Single-line macular optic coherence tomography to confirm optic neuropathies in awake infants and young children.

BACKGROUND: Handheld optical coherence tomography (HH-OCT) can image awake, young children but lacks integrated segmentation/analysis software. OCT imaging of eyes with optic neuropathies demonstrates ganglion cell layer (GCL) and ganglion cell complex (GCC) thinning, with a normal or thickened inner nuclear layer (INL). We compared pediatric normative data with GCL/INL and GCC/INL ratios from HH-OCT macular scans of awake young children with clinically diagnosed optic neuropathies. METHODS: Macular HH-OCT from awake children with optic neuropathies was prospectively obtained using Bioptigen (Leica Microsystems, Wetzlar, Germany). The GCL, GCC, and INL were manually measured by two readers using ImageJ from single-line macular scans at the thickest points nasal and temporal to the fovea, respectively, and the GCL/INL and GCC/INL ratios were calculated and compared with normative data. RESULTS: HH-OCT images from 17 right eyes of 17 children (mean age, 4.3 ± 2.9 years) with optic neuropathies were analyzed. Mean nasal (17 eyes) and temporal (16 eyes) GCL/INL ratios with optic neuropathies were 0.44 ± 0.38 (95% CI, 0.26-0.62) and 0.26 ± 0.22 (95% CI, 0.15-0.36), respectively. Corresponding normative GCL/INL ratios are 1.26 ± 0.20 (95% CI, 1.19-1.34) and 1.23 ± 0.27 (95% CI, 1.13-1.33), respectively (P < 0.0001). Severe thinning precluded GCL measurements in 2 eyes nasally and 5 eyes temporally, resulting in GCL measurements of zero. Mean nasal (17 eyes) and temporal (16 eyes) GCC/INL ratios were 1.93 ± 0.70 (95% CI,1.60-2.27) and 1.67 ± 0.44 (95% CI,1.46-1.87). Corresponding normative ratios are 2.85 ± 0.38 (95% CI, 2.71-2.99) and 2.87 ± 0.42 (95% CI, 2.70-3.03), respectively (P < 0.0001). CONCLUSIONS: GCL/INL and GCC/INL ratios calculated from single-line macular HH-OCT scans in awake young children with optic neuropathies differ significantly from normative values and may thus have utility in helping to establish a diagnosis of optic neuropathy.

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.

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.

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.

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.

Successful repair of a spontaneous scleral rupture in a patient with type VI Ehlers-Danlos syndrome.

PURPOSE: To describe ocular findings in a patient with Type VI Ehlers-Danlos syndrome (EDS) and make ophthalmologists aware of the potential ophthalmic complications of this particular type of EDS. To briefly report the surgical technique utilized for the repair of spontaneous scleral rupture that may be associated with Type VI Ehlers-Danlos syndrome. OBSERVATIONS: A 36-year-old female visited the Emergency Room due to sudden vision loss, edema, and redness of the right eye consistent with spontaneous scleral rupture secondary to scleral thinning due to Type VI EDS. Repair with scleral patch graft resulted in improvement in visual acuity, a decrease in hyphema, and discomfort resolution. CONCLUSIONS AND IMPORTANCE: Spontaneous scleral perforation may occur in patients with Type VI EDS. A scleral patch graft may serve as a viable surgical repair alternative for such patients.

The nervous system component of the mesentery of the sea cucumber Holothuria glaberrima in normal and regenerating animals.

The mesenterial tissues play important roles in the interactions between the viscera and the rest of the organism. Among these roles, they serve as the physical substrate for nerves connecting the visceral nervous components to the central nervous system. Although the mesenterial nervous system component has been described in vertebrates, particularly in mammals, a description in other deuterostomes is lacking. Using immunohistochemistry in tissue sections and whole mounts, we describe here the nervous component of the intestinal mesentery in the sea cucumber Holothuria glaberrima. This echinoderm has the ability to regenerate its internal organs in a process that depends on the mesentery. Therefore, we have also explored changes in the mesenterial nervous component during intestinal regeneration. Extensive fiber bundles with associated neurons are found in the mesothelial layer, extending from the body wall to the intestine. Neuron-like cells are also found within a plexus in the connective tissue layer. We also show that most of the cells and nerve fibers within the mesentery remain during the regenerative process, with only minor changes: a general disorganization of the fiber bundles and a retraction of nerve fibers near the tip of the mesentery during the first days of regeneration. Our results provide a basic description of mesenterial nervous component that can be of importance for comparative studies as well as for the analyses of visceral regeneration.