Unilateral Glaucoma Without Facial Angioma in a Pediatric Patient: A Suspected Sturge-Weber Syndrome Variant.

The authors report a case of a 5-month-old full-term infant with chronic conjunctival redness and elevated intraocular pressure in the right eye. Magnetic resonance imaging ruled out leptomeningeal angiomatosis. Despite lacking a typical port-wine mark, the diagnosis of Sturge-Weber syndrome was established based on specific observations in the right eye that involved unilateral vascular glaucoma. These findings included Haab striae, a larger axial length measurement, an increased number of episcleral vessels with blood in Schlemm canal, and a thicker choroid in the macular region. This is the first reported pediatric case with these specific ocular manifestations in the absence of facial angioma, highlighting the need for awareness among clinicians to avoid misdiagnosis and facilitate proper management. [J Pediatr Ophthalmol Strabismus. 2024;61(3):e19-e22.].

Identification of pathogenic genetic variants in patients with acquired early-onset bilateral cataracts using next-generation sequencing.

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_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.

Performance of VisuALL virtual reality visual field testing in healthy children.

BACKGROUND: Virtual reality field testing may provide an alternative to standard automated perimetry. This study evaluates a virtual reality game-based automated perimetry in a healthy pediatric population. METHODS: A prospective series of pediatric patients at one institution who performed VisuALL perimetry (Olleyes Inc, Summit, NJ) using a game-based algorithm. Participants were examined by an experienced pediatric optometrist or ophthalmologist, who confirmed that there was no evidence of ocular disease expected to affect visual fields. Testing was performed binocularly, with the child wearing their spectacle correction in place. Age, refractive error, test duration, false positives, and stereoacuity were evaluated for associations with performance on VisuALL, as defined by mean deviation (MD) and pattern standard deviation (PSD). RESULTS: A total of 191 eyes of 97 patients (54% female) were included, with a mean age of 11.9 ± 3.1 years. The average MD was -1.82 ± 3.5 dB, with a mean foveal sensitivity of 32.0 ± 4.7 dB. Fifty-nine eyes (30.9%) had MD < -2 dB. Better performance, as assessed by MD and PSD, was associated with shorter test duration (P < 0.001) and older age (P < 0.001). False positives (P = 0.442), wearing spectacles (P = 0.092), Titmus stereoacuity (P = 0.197), and refractive error (P = 0.120) did not appear to be associated with improved performance, adjusting for age as a covariate. CONCLUSIONS: VisuALL virtual reality field testing was well tolerated in this pediatric study cohort. Older age and shorter test duration were associated with better performance on field testing.

A pilot optical coherence tomography angiography classification of retinal neovascularization in retinopathy of prematurity.

Extraretinal neovascularization is a hallmark of treatment-requiring retinopathy of prematurity (ROP). Optical coherence tomography angiography (OCTA) offers vascular flow and depth information not available from indirect ophthalmoscopy and structural OCT, but OCTA is only commercially available as a tabletop device. In this study, we used an investigational handheld OCTA device to study the vascular flow in and around retinal neovascularization in seven preterm infants with treatment-requiring ROP and contrasted them to images of vascular flow in six infants of similar age without neovascular ROP. We showed stages of retinal neovascularization visible in preterm infants from 32 to 47 weeks postmenstrual age: Intraretinal neovascularization did not break through the internal limiting membrane; Subclinical neovascular buds arose from retinal vasculature with active flow through the internal limiting membrane; Flat neovascularization in aggressive ROP assumed a low-lying configuration compared to elevated extraretinal neovascular plaques; Regressed neovascularization following treatment exhibited decreased vascular flow within the preretinal tissue, but flow persisted in segments of retinal vessels elevated from their original intraretinal location. These findings enable a pilot classification of retinal neovascularization in eyes with ROP using OCTA, and may be helpful in detailed monitoring of disease progression, treatment response and predicting reactivation.

Computer versus human-expert ranking of posterior pole vascular tortuosity and dilation using retinal vessel maps generated from bedside optical coherence tomography: a proof-of-concept study.

Semiautomated computer software (ie, ROPtool) can trace and analyze optical coherence tomography (OCT)-generated retinal vessel maps for plus/pre-plus disease with high reliability and accuracy. This proof-of-concept study found that ROPtool can reliably rank OCT-generated vessel maps for tortuosity and combined tortuosity/dilation, which correlated well with human-expert rankings and clinical examination.

Refractive Accuracy and Visual Outcome by Self-Refraction Using Adjustable-Focus Spectacles in Young Children: A Randomized Clinical Trial.

Importance: Uncorrected refractive error is the most common cause of vision impairment in children. Most children 12 years or older can achieve visual acuity (VA) of 20/25 or better by self-refraction using adjustable-focus spectacles, but data on younger children are lacking. Objective: To assess refractive accuracy, corrected VA, and factors associated with not achieving VA of 20/25 or better among children aged 5 to 11 years performing self-refraction with Adspecs adjustable-focus spectacles (Adaptive Eyecare), compared with noncycloplegic autorefraction and cycloplegic refraction. Design, Setting, and Participants: This was a cross-sectional noninferiority trial conducted from September 2, 2015, to December 14, 2017. The study setting was an academic pediatric eye clinic. Children aged 5 to 11 years with uncorrected VA of 20/40 or worse in 1 or both eyes and without systemic or ocular conditions preventing best-corrected VA of 20/25 or better were enrolled. Children who had best-corrected VA worse than 20/25 were excluded. Study data were analyzed from September 2017 to June 2023. Exposures: Children were taught to self-refract with adjustable-focus spectacles. Main Outcomes and Measures: Spherical equivalent refractive error (using self-refraction, noncycloplegic autorefraction, and cycloplegic refraction) and VA (uncorrected and using self-refraction, noncycloplegic autorefraction, and cycloplegic refraction) for study eyes were evaluated. Potential predictors of failure to achieve VA of 20/25 or better with self-refraction were assessed using logistic regression. Results: A total of 127 consecutive children were enrolled. After exclusions, 112 children (median [IQR] age, 9.0 [8.0-10.3] years; 52 boys [46.4%]) were included in the study. Mean (SD) spherical equivalent refractive power was -2.00 (1.52) diopters (D) for self-refraction, -2.32 (1.43) D for noncycloplegic autorefraction, and -1.67 (1.49) D for cycloplegic refraction. Mean (SD) difference in refractive power between self-refraction and noncycloplegic autorefraction was 0.32 (1.11) D (97.5% 1-sided CI, 0.11 to ∞ D; P < .001) and between self-refraction and cycloplegic refraction was -0.33 (1.15) D (97.5% 1-sided CI, -0.54 to ∞ D; P = .77). The proportion of children with corrected VA of 20/25 or better was 79.5% (89 of 112) with self-refraction, 85.7% (96 of 112) with noncycloplegic autorefraction, and 79.5% (89 of 112) with cycloplegic refraction (self-refraction vs noncycloplegic autorefraction: McNemar P value = .27; self-refraction vs cycloplegic refraction: McNemar P value > .99). Those failing to achieve best-corrected VA of 20/25 or better with self-refraction had higher astigmatism (odds ratio [OR], 10.6; 95% CI, 3.1-36.4; P < .001) and younger age (OR, 1.5; 95% CI, 1.1-2.2; P = .02). Conclusions and Relevance: Self-refraction among children aged 5 to 11 years may result in more myopic power than cycloplegic refraction but not necessarily to a clinically relevant degree. Although the proportion of children achieving VA of 20/25 or better with self-refraction using adjustable-focus spectacles did not differ from cycloplegic refraction, it was less likely among younger children and those with higher astigmatism.

Determining relative thickness of inner retinal layers on single-line foveal optical coherence tomography (OCT) in healthy pediatric eyes-normative data for handheld OCT.

BACKGROUND: Handheld optical coherence tomography (HH-OCT) lacks integrated segmentation/analysis software. Optic neuropathies cause ganglion cell layer (GCL) thinning, with normal to thickened inner nuclear layer (INL), suggesting the potential value of estimating the GCL/INL ratio on single-line foveal HH-OCT scans. This study determines this ratio in macular scans from healthy eyes of young children using HH-OCT and overhead-mounted OCT. METHODS: Macular OCT scans were obtained using either HH-OCT or overhead-mounted Spectralis FLEX-OCT in children ages 0-5 years undergoing clinically indicated anesthesia/sedation. Exclusion criteria included gestational age <37 weeks, neurologic disease, amblyopia, ocular disease, or large refractive error (spherical equivalent beyond -3.00 D to +8.00 D). For HH-OCT, the GCL and INL were manually measured in pixels from single-line macular scans at the thickest point nasal and temporal to the fovea. For FLEX-OCT images, measurements were aided by automated software. RESULTS: HH-OCT was obtained on 38 eyes (38 children, mean age 2.4 ± 1.8 years) and FLEX-OCT on 56 eyes (56 children, mean age 2.3 ± 1.5 years). Mean nasal GCL/INL was 1.24 ± 0.18 (min/max = 0.92/1.75) for HH-OCT and 1.29 ± 0.18 (min/max = 0.96/1.66) for FLEX-OCT (P = 0.11). Mean temporal GCL/INL was 1.22 ± 0.24 (min/max = 0.66/1.70) for HH-OCT and 1.19 ± 0.16 (min/max = 0.86/1.47) for FLEX-OCT (P = 0.47). CONCLUSIONS: Mean normative GCL/INL ratios were approximately 1.2 at the thickest macular areas both nasal and temporal to the fovea, with either HH-OCT or FLEX-OCT in young children's eyes. These values may prove useful when HH-OCT is used to assess optic neuropathies, in which the GCL/INL ratio is expected to be decreased.

Ocular Pigmentation Impact on Retinal Versus Choroidal Optical Coherence Tomography Imaging in Preterm Infants.

Purpose: To evaluate the association of fundus pigmentation with the visibility of retinal versus choroidal layers on optical coherence tomography (OCT) in preterm infants. Methods: For infants enrolled in BabySTEPS, ophthalmologists recorded fundus pigmentation (blond, medium, or dark) at the first retinopathy of prematurity (ROP) examination. Bedside OCT imaging was performed at each examination, and a masked grader evaluated all OCT scans from both eyes of each infant for visibility (yes/no) of all retinal layers and of the chorio-scleral junction (CSJ). Multivariable logistic regression was used to assess associations between fundus pigmentation and visibility of all retinal layers and CSJ, controlling for potential confounders (i.e., birth weight, gestational age, sex, OCT system, pupil size, and postmenstrual age at imaging). Results: Among 114 infants (mean birth weight, 943 grams; mean gestational age, 27.6 weeks), 43 infants (38%) had blond, 56 infants (49%) had medium, and 15 infants (13%) had dark fundus pigmentation. Of 1042 scans, all retinal layers were visible in 977 (94%) and CSJ in 895 (86%). Pigmentation was not associated with retinal layer visibility (P = 0.49), but medium and dark pigmentation were associated with decreased CSJ visibility (medium: odds ratio [OR] = 0.34, P = 0.001; dark: OR = 0.24, P = 0.009). For infants with dark pigmentation, retinal layer visibility increased (OR = 1.87 per week; P ≤ 0.001) and CSJ visibility decreased (OR = 0.78 per week; P = 0.01) with increasing age. Conclusions: Although fundus pigmentation was not associated with the visibility of all retinal layers on OCT, darker pigmentation decreased CSJ visibility, and this effect increased with age. Translational Relevance: The ability of bedside OCT to capture retinal layer microanatomy in preterm infants, regardless of fundus pigmentation, may represent an advantage over fundus photography for ROP telemedicine.

Prevalence of cerebrotendinous xanthomatosis among patients diagnosed with early-onset idiopathic bilateral cataracts: final analysis.

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.

The effect of sex on retinopathy of prematurity severity among premature mixed-sex multiple gestation infants.

This retrospective study of 68 premature infants examined whether there was a difference between male and female mixed-sex multiple gestation infants with regard to stage of retinopathy of prematurity (ROP) developed or need for ROP treatment. We found that among mixed-sex twin infants there was no statistically significant difference between sexes in most severe ROP stage developed or need for ROP treatment, but males were treated at an earlier postmenstrual age (PMA) than females, despite females having a lower mean birthweight and slower mean growth velocity compared to males.

Incidence of Glaucoma-Related Adverse Events in the First 5 Years After Pediatric Lensectomy.

Importance: Glaucoma can develop following cataract removal in children. Objective: To assess the cumulative incidence of glaucoma-related adverse events (defined as glaucoma or glaucoma suspect) and factors associated with risk of these adverse events in the first 5 years after lensectomy prior to 13 years of age. Design, Setting, and Participants: This cohort study used longitudinal registry data collected at enrollment and annually for 5 years from 45 institutional and 16 community sites. Participants were children aged 12 years or younger with at least 1 office visit after lensectomy from June 2012 to July 2015. Data were analyzed from February through December 2022. Exposures: Usual clinical care after lensectomy. Main Outcomes and Measures: The main outcomes were cumulative incidence of glaucoma-related adverse events and baseline factors associated with risk of these adverse events. Results: The study included 810 children (1049 eyes); 443 eyes of 321 children (55% female; mean [SD] age, 0.89 [1.97] years) were aphakic after lensectomy, and 606 eyes of 489 children (53% male; mean [SD] age, 5.65 [3.32] years) were pseudophakic. The 5-year cumulative incidence of glaucoma-related adverse events was 29% (95% CI, 25%-34%) in 443 eyes with aphakia and 7% (95% CI, 5%-9%) in 606 eyes with pseudophakia; 7% (95% CI, 5%-10%) of aphakic eyes and 3% (95% CI, 2%-5%) of pseudophakic eyes were diagnosed as glaucoma suspect. Among aphakic eyes, a higher risk for glaucoma-related adverse events was associated with 4 of 8 factors, including age less than 3 months (vs ≥3 months: adjusted hazard ratio [aHR], 2.88; 99% CI, 1.57-5.23), abnormal anterior segment (vs normal: aHR, 2.88; 99% CI, 1.56-5.30), intraoperative complications at time of lensectomy (vs none; aHR, 2.25; 99% CI, 1.04-4.87), and bilaterality (vs unilaterality: aHR, 1.88; 99% CI, 1.02-3.48). Neither of the 2 factors evaluated for pseudophakic eyes, laterality and anterior vitrectomy, were associated with risk of glaucoma-related adverse events. Conclusions and Relevance: In this cohort study, glaucoma-related adverse events were common after cataract surgery in children; age less than 3 months at surgery was associated with elevated risk of the adverse events in aphakic eyes. Children with pseudophakia, who were older at surgery, less frequently developed a glaucoma-related adverse event within 5 years of lensectomy. The findings suggest that ongoing monitoring for the development of glaucoma is needed after lensectomy at any age.

Ocular and developmental outcomes of a dosing study of bevacizumab for retinopathy of prematurity.

PURPOSE: To report 2-year ocular and developmental outcomes for infants receiving low doses of intravitreal bevacizumab for type 1 retinopathy of prematurity (ROP). METHODS: A total of 120 premature infants (mean birthweight, 687 g; mean gestational age, 24.8 weeks) with type 1 ROP were enrolled in a multicenter, phase 1 dose de-escalation study. One eye per infant received 0.25 mg, 0.125 mg, 0.063 mg, 0.031 mg, 0.016 mg, 0.008 mg, 0.004 mg, or 0.002 mg of intravitreal bevacizumab; fellow eyes when treated received one dosage level higher. At 2 years, 70 of 120 children (58%) underwent ocular examinations; 51 (43%) were assessed using the Bayley Scale of Infant and Toddler Development. RESULTS: Correlation coefficients for the association of total dosage of bevacizumab with Bayley subscales were -0.20 for cognitive (95% CI, -0.45 to 0.08), -0.15 for motor (95% CI, -0.41 to 0.14), and -0.19 for language (95% CI, -0.44 to 0.10). Fourteen children (21%) had myopia greater than -5.00 D in one or both eyes, 7 (10%) had optic nerve atrophy and/or cupping, 20 (29%) had strabismus, 8 (11%) had manifest nystagmus, and 9 (13%) had amblyopia. CONCLUSIONS: In this study cohort, there was no statistically significant correlation between dosage of bevacizumab and Bayley scores at 2 years. However, the sample size was small and the retention rate relatively low, limiting our conclusions. Rates of high myopia and ocular abnormalities do not differ from those reported after larger bevacizumab doses.

Retinal injury identified by overhead-mounted optical coherence tomography in two young children with infantile-onset glaucoma.

PURPOSE: To report and characterize unexpected retinal findings identified by imaging with overhead-mounted optical coherence tomography (OCT) in 2 young children with infantile-onset glaucoma. METHODS: Children with glaucoma were imaged during clinically indicated examinations under anesthesia using overhead-mounted HRA+OCT Spectralis with Flex module (Flex-OCT, Heidelberg, Germany) from February 2017 through February 2022. Imagers prioritized scans of the peripapillary retinal nerve fiber layer (pRNFL), optic nerve head, and macula. Children imaged before age 2 years with images adequate for evaluation were included. Age at glaucoma diagnosis, glaucoma type, highest intraocular pressure (IOP), corneal diameter (CD), and axial length (AL) were recorded. RESULTS: A total of 41 children (71 eyes with glaucoma) were imaged before age 2 years. Macular imaging identified both inner and outer retinal thinning in 3 eyes of 2 young children (both eyes of a child with newborn primary congenital glaucoma (PCG) and 1 eye of a child with glaucoma following cataract surgery), which remained stable over time. These findings were present in 2 of 41 children (4.9%) and 3 of 71 eyes (4.2%) imaged with Flex-OCT. Neither highest IOP, CD, nor AL at imaging differentiated the 3 eyes with retinal changes from the larger group. CONCLUSIONS: Three eyes of 2 young children with refractory glaucoma of different etiologies and highly elevated IOP demonstrated areas of inner and outer retinal thinning, consistent with retinal injury from probable prior macular ischemia. Unexpected retinal pathology identified on Flex-OCT imaging in infantile-onset glaucomas highlights the need for continued study of the pathophysiology of this disease.

Associations between systemic health and retinal nerve fibre layer thickness in preterm infants at 36 weeks postmenstrual age.

BACKGROUND/AIMS: Neonatal insults from systemic diseases have been implicated in the pathway of impaired neurodevelopment in preterm infants. We aimed to investigate the associations between systemic health factors and retinal nerve fibre layer (RNFL) thickness in preterm infants. METHODS: We prospectively enrolled infants and imaged both eyes at 36±1 weeks postmenstrual age (PMA) using a hand-held optical coherence tomography system at the bedside in the Duke intensive care nurseries. We evaluated associations between RNFL thickness and 29 systemic health factors using univariable and multivariable regression models. RESULTS: 83 infants with RNFL thickness measures were included in this study. Based on the multivariable model, RNFL thickness was positively associated with infant weight at imaging and was negatively associated with sepsis/necrotising enterocolitis (NEC). RNFL thickness was 10.4 µm (95% CI -15.9 to -4.9) lower in infants with than without sepsis/NEC in the univariable analysis (p<0.001). This difference remained statistically significant after adjustment for confounding variables in various combinations (birth weight, birthweight percentile, gestational age, infant weight at imaging and growth velocity). A 250 g increase in infant weight at imaging was associated with a 3.1 µm (95% CI 2.1 to 4.2) increase in RNFL thickness in the univariable analysis (p<0.001). CONCLUSIONS: Low infant weight and sepsis/NEC were independently associated with thinner RNFL in preterm infants at 36 weeks PMA. To our knowledge, this study is the first to suggest that sepsis/NEC may affect retinal neurodevelopment. Future longitudinal studies are needed to investigate this relationship further.

Biphasic change in retinal nerve fibre layer thickness from 30 to 60 weeks postmenstrual age in preterm infants.

BACKGROUND/AIMS: The optic nerve development during the critical postnatal weeks of preterm infants is unclear. We aimed to investigate the change of retinal nerve fibre layer (RNFL) in preterm infants. METHODS: We used an investigational handheld optical coherence tomography (OCT) system to serially image awake preterm infants between 30 and 60 weeks postmenstrual age (PMA) at the bedside. We assessed RNFL thickness in the papillomacular bundle and nasal macular ganglion cell layer+inner plexiform layer (GCL+IPL) thickness. We applied a segmented mixed model to analyse the change in the thickness of RNFL and GCL+IPL as a function of PMA. RESULTS: From 631 OCT imaging sessions of 101 infants (201 eyes), RNFL thickness followed a biphasic model between 30 and 60 weeks, with an estimated transition at 37.8 weeks PMA (95% CI: 37.0 to 38.6). RNFL thickness increased at 1.8 µm/week (95% CI: 1.6 to 2.1) before 37.8 weeks and decreased at -0.3 µm/week (95% CI: -0.5 to -0.2) afterwards. GCL+IPL thickness followed a similar biphasic model, in which the thickness increased at 2.9 µm/week (95% CI: 2.5 to 3.2) before 39.5 weeks PMA (95% CI: 38.8 to 40.1) and then decreased at -0.8 µm/week (95% CI: -0.9 to -0.6). CONCLUSION: We demonstrate the feasibility of monitoring RNFL and GCL+IPL thickness from OCT during the postnatal weeks of preterm infants. Thicknesses follow a biphasic model with a transition age at 37.8 and 39.5 weeks PMA, respectively. These findings may shed light on optic nerve development in preterm infants and assist future study designs.

Performance of virtual reality game-based automated perimetry in patients with childhood glaucoma.

PURPOSE: To evaluate VisuALL, a game-based automated perimetry device, utilizing virtual reality (VR) goggles, in a cohort of patients with childhood glaucoma. METHODS: In this prospective series, the results of consecutive patients with childhood glaucoma performing both VisuALL VR field (VRF) and Humphrey visual field (HVF) 24-2 testing were compared. A masked ophthalmologist graded both VRF and HVF tests for field defects (three clustered abnormal points in total or pattern deviation plot). VRF testing was performed binocularly and with the child's own spectacles. The two devices were assessed with respect to agreement of (1) global indices, such as mean deviation (MD) and pattern standard deviation (PSD), (2) point-by-point sensitivity, and (3) the ability to detect visual field defects determined by a grader. RESULTS: A total of 39 children (77 eyes) were enrolled, with mean age 14.1 ± 3.6 years; 3 patients (5 eyes) could not complete the HVF. Average HVF MD was -6.3 ± 6.4 dB. There was strong correlation between VRF and HVF for MD (R = 0.68, P < 0.001), PSD (R = 0.78, P < 0.001), and point-by-point sensitivity (R = 0.63, P < 0.001). Bland Altman analysis showed no systematic difference between VRF and HVF in assessing MD and PSD. Of 72 eyes having results for both modalities, 63 (87.5%) had agreement between VRF and HVF with respect to the presence/absence of any field defect, and 52 (72.2%) had agreement regarding the presence/absence of fixation-threatening field loss. CONCLUSIONS: VRF is comparable to the gold standard HVF in both identification and quantification of visual field deficits in pediatric glaucoma patients and may offer a valuable supplement or alternative to standard automated perimetry.

Quantitative comparison of blood vessel changes after treatment of retinopathy of prematurity with laser versus bevacizumab.

Current therapies for treatment-indicated (type 1) retinopathy of prematurity mainly consist of laser photocoagulation and intravitreal anti-vascular endothelial growth factor (eg, bevacizumab) injection. The first visible signs of acute-phase regression are typically vascular, including decreased plus disease. Using a semiautomated computer program, we quantitatively compared posterior pole vascular changes following treatment with laser versus bevacizumab and found that in the first month following treatment, vascular dilation and tortuosity significantly decreased following either treatment modality, but tortuosity decreased earlier and faster after bevacizumab.

"Coming up short" with an illuminated microcatheter for circumferential trabeculotomy externo in the buphthalmic infant eye: frequency and options.

An illuminated microcatheter is frequently used to perform trabeculotomy ab externo in infants with primary congenital glaucoma (PCG). Enlarged corneas in these children can make it challenging to traverse 360o of Schlemm's canal (SC) before reaching the passable limit of the microcatheter. Using basic geometry, we predicted that the iTrack microcatheter would fail to cannulate SC completely in eyes with a horizontal corneal diameter (HCD) >14.8 mm. A retrospective chart review of patients with PCG undergoing angle surgery identified the frequency of corneas with HCD >14.8 mm and confirmed that this calculated cut-off predicts which eyes can-or cannot-be cannulated successfully (ie, 360o) by the iTrack microcatheter.

Outcomes of inferonasal glaucoma drainage device surgery in the management of childhood glaucoma.

PURPOSE: To evaluate indications and postoperative outcomes of inferonasal Ahmed and Baerveldt glaucoma drainage devices (GDDs) in pediatric glaucoma. METHODS: This retrospective study included patients ≤18 years of age at inferonasal GDD placement, from June 2013 to September 2021. Demographic information, indications, and outcomes were recorded. Successful eyes had intraocular pressure (IOP) ≥5 and ≤21 mm Hg or (in severe cases, where entry IOP was ≤21 mm Hg) 20% below preoperative values without additional IOP-lowering surgery or vision-threatening complications. RESULTS: A total of 68 eyes of 52 patients were included. Mean age was 9.8 ± 5.8 years. Most common diagnoses included primary congenital glaucoma (n = 28 eyes [41%]) and glaucoma following cataract surgery (n = 12 eyes [18%]). Success rates by Kaplan-Meier survival analysis (95% CI) at 1, 2, 3, and 5 years were 62.2% (49.4-72.7), 49.0% (36.3-60.6), 36.8% (24.0-49.8), and 31.9% (18.5-44.5), respectively. Surgical failure was associated with Ahmed (vs Baerveldt) GDD (HR = 3.2; P = 0.002) and inferonasal site as initial (vs subsequent) GDD received (HR = 2.2; P = 0.048). Twenty-five eyes (36.7%) had at least one complication. Although vision-threatening complications were uncommon (n = 2 eyes [3%]), less severe complications were relatively common and included new/worsened strabismus (n = 9 patients [17%]), corneal edema (n = 6 eyes [9%]), lens opacities (n = 4 [6%]), tube or patch graft exposure (n = 3 [4%]), tube blockage (n = 2 eyes [3%]), and other (n = 8 eyes [12%]). CONCLUSIONS: In this study cohort, inferonasal GDDs for childhood glaucoma had modest success, with frequent though rarely vision-threatening complications.