Genotype-Phenotype of CRB1-Associated Early-Onset Retinal Dystrophy: Novel Insights on Retinal Architecture and Therapeutic Window for Clinical Trials.

Purpose: The purpose of this study was to investigate the genotypic and phenotypic characteristics of CRB1-associated early onset retinal dystrophy (CRB1-eoRD) and retinal architecture by swept-source optical coherence tomography (SS-OCT). Methods: Eleven probands with CRB1-eoRD were recruited. Clinical information, genetic analysis, and comprehensive ophthalmic examinations including SS-OCT and SS-OCT angiography (SS-OCTA) were conducted. Results: A total of 81.8% (9/11) of CRB1-eoRD presented as Leber congenital amaurosis (LCA). Common clinical manifestations included coin-like yellow-white retinal spots (20/22, 90.9%) and para-arteriolar retinal pigment epithelial retention (12/22, 54.5%). Nineteen different CRB1 variants were detected in our case series, including 12 missense, 3 frameshifts, 3 nonsense, and 1 splicing. Of them, 12 variants had been reported, and 7 were novel. SS-OCT showed thinner central macula (the LCA group, P < 0.0001), thicker total retina (P < 0.0001), thinner outer retina (P < 0.05), and thicker inner retina (P < 0.0001) compared with the healthy control. The inner/outer (I/O) retina thickness ratio of CRB1-eoRD was 3.0, higher than the healthy control of 1.2 and other inherited retinal diseases (IRDs) of 2.2 (P < 0.0001 and P = 0.0027, respectively). SS-OCTA revealed an increased vascular density and perfusion area of the superficial vascular complex and deep vascular complex in CRB1-eoRD. Conclusions: LCA emerges as a frequently occurring phenotype in CRB1-eoRD. The unique features of SS-OCT and SS-OCTA are illustrated, and the novel biomarker, I/O ratio, may facilitate early diagnosis. The insights gained from this study hold significant value in determining the treatment window for potential forthcoming CRB1 gene therapy.

Pattern of choroidal thickness in early-onset high myopia.

Purpose: To explore the etiology and choroidal thickness (ChT) pattern in children with early-onset high myopia (eoHM). Methods: Sixty children with eoHM and 20 healthy controls were enrolled in this study between January 2019 and December 2021. All children underwent comprehensive ophthalmologic examinations including swept-source optical coherence tomography. ChT was measured in the subfoveal region and at 1000 µm and 2,500 µm nasal, temporal, superior, and inferior to the fovea. Results: Overall, 120 eyes of 60 children with eoHM were examined (mean spherical equivalent, -8.88 ± 3.05 D; mean axial length, 26.07 ± 1.59 mm). Simple high myopia (SHM), familial exudative vitreoretinopathy (FEVR), and Stickler syndrome (STL) were the most frequent etiologies of eoHM and were included in further ChT analysis. Adjusted the effect of SE, multivariate regression analysis showed that children with SHM had thinnest ChT at N2500 and I2500 among the subgroups (p = 0.039, p = 0.013). FEVR group showed thinner ChT at T2500 (p = 0.023), while STL patients exhibited thin ChT at all locations. Conclusion: This study revealed that SHM, STL and FEVR was the most frequent etiology, and showed a distinctive pattern of ChT. Asymmetric nasal ChT thinning is a distinctive biomarker for SHM, asymmetric temporal ChT thinning might serve as a biomarker for FEVR, and symmetric diffuse thinning is more common in STL. These ChT patterns may provide a convenient, fast, and noninvasive strategy to differentiate the potential etiology of eoHM.

Five novel dysfunctional variants in the TSPAN12 gene in familial exudative vitreoretinopathy.

Familial exudative vitreoretinopathy (FEVR) is an inheritable vitreoretinal disease characterized by incomplete retinal vascular development, which often leads to multiple retinal complications and causes severe vision loss in children. We reported the TSPAN12 variants' frequency in a cohort of FEVR and five novel TSPAN12 variants and related clinical features in six Chinese families. Seven hundred thirty-four families' genetic in-house data were reviewed. Whole-exome sequencing (WES) was performed in all probands; Sanger sequencing was conducted in the family members. Five novel variants from six families were noted, and clinical data were collected. Luciferase assays were applied to test the activity of the Norrin/ß-catenin signal caused by the mutant TSPAN12 genes. The frequency of TSPAN12 variants in FEVR is 8.79% (50/569). Five novel variants in TSPAN12 were identified in six families, including two missense variants, c.476G > A(p.Cys159Tyr) and c.81T > G(p.Ser27Arg), two frameshift variants, c.628_629insA(p.Met210Asnfs*42) and c.251delG(p.Gly84Glufs*3) and one nonsense, c.352G > T(p.Glu118*). Low vision, high myopia, nystagmus, and leukocoria are the common symptom at the first presentation. All variants were also predicted as pathogenic in silico. Moreover, the luciferase assay demonstrated that all variants caused severely compromised Norrin/ß-catenin signaling activity. In conclusion, the frequency of TSPAN12 variants in FEVR was 8.79% in our cohort. Five novel variants of TSPAN12 were identified. Moreover, we demonstrated the dysfunction of mutant variants via the downregulation of Norrin/ß-catenin signaling. These findings expanded the genetic and clinical spectrum of FEVR with TSPAN12 variants.

The Development of Electroretinographic Oscillatory Potentials in Healthy Young Children.

PURPOSE: This study aimed to summarize the electroretinographic oscillatory potential (OP) responses in healthy young children recorded by RETeval. METHODS: By using the RETeval system, we recorded the implicit times and amplitudes of the OPs (OP1-5), in 132 healthy children aged from 0 to 11 years old. The age, gender, and data of implicit time and amplitude of each child were recorded and analyzed. Correlation analysis was performed between age and implicit time/amplitude. RESULTS: No correlation was shown between the implicit times and amplitudes with gender. The implicit times and amplitudes of OP1-5 matured over 10 years of age, with exponential time constants of 1.9, 2.2, 1.8, 1.7, and 1.6 years and 2.1, 2.9, 2.8, 3.0, and 3.2 years, respectively. The majority of the trend occurred within the first 4.6 years. CONCLUSIONS: In order to diagnose and evaluate vision-related disorders, the OP response is commonly used. The percentiles and age dependence of OP responses calculated and shown in this study could be regarded as reference data in age-matched pediatric patients.

Oral Fluorescein Angiography with Ultra-Wide-Field Scanning Laser Ophthalmoscopy in Pediatric Patients Precis: Oral Fluorescein Angiography in Children.

Aims: To investigate the success rate of oral fluorescein angiography (oral FA) in children with ultrawide scanning laser ophthalmoscopy (SLO) system and whether it can provide images of sufficient quality compared with intravenous FA (IVFA). Methods: In this comparative case series study, a series of 40 consecutive pediatric patients of the age of 3−18 with retinal vascular diseases, in whom FA was needed for the diagnosis or treatment, were enrolled in this study. IVFA and oral FA were performed within one week and images were obtained with the SLO system. The image quality was scored blindly and compared based on: (I) visualization of the branch retinal vessel, (II) the foveal avascular zone (FAZ), and (III) clinically important findings, such as the presence of microaneurysms, neovascularization, leakage, or significant nonperfusion. All these were scored using a three-point scale. Results: In preschoolers (three to six years), all 19 children complete oral FA (100%), while only 7 (36.84%) complete IVFA (p < 0.0001). With the SLO system, the branch retinal vessels were well visualized both in oral and IV FA (all images were two scores). The visualization of FAZ was similar between oral and IV FA (p = 0.8972). The clinically important findings were well visualized in both groups (p > 0.9999). The overall image quality was similar between the two groups (p = 0.2500). Conclusion: Oral FA is more acceptable to preschoolers than IVFA owing to the needle-free procedure. With the SLO system, oral FA provided high-quality angiograms similar to IVFA. Oral FA is an effective alternative to IVFA and may be considered the first option for FA in pediatric patients, especially in preschoolers.

Clinical Characteristics and Risk Factors for Evisceration in Trauma-Dominant Orbital Cellulitis: A 10-Year Review.

Purpose: To analyze the clinical characteristics of trauma-dominant orbital cellulitis (OC) and evaluate the risk factors associated with evisceration. Methods: This retrospective study included inpatients diagnosed with orbital cellulitis at the Zhongshan Ophthalmic Center between January 2010 and December 2020. The demographic features, etiology, clinical characteristics, microbiological isolates, and risk factors associated with evisceration were analyzed. Results: Among 148 consecutive subjects (n = 148, 148 eyes), the mean age was 42.07 ± 20.35 years and 70.27% were male. Penetrating globe injuries were the most common (52.03%). No light perception (NLP) was recorded in 50% of patients on admission. Endophthalmitis was observed in 103 cases (69.59%), intraocular foreign bodies (IOFB) were detected in 43 cases (29.05%), and total corneal melting was observed in 31 cases (20.95%). Sixty patients (40.54%) underwent evisceration. Logistic regression analysis showed that total corneal dissolution [odds ratio (OR) = 83.019, P = 0.000], IOFB (OR = 3.402, P = 0.016), and NLP (OR = 0.185, P = 0.001) were risk factors for evisceration. Microorganism detection showed that Pseudomonas aeruginosa and Bacillus cereus were the leading pathogens. Conclusion: Among hospitalized trauma-dominant OC patients, middle-aged men were the major subjects and penetrating globe injury was the major cause. Significant complications such as complete visual loss and evisceration were unavoidable in many patients with OC in the current study. NLP, IOFB, and total corneal melting were the risk factors for evisceration.

Cytokine Profile in Aqueous Humor of Patients With Ocular Toxocariasis.

Purpose: Ocular toxocariasis (OT) is a vision-threatening disease with a largely unknown intraocular pathogenesis. Herein, we determined the cytokine expression profile in aqueous humor (AH) of patients with OT. Methods: This is a retrospective case-control study of cytokine levels in AH of patients with OT and uveitis and control subjects. Thirty samples from eyes with OT, 23 from eyes with non-OT uveitis, and 25 from eyes with age-related cataract were analyzed using a multiplexed magnetic bead immunoassay. Thirty-one cytokines were detected and classified into 5 categories: T-helper type 1 (Th1) -associated cytokines, Th2-associated cytokines, Th17 cytokine, proinflammatory mediators, and growth factors. Results: In the 31 cytokines, 9 cytokines were undetectable, including IL-1a, IL-1b, IL-2, IL-3, IL-12p70, IL-17A, TGF-a, TNF-ß, and IFN-g. From the 22 cytokines, 13 exhibited significantly increased expression in the OT group than in the control group, including TNF-a, IFN-a2, IL-4, IL-5, IL-6, IL-9, IL-10, IL-13, sCD40L, PDGF-AA, PDGF-AB/BB, FLT3l, and EGF. There were 5 cytokines exhibited significantly increased expression in the OT group than in non-OT group, including IL-5, IL-9, IL-10, IL-13, and PDGF-AA. There was no significantly decreased expression in any cytokines in the OT group when compared with control or non-OT groups. To the 5 cytokines that showed significant difference in OT group alone, IL-10 and IL-13 exhibited more than 13-fold increase, and IL-5 showed the most obvious as 27-fold increase in OT patients, when compared with that in control group. Conclusion: The cytokine profile expression in aqueous humor from patients with ocular toxocariasis was investigated, and our findings suggest that Th1 and Th17 cytokine responses are not enhanced, whereas the cytokine status was polarized toward a Th2 response. Our findings also suggest the involvement of IL-5, IL-10, and IL-13 in the immunopathogenesis of ocular toxocariasis.

FZD4 in a Large Chinese Population With Familial Exudative Vitreoretinopathy: Molecular Characteristics and Clinical Manifestations.

Purpose: The purpose of this study was to establish a genotype-phenotype correlation of familial exudative vitreoretinopathy (FEVR) caused by FZD4 gene mutations. Methods: Six hundred fifty-one probands and their family members were recruited based on a clinical diagnosis of FEVR between 2015 and 2021 at Zhongshan Ophthalmic Center. Ocular examinations were performed in all participants. Targeted gene panel sequencing and whole-exome sequencing were performed in the probands, and Sanger sequencing was used to verify the mutations and segregation analysis was performed in the family members. Results: Fifty-one FZD4 mutations (24 novels and 27 known) were detected in 84 families. Of these 168 eyes with FEVR, the eyes at stages 1, 2, 3, 4, and 5 were 29 (17.3%), 15 (8.9%), 19 (11.3%), 55 (32.7%), and 12 (7.1%), respectively. Exact stage of 38 (22.6%) eyes could not be determined. The FEVR phenotypes were more severe in the probands than the phenotypes in the family members (P < 0.001). The families were divided into two groups, probands that inherited the variant from the mother, and probands that inherited the variant from the father. In addition, the FEVR stage differences between these two groups were different (P < 0.05). Despite the mutations being located in different domains of FZD4, no significant differences were identified among the domains in terms of FEVR staging, retinal folds, retinal detachment, temporal midperipheral vitreoretinal interface abnormality, and foveal hypoplasia. Conclusions: The FZD4 probands had severer phenotype than the family members, and the FEVR stage difference was greater between the probands and mothers than that between the probands and fathers.

NONINVASIVE DIAGNOSTIC STRATEGY OF OCULAR TOXOCARIASIS BASED ON CLINICAL FEATURES.

PURPOSE: To develop a noninvasive diagnostic strategy based on the clinical manifestations of ocular toxocariasis (OT) and evaluate its sensitivity and specificity. METHODS: Patients with unilateral OT-like lesions were enrolled retrospectively and classified into OT and non-OT groups according to the immunologic diagnosis criterion of anti-OT immunoglobulin G. Nine clinical manifestations were recorded and compared between the groups. Among them, the retrolental membrane, branch-like vitreous strands, and retinal granulomas were the most common, which were further classified into three categories, including at least 1 of three signs, at least two of three signs, and all three signs positive. Diagnostic sensitivity and specificity were calculated for each strategy. RESULTS: There were 105 immunologically confirmed patients with OT and 70 patients with non-OT uveitis/vitreoretinopathy. Retinal granulomas, retrolental membrane, and branch-like vitreous strands were significantly more frequent in OT patients than in non-OT patients. At least 1 of 3 signs positive strategy showed the highest sensitivity (100.0%) but the lowest specificity (62.0%). At least 2 of 3 signs positive strategies showed 80.0% sensitivity and 94.3% specificity. All 3 signs positive strategies had the lowest sensitivity (46.7%) and the highest specificity (100.0%). The cutoff point of this revealed an area under the curve of 0.85 and a 95% confidence interval of 0.79 to 0.91. CONCLUSION: A comprehensive strategy based on at least two out of three positive signs showed excellent sensitivity and specificity and could serve as a noninvasive and fast screening strategy for the clinical diagnosis of OT.

Corneal Biometric Parameters and Refractive Properties in Microcornea With Normal Axial Length.

PURPOSE: The purpose of this study was to evaluate the features of and relationships among corneal diameter, central corneal thickness (CCT), endothelial cell density (ECD), and corneal refractive properties in cases of microcornea (MC) with normal axial length. METHODS: We included 22 patients (35 eyes) with MC and cataracts and 41 patients (48 eyes) with isolated cataracts as age-matched controls. They were divided into patients with MC younger than 18 years (MC-child), patients with MC 18 years or older (MC-adult), children with congenital cataracts (CCs), and adults with senile cataracts (SCs). Corneal diameter, axial and anterior chamber length, and keratometry were measured; central corneal endothelial cell imaging was performed. RESULTS: The mean horizontal corneal diameter was 7.71 ± 1.51 and 8.78 ± 0.52 mm in MC-child and MC-adult groups, respectively, and 11.89 ± 0.59 and 11.52 ± 2.42 mm in child and adult controls, respectively. The mean CCT was 641.26 ± 63.37 (MC-child) and 617.38 ± 45.40 mm (MC-adult), and 554.92 ± 34.64 (CC) and 551.58 ± 28.47 mm (SC). The mean ECD was 2898.47 ± 443.90 (MC-child) and 2825.81 ± 484.65 cells/mm 2 (MC-adult), and 3155.13 ± 372.67 (CC) and 2749.33 ± 399.63 cells/mm 2 (SC). The average keratometry was 44.22 ± 3.14 D (MC-child) and 43.86 ± 2.59 D (MC-adult), and 44.19 ± 1.44 D (CC) and 43.94 ± 1.34 D (SC). CONCLUSIONS: Patients with MC and normal axial length possess specific parameters, including significantly smaller corneal diameter and thicker CCT than the patients in the control groups. There were no significant differences in ECD and average keratometry. These parameters should be taken into account in the follow-up and treatment.

Post-traumatic endophthalmitis caused by streptococcus species in preschool children: clinical features, antibiotic susceptibilities and outcomes.

BACKGROUND/OBJECTIVES: Streptococcus is a common cause of post-traumatic endophthalmitis in children. This study aimed to analyse the clinical features, antibiotic susceptibilities and outcomes of traumatic endophthalmitis caused by streptococcus in preschool children. SUBJECTS/METHODS: Patients aged ≤6 years with traumatic streptococcal endophthalmitis seen at Zhongshan Ophthalmic Center between January 2013 and December 2018 were included in this retrospective study. RESULTS: In total, 21 patients (21 eyes) were included. The mean age of the patients was 3.3 ± 1.7 years, where 57.1% were males. Scissors (28.6%, n = 6) were the most common cause of injury; 86.7% of patients were injured at home. Zone I (80.9%) was the most common wound site; 90.5% of patients presented with a traumatic cataract. In general, Streptococcus pneumoniae (47.6%) was the most common isolate. Viridans group streptococci accounted for 58.3% of cases in children aged 0-3 years, while S. pneumoniae accounted for 66.7% of cases in children aged 4-6 years. The susceptibility rates of streptococcus to cefuroxime, levofloxacin and ofloxacin were 100%, 95.0% and 90.5%, respectively. Although all the patients underwent vitrectomy combined with silicone oil tamponade, the final visual outcomes were no better than counting fingers. CONCLUSIONS: Although S. pneumoniae was the most prevalent organism in general, the dominant species varied between different age groups. The commonly used antibiotics, cefuroxime and fluoroquinolone, showed higher antibiotic susceptibility. Despite prompt treatment, the visual outcomes of paediatric post-traumatic endophthalmitis in preschool children were poor.

αB-Crystallin Alleviates Endotoxin-Induced Retinal Inflammation and Inhibits Microglial Activation and Autophagy.

αB-Crystallin, a member of the small heat shock protein (sHSP) family, plays an immunomodulatory and neuroprotective role by inhibiting microglial activation in several diseases. However, its effect on endotoxin-induced uveitis (EIU) is unclear. Autophagy may be associated with microglial activation, and αB-crystallin is involved in the regulation of autophagy in some cells. The role of αB-crystallin in microglial autophagy is unknown. This study aimed to explore the role of αB-crystallin on retinal microglial autophagy, microglial activation, and neuroinflammation in both cultured BV2 cells and the EIU mouse model. Our results show that αB-crystallin reduced the release of typical proinflammatory cytokines at both the mRNA and protein level, inhibited microglial activation in morphology, and suppressed the expression of autophagy-related molecules and the number of autophagolysosomes in vitro. In the EIU mouse model, αB-crystallin treatment alleviated the release of ocular inflammatory cytokines and the representative signs of inflammation, reduced the apoptosis of ganglion cells, and rescued retinal inflammatory structural and functional damage, as evaluated by optical coherence tomographic and electroretinography. Taken together, these results indicate that αB-crystallin inhibits the activation of microglia and supresses microglial autophagy, ultimately reducing endotoxin-induced neuroinflammation. In conclusion, αB-crystallin provides a novel and promising option for affecting microglial autophagy and alleviating symptoms of ocular inflammatory diseases.

What Is Left After Resolution of Neonatal Retinal Hemorrhage: The Longitudinal Long-term Outcome in Foveal Structure and Visual Function.

PURPOSE: Neonatal retinal hemorrhage (NRH) is one of the most common neonatal fundus conditions. Hemorrhage resolves spontaneously; however, its long-term outcome is unknown yet. The current study explores the long-term role of NRH in foveal structure and visual function. DESIGN: Cohort study (a prospective longitudinal study, in which the participants were followed up for 4-6 years). METHODS: A total of 125 healthy newborns during 2013-2015, including 50 newborns with NRH and 75 newborns without NRH, were enrolled. The eyes with NRH were further categorized into the foveal hemorrhage (FH) group and non-FH group. A comprehensive ophthalmic examination including best-corrected visual acuity (BCVA) measurement, slit-lamp examination, refractive error measurement, scanning laser ophthalmoscopy, and spectral-domain OCT was performed. Total retinal thickness (TRT) and the inner and outer retinal layers in the fovea were measured and compared. RESULTS: The NRH was absorbed within 2.1 ± 0.98 weeks (median: 3 weeks). No difference was noted in the demographic characteristics between the groups; there was no significant difference in the logMAR BCVA (P = .83) or in the TRT. Subgroup analysis showed that TRT at the fovea in the FH group was significantly thicker (P = .005). Segmentation analysis showed a significantly thicker foveal outer nuclear layer (ONL) in the FH group (P = .017). CONCLUSIONS: Birth-related retinal hemorrhage, even FH, might not lead to obvious visual abnormalities at the age of 4 years, at least according to this study with relatively small sample size. However, a thicker fovea, mainly attributed to a wider ONL and a shallower foveal pit, is noted in our study.

Etiology, microbiological isolates, and antibiotic susceptibilities in culture-proven pediatric endophthalmitis: a 9-year review.

PURPOSE: To analyze the etiology, microbiological isolates, and antibiotic susceptibilities of endophthalmitis in pediatric patients. METHODS: Patients aged < 18 years with culture-positive endophthalmitis in Zhongshan Ophthalmic Center between January 2010 and December 2018 were included retrospectively. RESULTS: A total of 127 patients (127 eyes) were included, and 108 (85%) had posttraumatic endophthalmitis. Streptococcus (21.4%), coagulase-negative Staphylococcus (14.5%), Aspergillus (6.9%), and Bacillus cereus (5.3%) were the common organisms. The proportion of Streptococcus decreased with age (40.0% in 0-3 years, 16.3% in 4-12 years, and 6.3% in 13-17 years), while coagulase-negative Staphylococcus increased from 5.7% to 18.8%. Overall, fluoroquinolones achieved the highest antibiotic susceptibility rate (> 95%), while the susceptibility of isolated bacteria to tobramycin and cefazolin was only 60.2% and 59.4%, respectively. The susceptibility rates of Gram-positive cocci to cephalosporins were nearly 90%. For Gram-negative bacilli, susceptibility to neomycin was 91.3%. CONCLUSION: Trauma was the main etiology for pediatric endophthalmitis. Although Streptococcus was the most prevalent organism in general, the dominant pathogen varied with age, which merits clinical attention. Fluoroquinolones showed the highest antibiotic efficacy; however, commonly used antibiotics tobramycin and cefazolin showed relatively low antibiotic susceptibility. Thus, antibiotic resistance in pediatric populations merits clinical attention.

New PTEN mutation identified in a patient with rare bilateral choroidal ganglioneuroma.

BACKGROUND: Choroidal ganglioneuroma is an extremely rare tumor, and there is little knowledge regarding its pathogenesis. We aimed to investigate the phenotypic and genetic alterations in one sporadic patient with a rare case of bilateral choroidal ganglioneuroma. METHODS: A 6-year-old boy with histological diagnosis of bilateral ganglioneuroma was recruited for the study. Comprehensive ophthalmic examinations were performed. Genomic DNA was extracted from the peripheral blood samples collected from the patient, his unaffected family members, and 200 unrelated control subjects from the same population. Whole exome sequencing was performed and raw reads were aligned to the human genome reference (hg19) using Burrows-Wheeler Aligner. DNA from all available family members was Sanger sequenced for segregation analysis. RESULTS: Extensive bilateral retinal detachments were observed via optical coherence tomography. Diffuse thickening of choroid was identified with ultrasound B scan and magnetic resonance imaging. Genetic analysis revealed the presence of a novel heterozygous PTEN frameshift mutation, c.498delA (p.Thr167LeufsTer16), in exon 6. It was present in the affected individual, but not in any of the family members. Genetic analysis revealed that there was no mutation in neurofibromatosis-related genes in the family. Upon performing comprehensive systemic examinations, no obvious abnormalities in other organs were observed. CONCLUSIONS: A novel de novo PTEN mutation was identified in a patient with bilateral choroidal ganglioneuroma. Although PTEN mutations are known to induce multiple abnormalities, choroidal ganglioneuroma can be the first manifestation without abnormalities in other organs. Further studies are needed to confirm the association between choroidal ganglioneuroma and PTEN mutation.

Changing Trends in Firework-Related Eye Injuries in Southern China: A 5-Year Retrospective Study of 468 Cases.

Firework-related eye injury is a horrible medical problem and creates huge health and social burdens. Herein, we explored the changing trends and demographic and clinical features of firework-related eye injury, in an effort to inform strategies to prevent this injury. We reviewed the data of 468 hospitalized patients who underwent surgery for firework-related eye injury at the Zhongshan Ophthalmic Center between January 2013 and December 2017. During this period, the trend in firework-related eye injuries was stable (mean, 93 ± 14 cases per year), and fireworks were the major cause of explosive eye injury. The average age of the patients was 24 ± 18 years and 87% of the patients were male, with boys under 10 years of age comprising the largest group (27% of patients). There were an average of 24 ± 7 cases per year from urban areas and 70 ± 8 cases from rural areas (P < 0.05). Furthermore, 21 ± 5% of cases occurred during Spring Festival. After treatment, the best corrected visual acuity was increased compared to that before treatment, and the intraocular pressure tended to become normal by the final visit (P < 0.05). The top three diagnoses were cataract (39%), retinal detachment (18%), and choroidal detachment (14%). Additionally, the most common surgery was cataract extraction (25%), followed by pars plana vitrectomy (19%) and pars plana vitrectomy plus pars plana lensectomy (10%). Over the five-year study period, day surgery hospitalization increased from 1% to 32%. This was associated with a corresponding decrease in the length of hospitalization, without adverse events, demonstrating that day surgery is feasible in firework-related eye injury cases. The present study results suggest that greater attention should be paid to firework-related eye injury, and a variety of measures should be taken to prevent this kind of ocular tragedy.

Multimodal Imaging Features of Bilateral Choroidal Ganglioneuroma.

PURPOSE: Bilateral choroidal ganglioneuroma is extremely rare, and no cases have been described in the literature. Multimodal images are crucial for its diagnosis. Here, we evaluated multimodal images in the early stage of choroidal ganglioneuroma. METHODS: A 6-year-old boy was recruited who had experienced gradually progressive vision loss and rapidly progressive myopia in both eyes over the past 2 years. His eyes were comprehensively evaluated via slit-lamp microscopy, ultrasound biomicroscopy, swept-source optical coherence tomography (SS-OCT), fundoscopy, fundus fluorescein angiography, indocyanine green angiography (ICGA), ultrasound B scanning, and magnetic resonance imaging. Electrophysiological examinations included electrooculography and electroretinography. Choroid biopsy and pathological examination were performed. RESULTS: Over the past 2 years, the patient's best-corrected visual acuity had gradually decreased to hand motions at 10 cm in the right eye and 20/63 in the left, with axial length growth to 25.89 mm in the right and 28.99 mm in the left. Diffuse thickening in bilateral eyewalls was depicted in B scanning and magnetic resonance imaging. Secondary exudative retinal detachment was evident in SS-OCT and B scanning. SS-OCT depicted low optical reflections in the choroidal layer, revealing a lack of choroidal vasculature. Diffuse hypofluorescence in ICGA photography confirmed extensive loss of choroidal vasculature. In electrophysiological function investigations, electrooculography revealed remarkable bilateral low Arden ratios, with almost extinguished electroretinogram in the right eye. Right-eye choroid biopsy was performed, resulting in a histological diagnosis of choroidal ganglioneuroma. CONCLUSION: Choroidal ganglioneuroma is rare. To our knowledge, no bilateral cases have been described in the literature. Major clinical features include a rapid increase in axial length, diffuse choroidal thickening, hyper-reflectivity in the choroid on optical coherence tomography, and loss of choroidal vasculature on ICGA. The current report provides multimodal imaging of choroidal ganglioneuroma for the first time and can be valuable for early diagnosis.

Risk Factors for Trauma-Related Eviscerations: Analysis of 821 Cases.

Evisceration is the end therapeutic approach for the treatment of severe ocular trauma cases, and it is a tremendous loss for patients. In this study, we explored the changing trends in the number of surgeries performed, demographic data and ocular features, and risks for early evisceration, aiming to provide more data for the additional prevention and management strategies for this catastrophic problem. This was a retrospective study that included patients who underwent ocular evisceration at the Zhongshan Ophthalmic Center between January 2013 and December 2017. A total of 1229 evisceration cases were reviewed, and only trauma-related eviscerations were analyzed. Etiology, demographic data, ocular features, and hospitalization time were evaluated. The total number of trauma-related eviscerations recorded in the past five years was 821 cases. The number of surgeries performed was almost constant each year (164 ± 17 cases); 35% of the patients were less than ten years old at the time of the original ocular injury and 69% of them were male. Endophthalmitis led to significantly early evisceration compared with cases without endophthalmitis (P < 0.05). The group with a history of silicone oil tamponade showed a significantly longer surgical interval between trauma and evisceration than did the nonsilicone oil tamponade group (P < 0.05). Day-case hospitalization for evisceration increased from 0% to 32% over the past five years. The results of the present study show that the number of ocular trauma-related eviscerations performed in the past five years was almost unchanged and boys under ten are highly susceptible. This study also demonstrates that endophthalmitis leads to significantly early evisceration, and silicone oil tamponade may postpone ocular atrophy. Based on the study data, day-case surgery is safe for evisceration management.

Diltiazem Promotes Regenerative Axon Growth.

Axotomy results in permanent loss of function after brain and spinal cord injuries due to the minimal regenerative propensity of the adult central nervous system (CNS). To identify pharmacological enhancers of axon regeneration, 960 compounds were screened for cortical neuron axonal regrowth using an in vitro cortical scrape assay. Diltiazem, verapamil, and bromopride were discovered to facilitate axon regeneration in rat cortical cultures, in the presence of chondroitin sulfate proteoglycans (CSPGs). Diltiazem, an L-type calcium channel blocker (L-CCB), also promotes axon outgrowth in adult primary mouse dorsal root ganglion (DRG) and induced human sensory (iSensory) neurons.