Congenital Glaucoma Research Articles

A comparison of rebound and applanation tonometry in children with and without primary congenital glaucoma

Background: Intraocular pressure (IOP) measurement should be accurate in a paediatric population with primary congenital glaucoma (PCG).Aim: This study aims to investigate the difference between the change in IOP measurements using rebound tonometry (RBT) and handheld applanation tonometry (AT) (Perkins applanation tonometer [PAT]) in patients with and without PCG.Setting: Johannesburg, South Africa.Methods: Measurements were taken under anaesthesia, using RBT and AT at 0 min, 5 min and 10 mins after induction and prior to intubation. Corneal pachymetry and corneal diameters were measured.Results: Sixty-five children were included, 19 with PCG and 46 without PCG. The mean age (standard deviation [s.d.]) was 3.2 (2.25) years and 4.8 (2.4) years, respectively. The overall mean difference in IOP between RBT and PAT across both PCG and non-PCG groups was found to be 4.92 mmHg (95% confidence interval [CI]: 2.80 – 7.03) p 0.001, with RBT having higher readings. This difference was greater in the PCG group, with the IOP difference of 9.05 mmHg (95% CI: 2.6 – 15.5) p = 0.004. Mean corneal pachymetry (s.d.) was 585.6 (81.48) µm in the PCG group and 518.31 (39.9) µm in the non-PCG group. Univariate analysis showed that IOP was significantly related to corneal pachymetry, with a 11 mmHg increase in IOP for every 100 μm change in corneal thickness for measurements done with RBT (p 0.001), compared to 4 mmHg using PAT (p = 0.008).Conclusion: Intraocular pressure measurements done with RBT in children with and without PCG were overestimated compared to PAT.Contribution: This difference was more pronounced in PCG patients. In addition, IOP was significantly related to corneal thickness.

The Increased Burden of Rare Variants in Four Matrix Metalloproteinase-Related Genes in Childhood Glaucoma Suggests a Complex Genetic Inheritance of the Disease.

Childhood glaucoma encompasses congenital and juvenile primary glaucoma, which are heterogeneous, uncommon, and irreversible optic neuropathies leading to visual impairment with a poorly understood genetic basis. Our goal was to identify gene variants associated with these glaucoma types by assessing the mutational burden in 76 matrix metalloproteinase-related genes. We studied 101 childhood glaucoma patients with no identified monogenic alterations using next-generation sequencing. Gene expression was assessed through immunohistochemistry. Functional analysis of selected gene variants was conducted in cultured cells and in zebrafish. Patients presented a higher proportion of rare variants in four metalloproteinase-related genes, including CPAMD8 and ADAMTSL4, compared to controls. ADAMTSL4 protein expression was observed in the anterior segment of both the adult human and zebrafish larvae's eye, including tissues associated with glaucoma. In HEK-293T cells, expression of four ADAMTSL4 variants identified in this study showed that two variants (p.Arg774Trp and p.Arg98Trp) accumulated intracellularly, inducing endoplasmic reticulum stress. Additionally, overexpressing these ADAMTSL4 variants in zebrafish embryos confirmed partial loss-of-function effects for p.Ser719Leu and p.Arg1083His. Double heterozygous functional suppression of adamtsl4 and cpamd8 zebrafish orthologs resulted in reduced volume of both the anterior eye chamber and lens within the chamber, supporting a genetic interaction between these genes. Our findings suggest that accumulation of partial functional defects in matrix metalloproteinase-related genes may contribute to increased susceptibility to early-onset glaucoma and provide further evidence supporting the notion of a complex genetic inheritance pattern underlying the disease.

Updates on Pediatric Glaucoma: Medical and Surgical Interventions

Updates on Pediatric Glaucoma: Medical and Surgical Interventions

Spiroplasma infection as a cause of severe congenital keratouveitis, cataract and glaucoma

BackgroundOnly seven cases of ocular Spiroplasma infection have been reported to date, all presenting as congenital cataracts with concomitant intraocular inflammation. We describe the first case of Spiroplasma infection initially presenting as a corneal infiltrate.Case presentationA 1-month-old girl was referred for a corneal infiltrate in the left eye. She presented in our hospital with unilateral keratouveitis. Examination showed a stromal corneal infiltrate and dense white keratic precipitates in the left eye. Herpetic keratouveitis was suspected and intravenous acyclovir therapy was initiated. Two weeks later, the inflammation in the left eye persisted and was also noticed in the right eye. Acute angle-closure glaucoma and a cataract with dilated iris vessels extending onto the anterior lens capsule developed in the left eye. The inflammation resolved after treatment with azithromycin. Iridectomy, synechiolysis and lensectomy were performed. Bacterial metagenomic sequencing (16 S rRNA) and transmission electron microscopy revealed Spiroplasma ixodetis species in lens aspirates and biopsy. Consequently, a diagnosis of bilateral Spiroplasma uveitis was made.ConclusionsIn cases of congenital cataract with concomitant intraocular inflammation, Spiroplasma infection should be considered. The purpose of this case report is to raise awareness of congenital Spiroplasma infection as a cause of severe keratouveitis, cataract and angle-closure glaucoma in newborns. Performing molecular testing on lens aspirates is essential to confirm diagnosis. Systemic macrolides are suggested as the mainstay of treatment.

Increasing the diagnostic yield of childhood glaucoma cases recruited into the 100,000 Genomes Project

Childhood glaucoma (CG) encompasses a heterogeneous group of genetic eye disorders that is responsible for approximately 5% of childhood blindness worldwide. Understanding the molecular aetiology is key to improving diagnosis, prognosis and unlocking the potential for optimising clinical management. In this study, we investigated 86 CG cases from 78 unrelated families of diverse ethnic backgrounds, recruited into the Genomics England 100,000 Genomes Project (GE100KGP) rare disease cohort, to improve the genetic diagnostic yield. Using the Genomics England/Genomic Medicine Centres (GE/GMC) diagnostic pipeline, 13 unrelated families were solved (13/78, 17%). Further interrogation using an expanded gene panel yielded a molecular diagnosis in 7 more unrelated families (7/78, 9%). This analysis effectively raises the total number of solved CG families in the GE100KGP to 26% (20/78 families). Twenty-five percent (5/20) of the solved families had primary congenital glaucoma (PCG), while 75% (15/20) had secondary CG; 53% of this group had non-acquired ocular anomalies (including iris hypoplasia, megalocornea, ectopia pupillae, retinal dystrophy, and refractive errors) and 47% had non-acquired systemic diseases such as cardiac abnormalities, hearing impairment, and developmental delay. CYP1B1 was the most frequently implicated gene, accounting for 55% (11/20) of the solved families. We identified two novel likely pathogenic variants in the TEK gene, in addition to one novel pathogenic copy number variant (CNV) in FOXC1. Variants that passed undetected in the GE100KGP diagnostic pipeline were likely due to limitations of the tiering process, the use of smaller gene panels during analysis, and the prioritisation of coding SNVs and indels over larger structural variants, CNVs, and non-coding variants.

DDX58 variant triggers IFN-β-induced autophagy in trabecular meshwork and influences intraocular pressure.

Singleton-Merten syndrome (SMS) is a rare immunogenetic disorder affecting multiple systems, characterized by dental dysplasia, aortic calcification, glaucoma, skeletal abnormalities, and psoriasis. Glaucoma, a key feature of both classical and atypical SMS, remains poorly understood in terms of its molecular mechanism caused by DDX58 mutation. This study presented a novel DDX58 variant (c.1649A>C [p.Asp550Ala]) in a family with childhood glaucoma. Functional analysis showed that DDX58 variant caused an increase in IFN-stimulated gene expression and high IFN-β-based type-I IFN. As the trabecular meshwork (TM) is responsible for controlling intraocular pressure (IOP), we examine the effect of IFN-β on TM cells. Our study is the first to demonstrate that IFN-β significantly reduced TM cell viability and function by activating autophagy. In addition, anterior chamber injection of IFN-β remarkably increased IOP level in mice, which can be attenuated by treatments with autophagy inhibitor chloroquine. To uncover the specific mechanism underlying IFN-β-induced autophagy in TM cells, we performed microarray analysis in IFN-β-treated and DDX58 p.Asp550Ala TM cells. It showed that RSAD2 is necessary for IFN-β-induced autophagy. Knockdown of RSAD2 by siRNA significantly decreased autophagy flux induced by IFN-β. Our findings suggest that DDX58 mutation leads to the overproduction of IFN-β, which elevates IOP by modulating autophagy through RSAD2 inTM cells.

Choroidal thickness in juvenile open angle glaucoma: insights from a south asian case-control study.

The purpose of this study is to compare choroidal thickness in juvenile open angle glaucoma (JOAG) and healthy controls using spectral domain optical coherence tomography (SD-OCT) and study its correlations. In this case-control study, 56 eyes of 28 JOAG patients and an equal number of controls were recruited. SD-OCT was used to measure the choroidal thickness (ChT), in the macular region at 5 locations: subfoveal, 1500µm and 3000µm nasal and temporal to the foveal center, and in the peripapillary region at 6 locations: up to 1500µm, nasal and temporal to the disc, respectively. The ChT and its correlations with age, intraocular pressure, cup-to-disc ratio, central corneal thickness, mean deviation, and axial length were studied. The average macular ChT in JOAG was 306.30 ± 56.49µm vs. 277.12 ± 64.68µm in controls. The average peripapillary ChT in JOAG was 197.79 ± 44.05µm vs. 187.24 ± 38.89µm in controls. The average total ChT (p = 0.042), the average macular ChT (p = 0.022), the subfoveal ChT (p = 0.022), the ChT 1500µm (p < 0.001), and 3000µm temporal to the fovea (p = 0.002) were significantly thicker in the JOAG group. In the JOAG group, the average macular ChT had a significant negative correlation with age, whereas axial length was positively correlated with the average peripapillary ChT. In this South Asian cohort of JOAG, the average total ChT, average macular ChT, subfoveal ChT, and ChT at 1500µm, and 3000µm temporal to the fovea were significantly thicker when compared to healthy controls.

Research progress in the diagnosis and treatment of childhood glaucoma

Childhood glaucoma is a disease that seriously endangers children's visual health. It will accompany the patients throughout their lives and bring a heavy burden to families and society. Most childhood blindness caused by glaucoma is preventable or treatable. Relevant research has made progress in recent years. Based on the new consensus reached by the World Glaucoma Association and the latest medical evidence at home and abroad, this article summarizes the definition, classification, diagnosis, molecular genetics, pathogenesis and comprehensive treatments including drugs and surgery of childhood glaucoma, with a focus on the application of various surgical methods, so as to provide reference for clinical and scientific research and improve the clinical diagnosis and treatment of childhood glaucoma.

Clinical value of cognitive behavioral nursing model to patients with congenital glaucoma.

There have been studies on the use of cognitive behavioral nursing intervention (CBNI) but the main subjects were patients with secondary glaucoma and there are fewer studies on the care of congenital glaucoma. To explore the clinical value of cognitive behavioral nursing model in patients with congenital glaucoma. One hundred and fifty-three postoperative patients with congenital glaucoma treated in our hospital between January 2021 and December 2022 were prospectively selected for the study and randomly divided into a control group (n= 77) and an observation group (n= 76). The control group was given routine nursing, and the observation group was given cognitive behavioral nursing mode on the basis of the control group. Anxiety self-assessment scale (SAS), depression self-assessment scale (SDS), Connor-Davidson toughness scale, and treatment adherence evaluation scale were used to evaluate the psychological state, mental toughness, treatment adherence, treatment effect and nursing care satisfaction in the two groups before and after 2 weeks of intervention. The efficacy of the treatment was also assessed by determining the visual acuity (VA), intraocular pressure (IOP), and mean defective (MD) value of the visual field of the two groups of patients. After nursing, the SDS score (46.33 ± 6.16 versus 53.21 ± 5.94) and SAS score (44.41 ± 5.6 versus 52.82 ± 6.31) in the observation group were lower than those in the control group (P< 0.05). The scores of optimism (11.55 ± 1.90 versus 8.20 ± 1.95), self-improvement (22.05 ± 3.60 versus 17.60 ± 4.30), tenacity (37.45 ± 3.10 versus 28.90 ± 4.55) and total score (71.35 ± 8.00 versus 56.85 ± 8.50) in the observation group were higher than those in the control group (P< 0.05). After care, the VA of the observation group (0.95 ± 0.22) was greater than that of the control group (0.84 ± 0.16), and the IOP (14.25 ± 0.58 versus 15.89 ± 0.67) and the MD (5.42 ± 0.46 versus 6.68 ± 0.49) of the observation group were less than that of the control group. The difference between the two groups was statistically significant (P< 0.05). The compliance (96.05% versus 85.71%) and nursing satisfaction (96.10% versus 85.71%) of the observation group were higher than those of the control group (P< 0.05). Cognitive-behavioural nursing care for glaucoma patients can improve patients' mental toughness, improve visual acuity, reduce intraocular pressure and mean visual field defect values, and have a positive effect on enhancing patients' treatment adherence and nursing satisfaction.

Outcomes of a Second Ahmed Glaucoma Implant With Mitomycin-C in Pediatric Glaucoma After Initial Valve Failure.

After the failure of a glaucoma drainage device in children, implantation of a second Ahmed glaucoma valve with mitomycin-C allows a significant reduction of intraocular pressure and number of medications with good medium-term and long-term survival. The effectiveness of glaucoma drainage devices (GDD) is limited in time. There is little literature regarding the optimal management strategy after failure of a GDD in pediatric glaucoma. To report the outcomes of Ahmed glaucoma valve implantation (AGV) with mitomycin-C (MMC) after failure of a GDD in children. Retrospective chart review of patients with a history of at least 1 GDD receiving an AGV implantation with MMC between 2000 and 2019. We defined complete success as an IOP of 5-21mmHg without glaucoma medication and qualified success as a final IOP of 5-21mmHg with one or more glaucoma medication, without loss of vision. Twenty-one patients (22 eyes) met the inclusion criteria. The intraocular pressure and number of medications were significantly reduced. The probability of complete success was 47% [95% CI: 29%-75%] at 2 years and 34% [95% CI: 18%-65%] at 4 years. The probability of qualified success was 74% [95% CI: 56%-97%] at 2 years, and 54% [95% CI: 34%-84%] at 5 years. Failure happened in 10 eyes after a mean time of 4.3 years ±3.6 (6mo to 15y), 5 of which (23%) for severe complications or loss of vision. This study of implantation of a second valve with MMC reports a significant decrease in IOP and medications with medium-term and long-term success rates close to those reported for first valve implantation, although with a high risk of complications.

Increased late-onset glaucoma risk following vitrectomy for macular pucker or hole.

The long-term risk of developing glaucoma after vitrectomy remains uncertain. This retrospective population-based cohort study aimed to explore this risk following vitrectomy for macular pucker or hole. Utilizing Taiwan's National Health Insurance Research Database (NHIRD), we included patients who were older than 18 years and had undergone vitrectomy surgery between 2011 and 2019. Exclusions were made for patients with prior diagnoses of glaucoma, congenital or secondary glaucoma, as well as those who had received previous vitreoretinal treatments or had undergone multiple vitrectomies. After an average follow-up period of 51 and 53 months respectively for the vitrectomized and non-vitrectomized group, our results showed a relative risk of 1.71 for glaucoma development in the vitrectomized group. Higher adjusted hazard ratios were also observed for open-angle glaucoma and normal tension glaucoma. Increased risks were associated with male sex, obstructive sleep apnoea, and migraine. In the subgroup analysis, phakic eyes at baseline and those who had undergone cataract surgery post-vitrectomy were associated with a lower risk of glaucoma development during follow-up. Among all glaucoma events, pseudophakic status at baseline had the shortest interval to glaucoma development following vitrectomy. These findings underscore the potential relationship between vitrectomy and glaucoma onset, emphasizing the need for vigilant monitoring and early detection of glaucoma in post-vitrectomy patients.

Long-term efficacy and safety of posterior endoscopic cyclophotocoagulation in refractory glaucoma: A 5-year follow-up study at a tertiary eye center

PurposeTo evaluate the long-term efficacy of endoscopic cyclophotocoagulation (ECP) via a pars plana approach in a large cohort of refractory glaucoma patients DesignSingle-center, retrospective, longitudinal, cohort study. MethodsThis study recruited patients who underwent ECP and consecutively visited and were followed up for at least 5 years at Beijing Tongren Eye Center, China from January 2013 to December 2017. All patients underwent a complete ophthalmic examination. Treatment success was defined as 6 mmHg ≤ IOP ≤ 21 mmHg with or without anti-glaucoma medications. ResultsA total of 121 eyes of 105 patients including 51 children and 54 adults were enrolled. The mean follow-up was 7.2 ± 1.3 years. The most common glaucoma diagnoses were secondary glaucoma (74 eyes, 61.1 %) and primary congenital glaucoma (19 eyes 15.7 %). The mean extent of the first ECP was 259 degrees. There was an overall decrease in IOP of 38.3 % from 33.3 ± 9.0 mmHg preoperatively to 20.5 ± 7.5 mmHg after surgery, which was statistically significant (P < 0.001). The success rate after 1 or more ECP surgery was 65.3 %. After adjusting for sex, number of prior TCP surgeries and the extent of ECP degree, the failure of ECP was associated with being children (as compared with adults; P = 0.028; OR = 2.549) and higher preoperative IOP (P = 0.001; OR = 1.084). ConclusionECP is an effective procedure for lowing IOP in refractory glaucoma, particularly in patients who are also candidates for vitreoretinal interventions. Hence, a collaborative approach between glaucoma and retinal specialists is of utmost importance in devising an optimal management strategy for glaucoma treatment.

Comparative efficacies of 13 surgical interventions for primary congenital glaucoma in children: a network meta-analysis of randomized clinical trials.

Comparative efficacies of 13 surgical interventions for primary congenital glaucoma in children: a network meta-analysis of randomized clinical trials.

Evaluating neural crest cell migration in a Col4a1 mutant mouse model of ocular anterior segment dysgenesis

The periocular mesenchyme (POM) is a transient migratory embryonic tissue derived from neural crest cells (NCCs) and paraxial mesoderm that gives rise to most of the structures in front of the eye. Morphogenetic defects of these structures can impair aqueous humor outflow, leading to elevated intraocular pressure and glaucoma. Mutations in collagen type IV alpha 1 (COL4A1) and alpha 2 (COL4A2) cause Gould syndrome – a multisystem disorder often characterized by variable cerebrovascular, ocular, renal, and neuromuscular manifestations. Approximately one-third of individuals with COL4A1 and COL4A2 mutations have ocular anterior segment dysgenesis (ASD), including congenital glaucoma resulting from abnormalities of POM-derived structures. POM differentiation has been a major focus of ASD research, but the underlying cellular mechanisms are still unclear. Moreover, earlier events including NCC migration and survival defects have been implicated in ASD; however, their roles are not as well understood. Vascular defects are among the most common consequences of COL4A1 and COL4A2 mutations and can influence NCC survival and migration. We therefore hypothesized that NCC migration might be impaired by COL4A1 and COL4A2 mutations. In this study, we used 3D confocal microscopy, gross morphology, and quantitative analyses to test NCC migration in Col4a1 mutant mice. We show that homozygous Col4a1 mutant embryos have severe embryonic growth retardation and lethality, and we identified a potential maternal effect on embryo development. Cerebrovascular defects in heterozygous Col4a1 mutant embryos were present as early as E9.0, showing abnormal cerebral vasculature plexus remodeling compared to controls. We detected abnormal NCC migration within the diencephalic stream and the POM in heterozygous Col4a1 mutants whereby mutant NCCs formed smaller diencephalic migratory streams and POMs. In these settings, migratory NCCs within the diencephalic stream and POM localize farther away from the developing vasculature. Our results show for the first time that Col4a1 mutations lead to cranial NCCs migratory defects in the context of early onset defective angiogenesis without affecting cell numbers, possibly impacting the relation between NCCs and the blood vessels during ASD development.

Capturing a child with congenital glaucoma

Congenital glaucoma, though rare, is a sight threatening condition which can cause lifelong impact to a child’s life. It often presents at birth, arises from abnormal development of the eye’s drainage system, leading to increased pressure within the eye. Without timely intervention, it can result in irreversible vision loss.

Selective laser trabeculoplasty in the treatment of juvenile open angle glaucoma patients under 25years of age.

To retrospectively evaluate the effectiveness and reliability of selective laser trabeculoplasty (SLT) treatment in juvenile open angle glaucoma (JOAG) cases. The 46 eyes of 28 cases that had undergone SLT for JOAG and had a follow-up of at least one month were included in the study. The pre-SLT, month 1, month 6, year 1, year 2, and final follow-up intraocular pressure (IOP); the number of drugs before and after the SLT procedure; and the complications caused by the SLT were recorded. An IOP below 21mmHg without any additional medication or glaucoma surgery and an IOP decrease of 20% or more compared to the baseline were considered successful. There were 18 females and 10 males with a mean age of 17.52 ± 2.7 (13-22) years. The cases were followed-up for a mean duration of 39 (5-59) months. The mean IOP was 23.93 ± 0.52mmHg before SLT and decreased to 17.15 ± 0.57mmHg at month 1 after the procedure, 17.17 ± 0.52mmHg at month 6, 18.02 ± 0.77mmHg at year 1, 18.12 ± 0.48mmHg at year 2, and 20.93 ± 0.69mmHg at the final follow-up. The post-SLT IOP measurements at all times were found to be significantly lower than the pre-SLT values (p < 0.001). The mean number of drugs was 1.5 before the SLT and 0.59 afterwards (p < 0.001). The most common complications after SLT were anterior chamber inflammation in 29 cases (63.04%), hyperemia in 20 (43.40%) cases (4.34%), an early period IOP increase of 5mmHg or more in 11 cases (23.91%), and mild ocular pain in 2 cases (4.34%). SLT is an effective and reliable method for JOAG treatment.

Macular and peripapillary vascular parameters in the fellow eyes of unilateral primary congenital glaucoma: a comparative study

PurposeTo study the fellow eyes of patients with unilateral primary congenital glaucoma (PCG) using optical coherence tomography angiography (OCTA) and compare them to normal age- and refractive error–matched healthy controls. MethodsUsing OCTA, the foveal avascular zone (FAZ) area, cup:disk ratio, vessel density (VD) of the optic nerve head (ONH) and peripapillary area and the macular VD in superficial (SCP) and deep vascular complexes in both 3 mm and 6 mm scans of both groups were compared. Clinical data included best-corrected visual acuity (BCVA), cycloplegic refraction, intraocular pressure (IOP), anterior and posterior segments examination findings, including ONH cup:disk ratio. ResultsA total of 48 eyes of 48 children (24 eyes in each group) were included. There was no difference in the mean RNFL thickness, cup:disk ratio, baseline visual acuity, or spherical equivalent between groups (P > 0.05). In the 3 mm macular scan, the VD of the SCP at the fovea was significantly higher in the PCG group compared to controls (P = 0.04). In the ONH scans, there was a significantly reduced inside the disk VD in the PCG group compared to controls (P = 0.03). There was no significant difference in other macular and ONH vascular parameters between groups (P > 0.05). ConclusionsIn our study cohort, there was no difference in most of the macular and ONH vascular parameters between groups. However, the fellow eyes of PCG patients exhibited higher VD of the SCP at the fovea and reduced inside the disk VD compared with control eyes.

Chromatic vision and structural assessment in primary congenital glaucoma

Primary congenital glaucoma is a rare disease that occurs in early birth and can lead to low vision. Evaluating affected children is challenging and there is a lack of studies regarding color vision in pediatric glaucoma patients. This cross-sectional study included 21 eyes of 13 children with primary congenital glaucoma who were assessed using the Farnsworth D-15 test to evaluate color vision discrimination and by spectral domain optical coherence tomography to measure retinal fiber layer thickness. Age, visual acuity, cup-to-disc ratio and spherical equivalent data were also collected. Global and sectional circumpapillary and macular retinal fiber layer thicknesses were measured and compared based on color vision test performance. Four eyes (19%) failed the color vision test with diffuse dyschromatopsia patterns. Only age showed statistical significance in color vision test performance. Global and sectional circumpapillary and macular retinal fiber layer thicknesses were similar between the color test outcomes dyschromatopsia and normal. While the color vision test could play a role in assessing children with primary congenital glaucoma, further studies are needed to correlate it with damage to retinal fiber layer thickness.

Using Large Language Models to Generate Educational Materials on Childhood Glaucoma

PurposeTo evaluate the quality, readability, and accuracy of large language model (LLM) generated patient education materials (PEMs) on childhood glaucoma, and their ability to improve existing online information's readability. DesignCross-sectional comparative study. MethodsWe evaluated responses of ChatGPT-3.5, ChatGPT-4, and Bard to three separate prompts requesting they write PEMs on “childhood glaucoma.” Prompt A required PEMs be “easily understandable by the average American.” Prompt B required PEMs be written “at a 6th-grade level using Simple Measure of Gobbledygook (SMOG) readability formula.” We then compared responses’ quality (DISCERN questionnaire, Patient Education Materials Assessment Tool (PEMAT)), readability (SMOG, Flesch–Kincaid Grading Level (FKGL)), and accuracy (Likert Misinformation scale). To assess the improvement of readability for existing online information, Prompt C requested LLM rewrite 20 resources from a Google search of keyword “childhood glaucoma” to the American Medical Association-recommended “6th-grade level.” Rewrites were compared on key metrics such as readability, complex words (≥3 syllables), and sentence count. ResultsAll 3 LLM generated PEMs that were of high quality, understandability, and accuracy (DISCERN≥4, ≥70% PEMAT understandability, Misinformation score=1). Prompt B responses were more readable than Prompt A responses for all 3 LLM (p≤0.001). ChatGPT-4 generated the most readable PEMs compared to ChatGPT-3.5 and Bard (p≤0.001). Although Prompt C responses showed consistent reduction of mean SMOG and FKGL scores, only ChatGPT-4 achieved the specified 6th-grade reading level (4.8 ± 0.8 and 3.7 ± 1.9, respectively). ConclusionLLMs can serve as strong supplementary tools in generating high quality, accurate, and novel PEMs, and improving the readability of existing PEMs on childhood glaucoma.

Factors Associated With Visual Field Testing Reliability in Children With Glaucoma or Suspected Glaucoma

To evaluate Humphrey Visual Field (HVF) test reliability and its associated risk factors in children with glaucoma or glaucoma suspect. Retrospective cohort study. None. Single-center childhood glaucoma clinic. One hundred thirty-six patients aged ≤18 years with glaucoma/glaucoma suspect, and least 1 completed 24 to 2 HVF test between 2018 and 2023. Demographic and clinical characteristics including age, primary language, visual acuity (VA), and glaucoma diagnosis were extracted from electronic health records. HVF 24 to 2 testing metrics, including FP, FN, and FL. Tests were defined as reliable using manufacturer guidelines of ≤33% FP, ≤33% FN, and ≤20% FL. For each patient, a reliability score was calculated as the percentage of reliable tests among all tests completed. A multivariable logistic regression model was used to determine factors associated with test-level reliability (yes/no). A multivariable linear regression model was used to determine factors associated with patient-level reliability score. Among 634 HVFs from 136 patients (Mean ± SD age at first test 12.0 ± 3.2 years, 47.8% female), 51.3% were reliable. Older age, better baseline VA, and English as primary language were associated with greater odds of test-level reliability (P < .04). Mean ± SD patient-level reliability score was 51.7 ± 38.1%. Older age at first clinic visit, better baseline VA, and English as primary language were associated with higher reliability scores (all P < .02), and number of prior VF tests was not (P = .56). Younger age, worse visual acuity, and non-English as primary language were associated with decreased reliability and should be considered when interpreting VF testing in children. A significant learning effect was not observed with repeated testing.