. Residential census tract was used to determine the overall and domain-specific COI score for each child. Collected variables included age, sex, race/ethnicity, insurance type, and the International Classification of Retinoblastoma (ICRB) Group at initial examination. The primary outcome was Group D or E retinoblastoma at presentation. Mixed effects regression models were used to estimate the association of COI scores with disease severity at presentation., Results: This study included 125 children (51.2% male). Median age at diagnosis was 13 months (IQR, 5-24 months). One hundred nine (87.2%) children presented with Group D or E retinoblastoma and 33 (26.4%) resided in low or very low opportunity neighborhoods. Children residing in neighborhoods with low overall COI scores (OR, 1.62; 95% CI, 1.01-2.58; P = .044) and low education COI scores (OR, 1.77; 95% CI, 1.13-2.79; P = .013) were at increased odds of presenting with ICRB Group D or E retinoblastoma after adjusting for individual-level socioeconomic factors., Conclusion: Children residing in low opportunity neighborhoods-particularly low education opportunity-more often presented with advanced stage retinoblastoma than children residing in neighborhoods with higher opportunity scores. Efforts to improve preventative vision care and access to eye specialty care for children residing in low-resource areas are needed to reduce existing disparities in retinoblastoma., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose: To compare demographic and clinical factors associated with glaucoma following cataract surgery (GFCS) and glaucoma surgery rates between infants, toddlers, and older children using a large, ophthalmic registry., Design: Retrospective cohort study., Participants: Patients in the IRIS® Registry (Intelligent Research in Sight) who underwent cataract surgery at ≤ 17 years old and between January 1, 2013 and December 31, 2020., Methods: Glaucoma diagnosis and procedural codes were extracted from the electronic health records of practices participating in the IRIS Registry. Children with glaucoma diagnosis or surgery before cataract removal were excluded. The Kaplan-Meier estimator was used to determine the cumulative probability of GFCS diagnosis and glaucoma surgery after cataract surgery. Multivariable Cox regression was used to identify factors associated with GFCS and glaucoma surgery., Main Outcome Measures: Cumulative probability of glaucoma diagnosis and surgical intervention within 5 years after cataract surgery., Results: The study included 6658 children (median age, 10.0 years; 46.2% female). The 5-year cumulative probability of GFCS was 7.1% (95% confidence interval [CI], 6.1%-8.1%) and glaucoma surgery was 2.6% (95% CI, 1.9%-3.2%). The 5-year cumulative probability of GFCS for children aged < 1 year was 22.3% (95% CI, 15.7%-28.4%). Risk factors for GFCS included aphakia (hazard ratio [HR], 2.63; 95% CI, 1.96-3.57), unilateral cataract (HR, 1.48; 95% CI, 1.12-1.96), and Black race (HR, 1.61; 95% CI, 1.12-2.32). The most common surgery was glaucoma drainage device insertion (32.6%), followed by angle surgery (23.3%), cyclophotocoagulation (15.1%), and trabeculectomy (5.8%)., Conclusions: Glaucoma following cataract surgery diagnosis in children in the IRIS Registry was associated with young age, aphakia, unilateral cataract, and Black race. Glaucoma drainage device surgery was the preferred surgical treatment, consistent with the World Glaucoma Association 2013 consensus recommendations for GFCS management., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2023 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Background: Acquired early-onset bilateral cataracts can result from systemic etiologies or genetic disorders., Methods: In this observational study, we analyzed individuals 18 months to 35 years of age with acquired bilateral cataracts via a next-generation sequencing panel of 66 genes to identify disease-causing genetic variants., Results: Of 347 patients enrolled, 313 (90.2%) were <19 years (median, 8 years). We identified 74 pathogenic or likely pathogenic variants in 69 patients. Of the variants, we observed 64 single nucleotide variants (SNV) in 24 genes and 10 copy number variants (CNV) of varying size and genomic location. SNVs in crystallin genes were most common, accounting for 27.0% of all variants (20 of 74). Of those, recurrent variants included known cataract-causing variants CRYBA1 c.215+1G>A, observed in 3 patients, and CRYBA1 c.272&#95;274delGAG, CRYBB2 c.463C>T and c.562C>T, and CRYAA c.62G>A, each observed in 2 patients. In 5 patients, we identified CNV deletions ranging from 1.32-2.41 Mb in size associated with 1q21.1 microdeletion syndrome. Biallelic variants in CYP27A1 were identified in two siblings, one as part of targeted follow-up family testing, who were subsequently diagnosed with cerebrotendinous xanthomatosis, a rare but treatable autosomal recessive disease that often presents with acquired early-onset bilateral cataracts., Conclusions: This study demonstrates the utility of genetic testing in individuals with acquired early-onset bilateral cataracts to help clarify etiology. Identification of causative genetic variants can inform patient management and facilitate genetic counseling by identifying genetic conditions with risk of recurrence in families., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Purpose: Surgical removal of a vascularized pupillary membrane may be challenging with the risk of intraoperative bleeding and postoperative recurrence. We present a case of a 4-week-old who presented with anterior persistent fetal vasculature (PFV) and dense vascularized pupillary membrane in which the use of intracameral and intravitreal bevacizumab may have contributed to successful treatment., Observation: A 4-week-old-month-old otherwise healthy girl was referred to Boston Children's Hospital for evaluation of cataract. Ocular examination revealed right microcornea and vascularized pupillary membrane. The left eye exam was unremarkable. Only three weeks after surgical excision of the pupillary membrane and cataract extraction, recurrence of a vascular pupillary membrane was noted. Repeat membranectomy with pupilloplasty and use of intracameral bevacizumab was performed. The pupillary opening was further opened 5 months later, after repeat (intravitreal) bevacizumab, and the pupil has remained open and stable with >6 months' follow-up., Conclusion and Importance: This case suggests a role for bevacizumab in the management of PFV, however, a cause-and-effect relationship cannot be proven. Further prospective comparative studies are needed to confirm our findings., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: To determine the association between different neighborhood environment factors and the outcomes of childhood glaucoma., Design: A retrospective cohort., Participants: Childhood glaucoma patients ≤ 18 years of age at the time of diagnosis., Methods: A retrospective chart review of childhood glaucoma patients who presented to Boston Children's Hospital between 2014 and 2019. Data collected included etiology, intraocular pressure (IOP), management, and visual outcomes. Child Opportunity Index (COI) was used as a metric of neighborhood quality., Main Outcomes Measures: The association of visual acuity (VA) and IOP with COI scores using linear mixed-effect models, adjusting for individual demographics., Results: A total of 221 eyes (149 patients) were included. Of these, 54.36% were male and 56.4% were non-Hispanic Whites. The median age at the time of presentation was 5 months for primary glaucoma and 5 years for secondary glaucoma. The median age at the last follow-up was 6 and 13 years for primary and secondary glaucoma, respectively. A chi-square test revealed that the COI, health and environment, social and economic, and education indexes between primary and secondary glaucoma patients were comparable. For primary glaucoma, the overall COI and a higher education index were associated with a lower final IOP (P < 0.05), and higher education index was associated with a lower number of glaucoma medications at the last follow-up (P < 0.05). For secondary glaucoma, higher overall COI, health and environment, social and economic, and education indices were associated with better final VA (lower logarithms of the minimum angle of resolution VA) (P < 0.001)., Conclusions: Neighborhood environment quality is a potentially important variable for predicting outcomes in childhood glaucoma. Lower COI scores were associated with worse outcomes., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2023 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Background/aims: To establish normative curves for axial length and corneal curvature in the first decade of life., Methods: This is a cross-sectional study from a single institution in the United States. Children from 0- to 10-years of age with no underlying ocular pathology were prospectively enrolled to obtain ultrasound biometry and hand-held keratometry while under anaesthesia for an unrelated procedure. Older cooperative children had optical biometry obtained in-office. Logarithmic quantile regression models were used to determine the change in axial length and average keratometry as a function of age., Results: Single-eye measurements from 100 children were included. 75% of children were White and 49% female. Median axial length ranged from 20.6 mm (IQR, 20.2 to 21.1 mm) at age one year to 23.1 mm (IQR, 22.5 to 23.8 mm) at age ten years. Median average keratometry ranged from 44.1 D (IQR, 42.6 to 45.4 D) at age one year to 43.5 (IQR, 42.2 to 44.0 D) at age ten years. As age increased, there was a significant increase in axial length (0.74 mm per doubling of age; 95% CI, 0.62 to 0.82 mm), and a non-significant trend towards lower average keratometry (-0.21 D per doubling of age; 95% CI, -0.62 to 0.08 D)., Conclusions: We provide a set of normative charts for axial length and corneal curvature which may facilitate the identification of eyes outside the normal range and assist in the management of ocular conditions such as glaucoma or cataract., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: To compare baseline biometry measurements in eyes with pediatric cataract versus age-matched controls METHODS: This is a cross-sectional study conducted at a tertiary care hospital that included two arms-prospective arm to collect data from normal eyes and retrospective arm for eyes with pediatric cataract. In the prospective arm, biometry measurements were obtained in healthy children aged 0 to 10 years. Children under the age of four had measurements under anesthesia for an unrelated procedure, while older children had in-office measurements using optical biometry. For comparison, biometric data was collected in children with pediatric cataract through record review. One eye of each patient was randomly selected. Axial length (AL) and keratometry (K) were compared by age and laterality. The medians were compared using Wilcoxon rank-sum tests and variances using Levene's test., Results: There were 100 eyes in each arm, 10 eyes in each age bin of 1-year interval. There was more variability in baseline biometry in eyes with pediatric cataract and a trend for longer AL and steeper K in cataract eyes than aged-matched controls. The difference in AL means was significant in age group 2-4 years, and variances were significant across all age groups (p=0.018). Unilateral cataracts (n=49) showed a trend toward greater variability in biometry than bilateral cataracts, but this did not reach statistical significance., Conclusion: Baseline biometry measures are more variable in eyes with pediatric cataract compared to age-matched controls with a trend toward longer AL and steeper K., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Prcis: The effectiveness of Ahmed glaucoma valve (AGV) and Baerveldt glaucoma implant (BGI) was comparable in the management of childhood glaucoma over the long term despite initial better success rate with BGI. There were higher tube block and retraction rates in the BGI group and higher tube exposure rates in the AGV group., Purpose: To evaluate the outcomes and safety of AGV and BGI in childhood glaucoma., Materials and Methods: We performed a systematic literature review of publications from 1990 to 2022 in PubMed, EMBASE, ClinicalTrials.gov, Ovid MEDLINE, Cochrane CENTRAL, and google scholar for studies evaluating AGV and BGI in childhood glaucoma. Primary outcome measures were intraocular pressure (IOP) reduction and glaucoma medication reduction. The secondary outcome measures were the success rates and incidence of postoperative complications. We conducted a meta-analysis using a random effects model., Results: Thirty-two studies met the inclusion criteria. A total of 1480 eyes were included. The mean IOP reduction was 15.08 mm Hg ( P < 0.00001) for AGV and 14.62 ( P < 0.00001) for the BGI group. The mean difference between pre and postoperative glaucoma medications was 1 ( P < 0.00001) fewer medications in the AGV group and 0.95 ( P < 0.0001) fewer medications in the BGI group. There was a lower success rate in the AGV versus BGI groups at 2 years [63% vs 83%, respectively ( P < 0.0001) and 3 years (43% vs 79%, respectively ( P < 0.0001)]; however, the success was higher for AGV at 5 years (63% vs 56% in the BGI group, P < 0.001). The incidence of postoperative complications was comparable in the AGV and BGI groups, with rates of 28% and 27%, respectively., Conclusions: The IOP and glaucoma medication reduction, success rates, and incidence of postoperative complications were comparable in Ahmed and Baerveldt groups. Most literature comes from retrospective low-quality studies on refractory childhood glaucoma. Further larger cohort studies are needed., Competing Interests: Disclosure: The authors declare no conflict of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Cerebrotendinous xanthomatosis (CTX) is a rare, autosomal recessive bile acid synthesis disorder caused by pathologic variants in CYP27A1, a gene involved in bile acid synthesis. Impaired function in this gene leads to accumulation of plasma cholestanol (PC) in various tissues, often in early childhood, resulting in such clinical signs as infantile diarrhea, early-onset bilateral cataracts, and neurological deterioration. The current study aimed to identify cases of CTX in a population of patients with a greater CTX prevalence than the general population, to facilitate early diagnosis. Patients diagnosed with early-onset, apparently idiopathic, bilateral cataracts between the ages of 2 and 21 years were enrolled. Genetic testing of patients with elevated PC and urinary bile alcohol (UBA) levels was used to confirm CTX diagnosis and determine CTX prevalence. Of 426 patients who completed the study, 26 met genetic testing criteria (PC ≥ 0.4 mg/dL and positive UBA test), and 4 were confirmed to have CTX. Prevalence was found to be 0.9% in enrolled patients, and 15.4% in patients who met the criteria for genetic testing., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Purpose: Intravitreal injection of bevacizumab (IVB) offers advantages over laser photoablation for treatment of type 1 retinopathy of prematurity (ROP). However, retinal function has not, to date, been quantitatively compared following these interventions. Therefore, electroretinography (ERG) was used compare retinal function among eyes treated using IVB or laser, and control eyes. In addition, among the IVB-treated eyes, ERG was used to compare function in individuals in whom subsequent laser was and was not required., Design: Prospective clinical cohort study., Methods: ERG was used to record dark- and light-adapted stimulus/response functions in 21 children treated using IVB (12 of whom required subsequent laser in at least 1 eye for persistent avascular retina [PAR]). Sensitivity and amplitude parameters were derived from the a-wave, b-wave, and oscillatory potentials (OPs), representing activity in photoreceptor, postreceptor, and inner retinal cells, respectively. These parameters were then referenced to those of 76 healthy, term-born controls and compared to those of 10 children treated using laser only., Results: In children with treated ROP, every ERG parameter was significantly below the mean in controls. However, these significant ERG deficits did not differ between IVB- and laser-treated eyes. Among children treated using IVB, no ERG parameter was significantly associated with dose or need for subsequent laser., Conclusion: Retinal function was significantly impaired in treated ROP eyes. Function in IVB-treated eyes did not differ from that in laser-treated eyes. Functional differences also did not distinguish those IVB-treated eyes that would subsequently need laser for PAR., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Pediatric glaucoma is a constellation of challenging ophthalmic conditions that, left untreated, can result in irreversible vision loss. The mainstay of treatment for primary congenital glaucoma and select secondary glaucoma subtypes is angle surgery, either trabeculotomy or goniotomy. More recently, MIGS devices have been utilized to enhance the efficacy of these procedures. Despite the high success rates of these primary surgical options, refractory cases are challenging to manage. There is no consensus on the next step of treatment following primary angle surgery. Glaucoma drainage devices and trabeculectomies have been the traditional options, with laser treatment reserved for more severe cases. The benefits and disadvantages of each of these options are discussed.

Purpose: Evaluation for systemic diagnosis is an important part of pediatric cataract management. While there are reports on associated systemic and ocular associations in children with infantile cataracts, reports specifying associations in large cohorts of children undergoing cataract surgery are lacking., Methods: Retrospective chart review of consecutive patients undergoing cataract surgery at a pediatric tertiary referral center during 30-year period was performed. Associated systemic and ocular associations were recorded. The etiologies were analyzed depending on laterality, age, and gender., Results: Seven-hundred twenty-seven patients (1135 eyes) were included for analysis: 408 (56%) with bilateral and 319 (44%) with unilateral cataract. An identifiable cause for cataract was identified in 66% (270/408) bilateral and 55% (176/319) unilateral cataract patients. Hereditary cataract accounted for 22% of bilateral cataracts. An underlying syndrome or genetic diagnosis was found in 24% bilateral (97/408, 86 genetic/syndromic, 11 metabolic) but only in 2% of unilateral cases (5/319). Cataracts were the result of treatment for cancer, or other systemic conditions requiring steroids, in 60/408 bilateral (15%) and 15/319 (5%) unilateral cataract patients. In contrast, unilateral cataracts had higher ocular associations (49%, 156/319) than bilateral cataracts (6%, 23/408) primarily ocular trauma (20%, 64/319) and persistent fetal vasculature (20%, 62/319)., Conclusion: Clinicians should be aware of potential systemic and ocular associations among children with visually significant cataracts. Those with no family history of juvenile cataract should be evaluated for systemic associations, and referral to genetics may be warranted in select cases., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To determine the relative contribution of intraocular lens (IOL) calculation accuracy and ocular growth variability to the long-term refractive error predicted following pediatric cataract surgery., Methods: Pseudophakic eyes of children enrolled in the Infant Aphakia Treatment Study (IATS) were included in this study. Initial absolute prediction error (APE) and 10-year APE were calculated using the initial biometry, IOL parameters, postoperative refractions, and mean rate of refractive growth. The cohort was divided into children with a low-initial APE (≤1.0 D) and a high-initial APE ( >1.0 D). The 10-year APE was compared between the two groups using the Mann-Whitney U test. Linear regression was used to estimate the variability in prediction error explained by the initial IOL calculation accuracy., Results: Forty-two children with IOL placement in infancy were included. Seventeen eyes had a low initial APE, and 25 eyes had a high initial APE. There was no significant difference in APE 10 years following surgery between individuals with a low initial APE (median, 2.67 D; IQR, 1.61-4.12 D) and a high initial APE (median, 3.45 D; IQR, 1.64-5.10 D) (P = 0.7). Initial prediction error could explain 12% of the variability in the prediction error 10 years following surgery., Conclusions: IOL calculation accuracy contributed minimally to the refractive error predicted 10 years after cataract surgery in the setting of high variability in the rate of refractive growth., (Copyright © 2022 American Association for Pediatric Ophthalmology and Strabismus. All rights reserved.)

Purpose: To compare progression of myopia and refractive error in former premature infants with retinopathy of prematurity (ROP) treated using intravitreal bevacizumab (IVB) or laser., Design: Retrospective clinical cohort study., Methods: We identified premature infants with ROP treated using IVB from 2011 to 2020 and compared their longitudinal cycloplegic refraction data to that of infants with ROP treated using laser during the same timeframe. A subset of infants treated using IVB also underwent additional treatment using laser. We included cycloplegic refractions from 789 cumulative visits over a median 3.2 years. We used a linear mixed-effects model with a log decay function to evaluate how refraction changed with age after treatment., Results: In aggregate, the model estimated a significant (P < .001) trend in refraction-from slight hyperopia to relatively more myopic states. However, progression in laser-treated eyes was significantly (P < .001) more rapid, regardless of treatment with IVB. The number of laser spots resulted in increased myopic progression by approximately 0.16 diopters per 100 laser spots. Both ROP stage and zone had a significant effect on myopic progression, with more severe disease resulting in faster myopic progression. Random effects, including individual subject variation with nested variance for left and right eye, accounted for 86.4% of the remaining variance not explained by age and treatment., Conclusions: Laser treatment for severe ROP increases the trend to severe myopia. In our sample, IVB did not affect myopic progression but did substantially reduce the amount of consequent laser required to treat ROP. The effect of laser persists after accounting for differences in ROP stage and zone., (Copyright © 2022 Elsevier Inc. All rights reserved.)

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 <10 Δ . 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 <10 Δ versus 1.30 logMAR (20/400) for strabismus ≥10 Δ (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., (Copyright © 2022. Published by Elsevier Inc.)

Background: The refraction prediction error (PE) for infants with intraocular lens (IOL) implantation is large, possibly related to an effective lens position (ELP) that is different than in adult eyes. If these eyes still have nonadult ELPs as they age, this could result in persistently large PE. We aimed to determine whether ELP or biometry at age 10½ years correlated with PE in children enrolled in the Infant Aphakia Treatment Study (IATS)., Methods: We compared the measured refraction of eyes randomized to primary IOL implantation to the "predicted refraction" calculated by the Holladay 1 formula, based on biometry at age 10½ years. Eyes with incomplete data or IOL exchange were excluded. The PE (predicted - measured refraction) and absolute PE were calculated. Measured anterior chamber depth (ACD) was used to assess the effect of ELP on PE. Multiple regression analysis was performed on absolute PE versus axial length, corneal power, rate of refractive growth, refractive error, and best-corrected visual acuity., Results: Forty-three eyes were included. The PE was 0.63 ± 1.68 D; median absolute PE, 0.85 D (IQR, 1.83 D). The median absolute PE was greater when the measured ACD was used to calculate predicted refraction instead of the standard A-constant (1.88 D [IQR, 1.72] D vs 0.85 D [IQR, 1.83], resp. [P = 0.03]). Absolute PE was not significantly correlated with any other parameter., Conclusions: Variations in ELP did not contribute significantly to PE 10 years after infant cataract surgery., (Copyright © 2022 American Association for Pediatric Ophthalmology and Strabismus. All rights reserved.)

Purpose: To evaluate whether pediatric eyes that deviate from age-adjusted normative biometry parameters predict variation in myopic shift after cataract surgery., Methods: This is a single institution longitudinal cohort study combining prospectively collected biometry data from normal eyes of children <10 years old with biometry data from eyes undergoing cataract surgery. Refractive data from patients with a minimum of 5 visits over ≥5 years of follow-up were used to calculate myopic shift and rate of refractive growth. Cataractous eyes that deviated from the middle quartiles of the age-adjusted normative values for axial length and keratometry were studied for variation in myopic shift and rate of refractive growth to 5 years and last follow-up visit. Multivariable analysis was performed to determine the association between myopic shift and rate of refractive growth and factors of age, sex, laterality, keratometry, axial length, intraocular lens power, and follow-up length., Results: Normative values were derived from 100 eyes; there were 162 eyes in the cataract group with a median follow-up of 9.6 years (interquartile range: 7.3-12.2 years). The mean myopic shift ranged from 5.5 D (interquartile range: 6.3-3.5 D) for 0- to 2-year-olds to 1.0 D (interquartile range: 1.5-0.6 D) for 8- to 10-year-olds. Multivariable analysis showed that more myopic shift was associated with younger age (P < .001), lower keratometry (P = .01), and male gender (P = .027); greater rate of refractive growth was only associated with lower keratometry measures (P = .001)., Conclusions: Age-based tables for intraocular lens power selection are useful, and modest adjustments can be considered for eyes with lower keratometry values than expected for age., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Purpose: To describe the etiology, clinical features, and outcomes for a large contemporary cohort of children presenting with glaucoma at a tertiary referral center., Methods: The medical records of patients presenting to Boston Children's Hospital from January 2014 to July 2019 with a diagnosis of childhood glaucoma were retrospectively reviewed. Data regarding etiology, treatment, and visual and anatomic outcomes were collected; visual acuity outcomes were analyzed by laterality and diagnosis categories, using the Childhood Glaucoma Research Network (CGRN) classifications., Results: A total of 373 eyes of 246 patients (51% males) diagnosed with glaucoma before 18 years of age were identified. Mean follow-up was 7.04 ± 5.61 years; 137 cases were bilateral. The mean age at diagnosis was 4.55 ± 5.20 years. The most common diagnoses were glaucoma following cataract surgery (GFCS, 36.5%) and primary congenital glaucoma (PCG, 29.0%). Overall, 164 eyes (44.0%) underwent at least one glaucoma surgery. Intraocular pressure (IOP) was ≤21 mm Hg with or without glaucoma medications in 300 eyes (80.4%) at the last follow-up visit. Poor final best-corrected visual acuity (≤20/200) was found in 110 eyes; patients with poor final visual acuity tended to have poor visual acuity at presentation. The most common reason for poor vision was amblyopia. Uncontrolled IOP was an uncommon cause for vision loss., Conclusions: Childhood glaucoma can be challenging to manage, but poor vision usually results from amblyopia or presence of other ocular abnormalities or syndromes rather than glaucomatous optic neuropathy., (Copyright © 2022 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Refraction predictions from intraocular lens (IOL) calculation formulae are inaccurate in children. We sought to quantify the relationship between age and prediction error using a model derived from the biometry measurements of children enrolled in the Infant Aphakia Treatment Study (IATS) when they were ≤7 months of age. We calculated theoretical predicted refractions in diopters (D) using axial length, average keratometry, and IOL powers at each measurement time point using the Holladay 1 formula. We compared the predicted refraction to the actual refraction and calculated the absolute prediction error (APE). We found that the median APE was 1.60 D (IQR, 0.73-3.11 D) at a mean age (corrected for estimated gestational age) of 0.20 ± 0.14 years and decreased to 1.11 D (IQR, 0.42-2.20 D) at 10.60 ± 0.27 years. We analyzed the association of age with APE using linear mixed-effects models adjusting for axial length, average keratometry, and IOL power and found that as age doubled, APE decreased by 0.25 D (95% CI, 0.09-0.40 D). The accuracy of IOL calculations increases with age, independent of biometry measurements and IOL power., (Copyright © 2022 American Association for Pediatric Ophthalmology and Strabismus. All rights reserved.)

Purpose: This study aims to report long-term outcomes of secondary intraocular lens (IOL) implantation after early cataract surgery in children., Methods: This is a retrospective case series that included children undergoing secondary IOL implantation. The patients had either in-the-bag (ITB) or sulcus implantation; alternative methods of IOL fixation were excluded. Single-piece acrylic IOL was used for ITB and 3-piece acrylic or PMMA IOL for sulcus implantation. The visual acuity outcomes and rate of complications at the last follow-up visit were evaluated., Results: One hundred six eyes (70 patients) were analyzed. The mean follow-up was 5.5 ± 3.8 years. Sixty-two eyes (58.5%) had ITB; 44 eyes (41.5%) had sulcus IOL. All but 3 eyes (97.2%) showed stable or improvement in visual acuity. Early inflammation > grade 2 + was noted with sulcus IOL (84% vs 34%, p = 0.01); late inflammation requiring vitrectomy occurred in one eye with sulcus IOL. Mild decentration was seen in 2 eyes with sulcus IOL; one additional subluxed sulcus IOL was exchanged. Sixteen out of 106 eyes (16%) had glaucoma. Eyes that developed glaucoma had early primary surgery (mean, 0.2 years, p < 0.001, significant); there was no difference in glaucoma rates based on implantation site., Conclusion: Early postoperative inflammation is higher in eyes with sulcus implantation, but good visual acuity outcomes are noted after secondary IOL implantation in children. Glaucoma is the main complication that requires close monitoring and is associated with early age at primary surgery., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To review the scientific literature that evaluates the effectiveness of adjustable sutures in the management of strabismus for adult and pediatric patients., Methods: Literature searches were performed in the PubMed database through April 2021 with no date limitations and were restricted to publications in English. The searches identified 551 relevant citations, of which 55 were reviewed in full text. Of these, 17 articles met the inclusion criteria and were assigned a level of evidence rating by the panel methodologist. The search included all randomized controlled studies regardless of study size and cohort studies of 100 or more patients comparing the adjustable versus nonadjustable suture technique, with a focus on motor alignment outcomes or reoperation rates., Results: The literature search yielded no level I studies. Of the 17 articles that met the inclusion criteria, 11 were rated level II and 6 were rated level III. Among the 12 studies that focused on motor alignment outcomes, 4 small randomized clinical trials (RCTs) did not find a statistically significant difference between groups, although they were powered to detect only very large differences. Seven of 8 nonrandomized studies found a statistically significant difference in motor alignment success in favor of the adjustable suture technique, both overall and in certain subgroups of patients. Successful motor alignment was seen in both exotropia (in 3 studies that were not limited to children) and esotropia (in 1 study of adults and 2 of children). The majority of included studies that reported on reoperation rates found the rates to be lower in patients who underwent strabismus surgery with adjustable sutures, but this finding was not uniformly demonstrated., Conclusions: Although there are no level I studies evaluating the effectiveness of adjustable sutures for strabismus surgery, the majority of nonrandomized studies that met the inclusion criteria for this assessment reported an advantage of the adjustable suture technique over the nonadjustable technique with respect to motor alignment outcomes. This finding was not uniformly demonstrated among all studies reviewed and warrants further investigation in the development and analysis of adjustable suture techniques., (Copyright © 2021 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Reversal of optic nerve head (ONH) cupping has been considered an important clinical observation that signals surgical success and control of intraocular pressure (IOP) in childhood glaucoma. Many theories based on elasticity of pediatric eyes have been proposed, including anterior movement of the elastic lamina cribrosa or shrinkage of the scleral canal. The relationship between these factors and axonal loss is unclear when reversal of cupping has been observed. Retinal nerve fiber layer (RNFL) optical coherence tomography (OCT) can help to clarify this. We present a case series of 4 pediatric patients with secondary glaucoma that demonstrated ONH cupping reversal with pre- and postoperative clinical images and RNFL OCT., (Copyright © 2021 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Purpose: The aim was to evaluate the short-term outcomes of Ahmed clear path (ACP) valveless glaucoma drainage device in childhood glaucoma., Methods: Retrospective chart review of all patients 16 years or below with childhood glaucoma who had ACP implantation at Boston Children's Hospital from December 2019 to June 2020 with at least 6 months follow-up period., Results: The study included 7 eyes of 5 patients implanted by a single surgeon. The median follow-up was 12 months. The mean intraocular pressure (IOP) was reduced from 36±3.5 mm Hg on a mean of 2.7±0.6 glaucoma medications preoperatively to a mean IOP of 12.4±2.8 mm Hg (P<0.001) on a mean of 0.7±0.8 medications postoperatively at final follow-up (P=0.0009). Complete success was achieved in 4 eyes while qualified success was achieved in 3 eyes., Conclusion: The ACP glaucoma drainage device provided good short-term IOP control and technical advantages for implantation for pediatric eyes were observed., Competing Interests: Disclosure: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: The coronavirus disease 2019 (COVID-19) pandemic has significantly disrupted the delivery of healthcare. Although most nonurgent ophthalmology visits at Boston Children's Hospital were canceled, premature infants at risk for retinopathy of prematurity (ROP) still required timely, in-person care during the initial 3-month period of the infection surge in Massachusetts. The purpose of the current study was to report our protocols for mitigating risk of exposure to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) between infants and eye care providers and to compare examination rates and results with the same 3-month period in 2019., Methods: During the infection surge, we added new infection control measures and strengthened existing ones. Additional personal protective equipment was used, and the number of ophthalmologists rotating in the three high-capacity NICUs we service was limited., Results: More infants required ROP examinations during the study period in 2020 than in the same period in 2019, but fewer examinations were performed. There were no cases of missed progression to severe ROP during this time and no known transmission of SARS-CoV-2 between ROP patients and ophthalmology staff., Conclusions: Overall, effective ROP care was safely provided during the COVID-19 pandemic, and contact with this vulnerable population was minimized., (Copyright © 2021 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Importance: Glaucoma-related adverse events constitute serious complications of cataract removal in infancy, yet long-term data on incidence and visual outcome remain lacking., Objective: To identify and characterize incident cases of glaucoma and glaucoma-related adverse events (glaucoma + glaucoma suspect) among children in the Infant Aphakia Treatment Study (IATS) by the age of 10.5 years and to determine whether these diagnoses are associated with optic nerve head (ONH) and peripapillary retinal nerve fiber layer (RNFL) assessment., Design, Setting, and Participants: Analysis of a multicenter randomized clinical trial of 114 infants with unilateral congenital cataract who were aged 1 to 6 months at surgery. Data on long-term glaucoma-related status and outcomes were collected when children were 10.5 years old (July 14, 2015, to July 12, 2019) and analyzed from March 30, 2019, to August 6, 2019., Interventions: Participants were randomized at cataract surgery to either primary intraocular lens (IOL), or aphakia (contact lens [CL]). Standardized definitions of glaucoma and glaucoma suspect were created for IATS and applied for surveillance and diagnosis., Main Outcomes and Measures: Development of glaucoma and glaucoma + glaucoma suspect in operated-on eyes up to age 10.5 years, plus intraocular pressure, axial length, RNFL (by optical coherence tomography), and ONH photographs., Results: In Kaplan-Meier analysis, for all study eyes combined (n = 114), risk of glaucoma after cataract removal rose from 9% (95% CI, 5%-16%) at 1 year, to 17% (95% CI, 11%-25%) at 5 years, to 22% (95% CI, 16%-31%) at 10 years. The risk of glaucoma plus  glaucoma suspect diagnosis after cataract removal rose from 12% (95% CI, 7%-20%) at 1 year, to 31% (95% CI, 24%-41%) at 5 years, to 40% (95% CI, 32%-50%) at 10 years. Risk of glaucoma and glaucoma plus glaucoma suspect diagnosis at 10 years was not significantly different between treatment groups. Eyes with glaucoma (compared with eyes with glaucoma suspect or neither) had longer axial length but relatively preserved RNFL and similar ONH appearance and visual acuity at age 10 years., Conclusions and Relevance: Risk of glaucoma-related adverse events continues to increase with longer follow-up of children following unilateral cataract removal in infancy and is not associated with primary IOL implantation. Development of glaucoma (or glaucoma suspect) after removal of unilateral congenital cataract was not associated with worse visual acuity outcomes at 10 years., Trial Registration: ClinicalTrials.gov Identifier: NCT00212134.

Purpose: To evaluate outcomes of bilateral cataract surgery in children aged 7 to 24 months and compare rates of adverse events (AEs) with other Toddler Aphakia and Pseudophakia Study (TAPS) registry outcomes., Design: Retrospective clinical study at 10 Infant Aphakia Treatment Study (IATS) sites. Statistical analyses comparing this cohort with previously reported TAPS registry cohorts., Participants: Children enrolled in the TAPS registry between 2004 and 2010., Methods: Children underwent bilateral cataract surgery with or without intraocular lens (IOL) placement at age 7 to 24 months with 5 years of postsurgical follow-up., Main Outcome Measures: Visual acuity (VA), occurrence of strabismus, AEs, and reoperations., Results: A total of 40 children (76 eyes) who underwent bilateral cataract surgery with primary posterior capsulectomy were identified with a median age at cataract surgery of 11 months (7-23); 68% received a primary IOL. Recurrent visual axis opacification (VAO) occurred in 7.5% and was associated only with the use of an IOL (odds ratio, 6.10; P = 0.005). Glaucoma suspect (GS) was diagnosed in 2.5%, but no child developed glaucoma. In this bilateral cohort, AEs (8/40, 20%), including glaucoma or GS and VAO, and reoperations occurred in a similar proportion to that of the published unilateral TAPS cohort. When analyzed with children aged 1 to 7 months at bilateral surgery, the incidence of AEs and glaucoma or GS correlated strongly with age at surgery (P = 0.011/0.004) and glaucoma correlated with microcornea (P = 0.040) but not with IOL insertion (P = 0.15)., Conclusions: Follow-up to age 5 years after bilateral cataract surgery in children aged 7 to 24 months reveals a low rate of VAO and very rare glaucoma or GS diagnosis compared with infants with cataracts operated at < 7 months of age despite primary IOL implantation in most children in the group aged 7 to 24 months. The use of an IOL increases the risk of VAO irrespective of age at surgery., (Copyright © 2020 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To report outcomes of secondary intraocular lens (IOL) implantation in the Infant Aphakia Treatment Study (IATS)., Setting: Multicenter clinical practice., Design: Secondary analysis of patients enrolled in a randomized clinical trial., Methods: Details regarding all secondary IOL surgeries conducted in children enrolled in the IATS were compiled. Visual outcomes, refractive outcomes, and adverse events at the age of 10½ years were evaluated. Comparisons were made with eyes that remained aphakic and with eyes randomized to primary IOL placement., Results: The study included 114 infants, 57 in the aphakic group and 57 in the primary IOL group; 55 of 57 patients randomized to aphakia with contact lens correction were seen for the 10½-year study visit; 24 (44%) of 55 eyes had secondary IOL surgery. Median age at IOL surgery was 5.4 years (range 1.7 to 10.3 years). Mean absolute prediction error was 1.00 ± 0.70 diopters (D). At age 10½ years, the median logarithm of the minimum angle of resolution visual acuity (VA) was 0.9 (range 0.2 to 1.7), similar to VA in the 31 eyes still aphakic (0.8, range 0.1 to 2.9); the number of eyes with stable or improved VA scores between the 4½-year and 10½-year study visits was also similar (78% secondary IOL eyes; 84% aphakic eyes). For eyes undergoing IOL implantation after the 4½-year study visit (n = 22), the mean refraction at age 10½ years was -3.20 ± 2.70 D (range -9.90 to 1.10 D), compared with -5.50 ± 6.60 D (n = 53, range -26.50 to 3.00 D) in eyes with primary IOL (P = .03)., Conclusions: Delayed IOL implantation allows a more predictable refractive outcome at age 10½ years, although the range of refractive error is still large., (Copyright © 2021 Published by Wolters Kluwer on behalf of ASCRS and ESCRS.)

Purpose: The purpose of this assessment is to evaluate the accuracy of autorefraction compared with cycloplegic retinoscopy in children., Methods: Literature searches were last conducted in October 2019 in the PubMed and the Cochrane Library databases for studies published in English. The combined searches yielded 118 citations, of which 53 were reviewed in full text. Of these, 31 articles were deemed appropriate for inclusion in this assessment and subsequently assigned a level of evidence rating by the panel methodologists. Four articles were rated level I, 11 were rated level II, and 16 were rated level III articles. The 16 level III articles were excluded from this review., Results: Thirteen of the 15 studies comparing cycloplegic autorefraction with cycloplegic retinoscopy found a mean difference in spherical equivalent or sphere of less than 0.5 diopters (D); most were less than 0.25 D. Even lower mean differences were found when evaluating the cylindrical component of cycloplegic autorefraction versus cycloplegic retinoscopy. Despite low mean variability, there was significant individual measurement variability; the 95% limits of agreement were wide and included clinically relevant differences. Comparisons of noncycloplegic with cycloplegic autorefractions found that noncyloplegic refraction tends to over minus by 1 to 2 D., Conclusions: Cycloplegic autorefraction is appropriate to use in pediatric population-based studies. Cycloplegic retinoscopy can be valuable in individual clinical cases to confirm the accuracy of cycloplegic autorefraction, particularly when corrected visual acuity is worse than expected or the autorefraction results are not consistent with expected findings., (Copyright © 2020 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Retinopathy of prematurity (ROP) is the leading cause of childhood blindness in very-low-birthweight and very preterm infants in the United States. With improved survival of smaller babies, more infants are at risk for ROP, yet there is an increasing shortage of providers to screen and treat ROP. Through a literature review of new and emerging technologies, screening criteria, and analysis of a national survey of pediatric ophthalmologists and retinal specialists, the authors found the shortage of ophthalmology workforce for ROP a serious and growing concern. When used appropriately, emerging technologies have the potential to mitigate gaps in the ROP workforce., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder characterized by benign hamartomas occurring in multiple organ systems including the brain, kidneys, heart, lungs, liver, skin, and the eyes. Typical retinal findings associated with TSC include astrocytic hamartoma and achromic patch. While rare cases of cataract occurring in the setting of TSC have been reported, this is the first analysis of a large series of individuals with TSC that aims to quantify the frequency of this finding and to describe its clinical and genetic associations., Materials and Methods: This is a retrospective chart review of 244 patients from the Herscot Center for Tuberous Sclerosis Complex at the Massachusetts General Hospital who underwent complete ophthalmic examination. We describe the clinical and genetic findings in five individuals with TSC and juvenile cataract., Results: Four of five cases (80%) were unilateral. The cataract was described as having an anterior subcapsular component in 3 of 5 cases (60%). Three individuals (60%) underwent lensectomy with intraocular lens (IOL) implant and two individuals (40%) were observed. Genetic testing revealed a known disease-causing mutation in TSC2 in 100% of cases., Conclusions: Recent evidence suggests that mTOR signaling may play a role in cataract formation which could explain the relatively high incidence of juvenile cataract in this population. Juvenile cataract is a potentially under-recognized ocular manifestation of TSC.

Intraocular injury by epinephrine auto-injector has been rarely reported. Toxic risk to the intraocular structures is suspected, but the evidence is inconclusive. We present the case of a 2-year-old girl who sustained an injury to her right eye by inadvertent epinephrine injection. Cataract surgery was performed to treat an increasingly opaque lens, and an intraocular lens was implanted. The visual outcome was good, with no retinal damage., (Copyright © 2020 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Importance: Intravitreous bevacizumab (0.25 mg to 0.625 mg) is commonly used to treat type 1 retinopathy of prematurity (ROP), but there are concerns about systemic toxicity, particularly the risk of neurodevelopmental delay. A much lower dose may be effective for ROP while reducing systemic risk. Previously, after testing doses of 0.25 mg to 0.031 mg, doses as low as 0.031 mg were found to be effective in small cohorts of infants., Objective: To find the lowest dose of intravitreous bevacizumab effective for severe ROP., Design, Setting, and Participants: Between April 2017 and May 2019, 59 premature infants with type 1 ROP in 1 or both eyes were enrolled in a masked, multicenter, dose de-escalation study. In cohorts of 10 to 14 infants, 1 eye per infant received 0.016 mg, 0.008 mg, 0.004 mg, or 0.002 mg of intravitreous bevacizumab. Diluted bevacizumab was prepared by individual research pharmacies and delivered using 300-µL syringes with 5/16-inch, 30-guage fixed needles. Analysis began July 2019., Interventions: Bevacizumab intravitreous injections at 0.016 mg, 0.008 mg, 0.004 mg, or 0.002 mg., Main Outcomes and Measures: Success was defined as improvement by 4 days postinjection and no recurrence of type 1 ROP or severe neovascularization requiring additional treatment within 4 weeks., Results: Fifty-five of 59 enrolled infants had 4-week outcomes completed; the mean (SD) birth weight was 664 (258) g, and the mean (SD) gestational age was 24.8 (1.6) weeks. A successful 4-week outcome was achieved for 13 of 13 eyes (100%) receiving 0.016 mg, 9 of 9 eyes (100%) receiving 0.008 mg, 9 of 10 eyes (90%) receiving 0.004 mg, but only 17 of 23 eyes (74%) receiving 0.002 mg., Conclusions and Relevance: These data suggest that 0.004 mg may be the lowest dose of bevacizumab effective for ROP. Further investigation is warranted to confirm effectiveness of very low-dose intravitreous bevacizumab and its effect on plasma vascular endothelial growth factor levels and peripheral retinal vascularization.

Purpose: Angle surgery is the gold standard for the management of many types of childhood glaucoma, yet glaucoma drainage devices (GDD) are effective tools for refractory advanced cases or secondary childhood glaucomas. The purpose of this article is to review recently published literature focused on the use of GDDs for pediatric glaucoma, including GDD general principles and surgical outcomes., Methods: Literature review of various electronic databases was performed., Results: 71 papers were reviewed for outcomes of GDD in childhood glaucomas. Success rates were usually defined by intraocular pressure (IOP) of 5-22 mmHg, with or without medications. Success rates were typically higher for non-valved GDDs but varied by length of follow-up. Non-valved GDDs afford lower and longer-lasting IOP control in pediatric eyes than valved GDD, however, no randomized controlled trials exist in childhood glaucoma., Conclusion: Various designs of GDDs are available for management of childhood glaucoma with good short-term success rates; individual patient factors should be taken into consideration when selecting a specific device.

Purpose: To evaluate outcomes of bilateral cataract surgery in infants 1 to 7 months of age performed by Infant Aphakia Treatment Study (IATS) investigators during IATS recruitment and to compare them with IATS unilateral outcomes., Design: Retrospective case series review at 10 IATS sites., Participants: The Toddler Aphakia and Pseudophakia Study (TAPS) is a registry of children treated by surgeons who participated in the IATS., Methods: Children underwent bilateral cataract surgery with or without intraocular lens (IOL) placement during IATS enrollment years 2004 through 2010., Main Outcome Measures: Visual acuity (VA), strabismus, adverse events (AEs), and reoperations., Results: One hundred seventy-eight eyes (96 children) were identified with a median age of 2.5 months (range, 1-7 months) at the time of cataract surgery. Forty-two eyes (24%) received primary IOL implantation. Median VA of the better-seeing eye at final study visit closest to 5 years of age with optotype VA testing was 0.35 logarithm of the minimum angle of resolution (logMAR; optotype equivalent, 20/45; range, 0.00-1.18 logMAR) in both aphakic and pseudophakic children. Corrected VA was excellent (<20/40) in 29% of better-seeing eyes, 15% of worse-seeing eyes. One percent showed poor acuity (≥20/200) in the better-seeing eye, 12% in the worse-seeing eye. Younger age at surgery and smaller (<9.5 mm) corneal diameter at surgery conferred an increased risk for glaucoma or glaucoma suspect designation (younger age: odds ratio [OR], 1.44; P = 0.037; and smaller cornea: OR, 3.95; P = 0.045). Adverse events also were associated with these 2 variables on multivariate analysis (younger age: OR, 1.36; P = 0.023; and smaller cornea: OR, 4.78; P = 0.057). Visual axis opacification was more common in pseudophakic (32%) than aphakic (8%) eyes (P = 0.009). Unplanned intraocular reoperation occurred in 28% of first enrolled eyes (including glaucoma surgery in 10%)., Conclusions: Visual acuity after bilateral cataract surgery in infants younger than 7 months is good, despite frequent systemic and ocular comorbidities. Although aphakia management did not affect VA outcome or AE incidence, IOL placement increased the risk of visual axis opacification. Adverse events and glaucoma correlated with a younger age at surgery and glaucoma correlated with the presence of microcornea., (Copyright © 2019 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)