Pediatric Ophthalmology

PARTICIPANTS: This article includes 7293 infants (14 586 eyes) screened for ROP across 5 centers in the United States (Austin Retina Associates, Austin, TX; Bascom Palmer Eye Institute, Miami, FL; Beaumont Eye Institute, Royal Oak, MI; Massachusetts Eye and Ear, Boston, MA; and Stanford Byers Eye Institute, Stanford, CA). PURPOSE: To analyze the incidence and timing of treatment requiring retinopathy of prematurity (ROP) in extremely small premature infants. We hypothesize that the smaller the infant by gestational age and birthweight, the higher their likelihood of requiring treatment for ROP. DESIGN: Premature infants screened for Retinopathy of Prematurity from 2002-2022 were divided into cohorts based on the following criteria based on gestational age (GA) and birth weight (BW). "Micropremature infants" are infants born between 24-26 weeks GA and between 600-799 g BW. "Nanopremature infants" are born ≤ 24 weeks GA and ≤ 600 g BW. METHODS: Retrospective chart review. MAIN OUTCOME MEASURES: The incidence and timing of treatment-requiring ROP. RESULTS: We found that infants defined as nanopremature had a ∼63% chance of requiring treatment at an average postmenstrual age (PMA) of 36.6 weeks, whereas those defined as micropremature had a 30% chance of requiring treatment at an average PMA of 36.3 weeks. This significantly contrasts with the risk of all screened babies for ROP where the risk of requiring treatment was 8.5%. CONCLUSION: Micropremature and nanopremature infants are significantly more likely to require treatment for ROP. With demographic data matched to all 5 major US regions spanning the last decade, these results have the potential to inform neonatologists, pediatricians, and ophthalmologists of an important shift in the landscape of prematurity in the United States. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Ocular melanocytosis is a well-established risk factor for choroidal melanomas but, despite its reported associations in the literature, it is infrequently discussed in relation to orbital melanomas. The authors describe a teenage patient with ocular melanocytosis who presented with an asymptomatic ipsilateral right orbital mass associated with the lateral rectus muscle. An exploratory orbitotomy revealed a lesion lightly adherent to the underlying sclera. Histopathology demonstrated a markedly atypical epithelioid melanocytic proliferation, bound by a thin rim of superficial sclera, implying an origin from intrascleral melanocytes, likely within an emissary canal. Next-generation sequencing identified GNAQ and NF1 mutations. The histopathology and molecular genetics designated the lesion as having a uveal melanoma-like profile, suggesting that it may behave as a choroidal melanoma. This case underscores the importance of the association between ocular melanocytosis and orbital melanoma and provides additional evidence for primary orbital melanoma etiopathogenesis.

PURPOSE: Cerebral venous sinus thrombosis (CVST) is a rare but life-threatening event with significant neurologic and visual morbidity. In this study, we report on the natural history and visual outcomes of papilledema in children with CVST. DESIGN: Retrospective case series. METHODS: Patients with CVST evaluated by the Department of ophthalmology between 2000 and 2023 were included. Records were reviewed for presence and course of papilledema, treatment, and final visual outcomes following papilledema resolution. RESULTS: The study included 35 patients with a mean age of 9 ± 5 years and 40% were female. The most common risk factors for CVST were infection (69%), dehydration (26%), and hypercoagulability (23%). 31 patients (89%) had papilledema. Of these patients, 9 (29%) had progression of papilledema despite treatment, 17 patients (55%) did not have progression, and 5 patients (16%) lacked follow-up records. Initial Frisén grade among all cases was 2 ± 1, and cases with progression reached a grade of 4 ± 1 between 10 and 32 days following initial identification. Most patients (97%) were treated with anticoagulation and 100% required acetazolamide and/or lumbar puncture. Among 26 patients with follow-up, papilledema resolved in 107 ± 128 days. 54% of patients had permanent ophthalmic sequelae. An initial Frisén grade ≥3 (odds ratio [OR] 7.54, 95% confidence interval [CI] 6.53-8.70, p <0.001) was significantly associated with eventual optic atrophy. CONCLUSIONS: Children with CVST are at high risk for ophthalmologic sequelae. Papilledema can progress despite appropriate therapy. Our results highlight the importance of ophthalmologic follow-up during treatment course to prevent irreversible vision loss.

IMPORTANCE: Strabismus is a common ocular disorder of childhood. There is a clear genetic component to strabismus, but it is not known if esotropia and exotropia share genetic risk factors. OBJECTIVE: To determine whether genetic duplications associated with esotropia are also associated with exotropia. DESIGN, SETTING, AND PARTICIPANTS: This was a cross-sectional study conducted from November 2005 to December 2023. Individuals with constant or intermittent exotropia of any magnitude or a history of surgery for exotropia were recruited from pediatric ophthalmic practices. Data were analyzed from March to December 2023. EXPOSURE: Genetic duplication. MAIN OUTCOMES AND MEASURES: Presence of genetic duplications at 2p11.2, 4p15.2, and 10q11.22 assessed by digital droplet polymerase chain reaction. Orthoptic measurements and history of strabismus surgery were performed. RESULTS: A total of 234 individuals (mean [SD] age, 19.5 [19.0] years; 127 female [54.3%]) were included in this study. The chromosome 2 duplication was present in 1.7% of patients with exotropia (4 of 234; P = .40), a similar proportion to the 1.4% of patients with esotropia (23 of 1614) in whom it was previously reported and higher than the 0.1% of controls (4 of 3922) previously reported (difference, 1.6%; 95% CI, 0%-3.3%; P < .001). The chromosome 4 duplication was present in 3.0% of patients with exotropia (7 of 234; P = .10), a similar proportion to the 1.7% of patients with esotropia (27 of 1614) and higher than the 0.2% of controls (6 of 3922) in whom it was previously reported (difference, 2.8%; 95% CI, 0.6%-5.0%; P < .001). The chromosome 10 duplication was present in 6.0% of patients with exotropia (14 of 234; P = .08), a similar proportion to the 4% of patients with esotropia (64 of 1614) and higher than the 0.4% of controls (18 of 3922) in whom it was previously reported (difference, 5.6%; 95% CI, 2.5%-8.6%; P < .001). Individuals with a duplication had higher mean (SD) magnitude of deviation (31 [13] vs 22 [14] prism diopters [PD]; difference, 9 PD; 95% CI, 1-16 PD; P = .03), were more likely to have constant (vs intermittent) exotropia (70% vs 29%; difference, 41%; 95% CI, 20.8%-61.2%; P < .001), and had a higher rate of exotropia surgery than those without a duplication (58% vs 34%; difference, 24%; 95% CI, 3%-44%; P = .02). CONCLUSIONS AND RELEVANCE: In this cross-sectional study, results suggest that the genetic duplications on chromosomes 2, 4, and 10 were risk factors for exotropia as well as esotropia. These findings support the possibility that esotropia and exotropia have shared genetic risk factors. Whether esotropia or exotropia develops in the presence of these duplications may be influenced by other shared or independent genetic variants or by environmental factors.

Down syndrome (DS) is the most common chromosomal disorder in humans. DS is associated with increased prevalence of several ocular sequelae, including characteristic blue-dot cerulean cataract. DS is accompanied by age-dependent accumulation of Alzheimer's disease (AD) amyloid-β (Aβ) peptides and amyloid pathology in the brain and comorbid early-onset Aβ amyloidopathy and colocalizing cataracts in the lens. Quasi-elastic light scattering (QLS) is an established optical technique that noninvasively measures changes in protein size distributions in the human lens in vivo. In this cross-sectional study, lenticular QLS correlation time was decreased in adolescent subjects with DS compared to age-matched control subjects. Clinical QLS was consistent with alterations in relative particle hydrodynamic radius in lenses of adolescents with DS. These correlative results suggest that noninvasive QLS can be used to evaluate molecular changes in the lenses of individuals with DS.

PURPOSE: This study assesses the effectiveness and safety of using Ologen implants (Aeon Astron Europe BV, Leiden, The Netherlands) as an adjunctive therapy in childhood glaucoma surgeries. METHODS: We systematically reviewed the existing literature across various electronic databases to examine the effectiveness and safety of Ologen implants in childhood glaucoma surgeries. RESULTS: Our analysis encompassed 14 studies on the use of Ologen implants in childhood glaucoma. Among these, seven were prospective, five were retrospective, and two did not specify their study design. Success rates varied depending on the type of surgery and the included childhood glaucoma subtype. The success rates for Ologen implants-augmented surgeries were as follows: 33.3-70% for trabeculectomy, 50-81% for combined trabeculotomy-trabeculectomy procedure, 33%-87% for glaucoma drainage device, and 60% in deep sclerectomy. CONCLUSION: Ologen implant has a potential role in mitigating postoperative fibrosis and enhancing success rates in various childhood glaucoma surgeries. However, the existing literature is limited. Future comparative prospective studies with larger cohorts are needed.

IMPORTANCE: There is no consensus on the optimal surgical treatment for children with intermittent exotropia (IXT). OBJECTIVE: To compare the 5-year reoperation rates for children with IXT treated with horizontal muscle strabismus surgery using bilateral lateral rectus recession (BLR) vs unilateral lateral rectus recession with medial rectus resection (RR). DESIGN, SETTING, AND PARTICIPANTS: This cohort study examined data obtained from the Intelligent Research in Sight (IRIS) Registry on 7482 children (age, <18 years) with IXT who underwent horizontal eye muscle strabismus surgery between January 1, 2013, and December 31, 2017. Children undergoing initial surgeries involving 3 or more horizontal muscles, vertical muscles, or reoperations were excluded. MAIN OUTCOMES AND MEASURES: The primary outcome was the adjusted cumulative incidence of repeat horizontal muscle surgery within 5 years after the initial surgery. Reoperation risk was analyzed using adjusted hazard ratios (AHRs) derived from multivariable Cox regression models, adjusting for individual demographic and surgical factors (age, sex, race and ethnicity, US Census region, and surgeon subspecialty). Data were analyzed between January 16 and September 20, 2023. RESULTS: The study included 7482 children (median [IQR] age at initial surgery, 6 [4-9] years; 3945 females [53%]) with IXT treated with horizontal muscle strabismus surgery. Bilateral lateral rectus recession was performed more frequently than RR (85.3% vs 14.7%, P < .001), especially in younger children (rates of BLR vs RR by age: age 0 to ≤4 years, 88.4% vs 11.6%; age 5 to ≤11 years, 84.7% vs 15.3%; age 12 to ≤17 years, 78.1% vs 21.9%; P < 0.001). After data adjustment, the 5-year cumulative incidence of reoperation was 21.3% (95% CI, 20.1%-22.5%). The adjusted 5-year cumulative incidence of reoperation was higher for BLR than for RR (22.2% vs 17.2%; difference, 4.9%; 95% CI, 1.9%-8.0%). Unilateral lateral rectus recession with medial rectus resection was associated with a lower 5-year reoperation risk compared with BLR (AHR, 0.77; 95% CI, 0.64-0.93). Younger age at time of initial surgery was associated with a higher reoperation risk (AHR per 1-year decrease, 1.09; 95% CI, 1.07-1.11) after adjusting for all other covariates. CONCLUSIONS AND RELEVANCE: In this nationwide registry, approximately 1 in 5 children with IXT underwent reoperation within 5 years after the initial surgery. Children treated with RR were less likely to require a reoperation within 5 years compared with those treated with BLR. Further efforts to identify modifiable risk factors for reoperation are needed to reduce the surgical burden and improve outcomes for children with IXT.

PURPOSE: To longitudinally assess macular thickness and microvascular changes in children with sickle cell disease (SCD). DESIGN: A retrospective consecutive series. SUBJECTS: Children with SCD aged ≤ 18 years who had an ophthalmic examination at Boston Children's Hospital between January 1998 and August 2022. METHODS: Qualitative and quantitative analyses of both OCT and OCT angiography (OCTA) images were performed. MAIN OUTCOME MEASURES: Total retinal thickness measured on macular OCT, superficial capillary plexus and deep capillary plexus (DCP) vessel density (VD), and foveal avascular zone (FAZ) area measured on 6- × 6-mm OCTA scans. RESULTS: International Classification of Diseases, 10th Revision, code search identified 303 pediatric SCD patients who underwent ophthalmic examination during the study period. OCT and OCTA images were acquired on 104 (17.2%) and 60 (9.9%) eyes at presentation and on 159 (26.2%) and 100 (16.5%) eyes at final visit, respectively. Overall, temporal retinal thinning was noted qualitatively in 35.6% of SCD patients at presentation and 39.6% at final visit. Of those patients with macular thinning, 94.6% and 90.5% had peripheral sickle cell retinopathy (SCR) at presentation and final visit. On quantitative OCT analysis, HbSS eyes had a lower retinal thickness in the fovea and temporal parafovea compared with HbSC (P < 0.05). Eyes with peripheral SCR had a larger FAZ at presentation compared with eyes without peripheral SCR (P = 0.004), a lower DCP VD at final visit in the inferior temporal macula (P = 0.03), and a higher DCP VD at final visit in the superior nasal macula (P = 0.01). Eighty eyes of 40 patients had OCT, and 34 eyes of 20 patients had both OCT and OCTA images acquired at both initial and final visits. At final visit, retinal thickness decreased at the fovea, inferior perifovea, and temporal perifovea compared with presentation (P < 0.05). In parallel, VD DCP in the superonasal quadrant increased at final visit (P = 0.03). CONCLUSIONS: Macular retinal thinning was progressive and observed in eyes with and without peripheral SCR. Over time, there was a compensatory increase in DCP VD in the nasal macula on OCTA. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

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.

PURPOSE: To report a pediatric patient with bilateral temporal keratoconus. OBSERVATIONS: A 14-year-old male presented with a two-year history of progressively worsening visual acuity in both eyes and suspicion for undiagnosed amblyopia in the right eye. Retinoscopy revealed a scissoring reflex in both eyes and corneal topography demonstrated high keratometry values (Kmax 57.9 D and 46.1 D in the right and left eyes, respectively), with relative temporal steepening approximately coinciding with the thinnest pachymetry in both eyes. Corneal cross-linking was recommended. CONCLUSIONS AND IMPORTANCE: Keratoconus can present as a temporal variant with relative temporal steepening and thinning. It is important to maintain a high index of suspicion for keratoconus in pediatric patients with sub-normal visual acuities. Prompt assessment and diagnosis may prevent progression of keratoconus and development of amblyopia.

BACKGROUND: Intermittent exotropia (IXT) is one of the most common forms of strabismus usually seen in the pediatric age group, the prevalence of IXT is higher in Africa and the Middle East. IXT treatment strategies include both surgical and non-surgical methods, non-surgical management is preferred in general as it is less invasive and avoids the risks associated with surgery and anesthesia. AIMS: This study aims to determine the effectiveness of patching therapy for the treatment of IXT in different age groups and to compare the success of patching therapy in preventing surgery in IXT patients in different age groups. METHODOLOGY: A retrospective chart review was conducted from September 2022 until February 2023 at King Abdulaziz University Hospital in Riyadh. The data was collected retrospectively from electronic medical records from 2016 to 2021 of all patients diagnosed with IXT and were managed by patching therapy fitting the inclusion criteria. RESULTS: A total of 76 patients with IXT enrolled in the study with 56.5% of the participants were older than 7 years old. Overall, there was no improvement in the angle of deviation but 34% of patients had improved control over the follow-up period. 55.3% of the participants didn't require surgery. Younger age, longer duration of patching per month, and good compliance were significantly associated with treatment success. CONCLUSION: Younger age groups were more likely to benefit from patching therapy than older age groups, and good compliance to patching therapy is an important factor in preventing the need for surgery.

IMPORTANCE: Untreated refractive error contributes to the racial, ethnic, and socioeconomic disparities in visual function of adolescent children in the US. OBJECTIVE: To describe patterns in vision testing as a function of age among US adolescents and identify sociodemographic factors associated with vision testing. DESIGN, SETTING, AND PARTICIPANTS: This cross-sectional study used data from the National Survey of Children's Health (2018-2019), a nationally representative survey of the noninstitutionalized US pediatric population. A total of 24 752 adolescent children (aged 12 to <18 years) were included. Data were analyzed from March 22 to August 11, 2023. MAIN OUTCOMES AND MEASURES: The primary outcome was the caregiver report of vision testing within the last 12 months. Linear regression was used to describe the patterns in reported vision testing as a function of participant age. Logistic regression was used to describe the association of sociodemographic factors with the report of vision testing in each setting. RESULTS: Among 24 752 adolescents, the median (IQR) age was 14 (13-16) years; 12 918 (weighted, 51%) were male. Vision testing in any setting within the previous year was reported by caregivers of 18 621 adolescents (weighted, 74%). Vision testing was reported to have occurred at an eye clinic in 13 323 participants (weighted, 51%), at a primary care clinic in 5230 participants (weighted, 22%), at a school in 2594 participants (weighted, 11%), and at a health center in 635 participants (weighted, 4%). The percentage of adolescents reported to have vision tested decreased with age (-1.3% per year; 95% CI, -2.5% to 0% per year) due to a decrease in testing in primary care and school settings. After adjusting for age and sex, there were lower odds of vision testing reported for adolescents who were uninsured vs insured (adjusted odds ratio [AOR], 0.81; 95% CI, 0.76-0.87), had caregivers with less than vs greater than high school education (AOR, 0.89; 95% CI, 0.84-0.95), and were from a family born outside vs inside the US (AOR, 0.90; 95% CI, 0.82-0.98). CONCLUSIONS AND RELEVANCE: In this cross-sectional study, vision testing in adolescents decreased as a function of age due to fewer reported tests performed in primary care and school-based settings. Relative to children in socioeconomically advantaged families, those from disadvantaged families were less likely to report receiving vision testing in clinical settings. Efforts to expand the role of school-based vision testing for older adolescents from disadvantaged backgrounds may enable opportunities to address disparities in untreated refractive error.