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Purpose: To investigate the utility of biomechanical property measurements using a Scheimpflug-based tonometer (SBT) and/or anterior segment optical coherence tomography (AS-OCT) for diagnosing forme fruste keratoconus (FFK)., Methods: In this retrospective interventional case series, 23 eyes with FFK of 23 consecutive patients and 52 eyes of 52 healthy volunteers who visited our keratoconus outpatient clinic were enrolled. Logistic regression analysis was conducted to determine the causal relationship between FFK diagnosis and each parameter., Results: When only SBT was used, the corneal stiffness parameter, stiffness parameter A1 (SP-A1) and the corneal velocity at first applanation were selected as explanatory variables, and sensitivity, specificity and area under the receiver operating characteristic curve (AUROC) were 82.9%, 86.9% and 0.938, respectively. When only AS-OCT parameters were used, the posterior corneal asymmetric component and central corneal thickness were selected, and the sensitivity, specificity and AUROC were 82.6%, 94.2% and 0.893, respectively. When parameters from both methods were used, SP-A1 and the posterior corneal asymmetry component derived from Fourier analysis were selected as explanatory variables, and sensitivity, specificity and AUROC were 91.30%, 90.38% and 0.947, respectively. No significant differences in AUROC were observed between diagnoses using each device and the combination of both devices (AS-OCT versus SBT, p = 0.314; integrated parameters versus AS-OCT, p = 0.081; integrated parameters versus SBT, p = 0.234)., Conclusion: Optimization of SBT and AS-OCT parameters allowed for the diagnosis of FFK at a clinically usable level. Forme fruste keratoconus (FFK) diagnosis integrating biomechanical properties with AS-OCT showed no superiority compared to diagnosis based on a single device., (© 2020 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Intraocular lens (IOL) misalignment and angle κ have had significant negative impact on post-operative quality of vision. ZEMAX software has been used to imitate the optical performance of pseudophakic eyes with different IOL surface designs at different orientations of IOL misalignment (decentration of 0.4 mm and tilt of 7°, and with the existence of 0.5 mm angle κ ). We found that an aspheric balanced curve optic surface maintained better optical performance via inducing less coma aberration. Coma aberration played an unexpectedly important role in the optical performance. With angle κ , the impact of IOL misalignment on visual quality was associated with the orientation of decentration and tilt, indicating that the coma compensation also took effect in pseudophakic eyes. Due to the high incidence of post-operative IOL misalignment, our results provide evidence of the importance of considering personalized angle κ before cataract surgery for patients.

To investigate the association of peripheral anterior synechiae (PAS) with intraocular pressure (IOP) and anterior-segment parameters in subjects with primary angle-closure glaucoma (PACG). A total of 267 subjects with PACG were recruited and underwent gonioscopy and anterior-segment optical coherence tomography (ASOCT). Customized software was used to measure ASOCT parameters, including angle opening distance (AOD750) and trabecular-iris-space-area (TISA750) at 750 µm from the scleral spur, anterior chamber depth, width, area and volume (ACD, ACW, ACA, ACV), iris thickness (IT750), iris area (IAREA), and lens vault (LV). Presenting IOP was defined as the first IOP reading before the initiation of IOP-lowering treatment. The mean age of the 267 subjects was 67.0 ± 8.9 years, 140 (52.4%) were male, and 246 (92.1%) were of Chinese ethnicity. PAS was present in 122 (45.7%) subjects, and was most frequently found in the superior quadrant (79.5%). Subjects with PAS had greater presenting IOP (28.7 ± 12.9 vs 22.4 ± 9.7 mmHg, p < 0.001), narrower AOD750 (p < 0.001), smaller TISA750 (p < 0.001), ACD (p = 0.04), ACA (p = 0.02), ACV (p = 0.01) and larger LV (p = 0.01) compared to PACG eyes without PAS. No significant differences were noted for iris parameters. A multivariate logistic regression analysis showed that higher presenting IOP (β = 0.20, p < 0.001), worse visual field mean deviation (β = - 0.20, p = 0.01) and narrower AOD750 (β = - 0.25, p = 0.03) were the only parameters that significantly correlated with the extent of PAS in clock hours. Almost one-half of the subjects with PACG demonstrated PAS; these eyes were associated with higher presenting IOP, smaller anterior segment dimensions and more severe disease.

Purpose: To present long-term clinical and visual outcomes of patients with Peters anomaly., Methods: The charts of all patients diagnosed with Peters anomaly from January 2000 to December 2012 were reviewed retrospectively. Peters anomaly was classified as type I (with no lens involvement) or type II (presence of keratolenticular adhesions or cataract), with further severity grading to mild, moderate, and severe disease depending on corneal opacity location and size. Mild cases were observed. Moderate cases were managed with pupillary dilation either pharmacologically or surgically. Penetrating keratoplasty (PKP) was reserved for more severe opacity. The main outcome measures were final best spectacle-corrected visual acuity (BSCVA), incidence of glaucoma, graft survival, and nystagmus rates., Results: Sixty eyes of 40 patients were included in the study. The median age of patients at presentation was 0.5 ± 20.7 months (range, 0.0-111.0 months), with a mean follow-up time of 75.8 ± 52.9 months (range, 12.1-225.3 months). Overall, final best spectacle-corrected visual acuity ranged from 0.1 logMAR to no light perception with 33 eyes (55.9%) achieving vision of 1.0 logMAR or better. Clear grafts at the last follow-up were obtained in 67.6% (25/37) of transplanted eyes, 76.0% (19/25) in Peters type I, and 50.0% (6/12) in Peters type II (P = 0.11). The probability of a clear graft at 10 years was 74.2% and 38.9% for type I and type II, respectively. Glaucoma was diagnosed in 33.3% eyes, 90.0% of which occurred after PKP. Nystagmus was highly associated with PKP intervention, occurring in 81.1% (30/37) of eyes undergoing PKP compared with 34.8% (8/23) of eyes with no PKP (P = 0.0003)., Conclusions: Visual rehabilitation in Peters anomaly remains a challenge, but outcomes can be optimized using a comprehensive clinical management algorithm according to disease severity., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: To determine the prevalence of survival of corneal grafts and visual outcomes of primary penetrating keratoplasty (PK) in infants with Peters anomaly (PA) in Beijing, China., Methods: Twenty-nine patients (37 eyes) with PA who underwent PK before the age of 1 year were included. Optical correction for all eyes and occlusion therapy of amblyopia for a unilateral opacity were performed 2 weeks after suture removal. All infants underwent assessment of visual acuity after surgery using Teller Acuity Cards. Survival probabilities were estimated using the Kaplan-Meier method and log-rank test. Visual outcomes and prognosis factors were analyzed using the χ2 test., Results: The mean age of 29 infants undergoing primary PK was 5.7 ± 2.3 months. The mean follow-up duration was 18.0 ± 3.0 months. Twenty-seven (73.0%) of 37 grafts retained full clarity at final follow-up. Visual acuity above ambulatory was achieved in 67.6% (25/37) and >20/260 was achieved in 48.6% (18/37) of cases. Of all surgical indications, vascularized PA I (50.0%, 6/12) and PA II (18.2%, 2/11) showed a lower proportion achieving visual acuity >20/260 than nonvascularized PA I (71.4%, 10/14) (P = 0.030 < 0.05). There was no significant difference in the prevalence of graft survival and vision outcome between infants younger than 6 months and older than >6 months., Conclusions: For infants with PA who underwent PK, the prevalence of graft survival and visual acuity were related mainly to the indication. The main risk factors were corneal vascularization and an abnormal lens., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc.)

Variants in the LIM homeobox transcription factor 1-beta ( LMX1B ) gene predispose individuals to elevated intraocular pressure (IOP), a key risk factor for glaucoma. However, the effect of LMX1B mutations varies widely between individuals. To better understand the mechanisms underlying LMX1B-related phenotypes and individual differences, we backcrossed the Lmx1b V265D (also known as Lmx1b Icst ) allele onto the C57BL/6J (B6), 129/Sj (129), C3A/BLiA- Pde6b + /J (C3H) and DBA/2J- Gpnmb + (D2-G) mouse strain backgrounds. Strain background had a significant effect on the onset and severity of ocular phenotypes in Lmx1b V265D/+ mutant mice. Mice of the B6 background were the most susceptible to developing abnormal IOP distribution, severe anterior segment developmental anomalies (including malformed eccentric pupils, iridocorneal strands and corneal abnormalities) and glaucomatous nerve damage. By contrast, Lmx1b V265D mice of the 129 background were the most resistant to developing anterior segment abnormalities, had less severe IOP elevation than B6 mutants at young ages and showed no detectable nerve damage. To identify genetic modifiers of susceptibility to Lmx1b V265D -induced glaucoma-associated phenotypes, we performed a mapping cross between mice of the B6 (susceptible) and 129 (resistant) backgrounds. We identified a modifier locus on Chromosome 18, with the 129 allele(s) substantially lessening severity of ocular phenotypes, as confirmed by congenic analysis. By demonstrating a clear effect of genetic background in modulating Lmx1b -induced phenotypes, providing a panel of strains with different phenotypic severities and identifying a modifier locus, this study lays a foundation for better understanding the roles of LMX1B in glaucoma with the goal of developing new treatments., Competing Interests: Competing interestsThe authors declare no competing or financial interests., (© 2021. Published by The Company of Biologists Ltd.)

Purpose: Convergence plays a fundamental role in the performance of near visual tasks. We measured the effect of two levels of convergence on anterior scleral thickness and shape in emmetropes, low to moderate myopes and high myopes., Methods: Forty-five healthy young adults aged between 18 and 35 years including 15 emmetropes, 15 low/moderate myopes, and 15 high myopes were recruited. Anterior segment optical coherence tomography and eye surface profilometry were used to evaluate the anterior scleral thickness (nasal only, n = 42) and shape (n = 40), before and during two visual tasks involving 9° and 18° convergence, in those participants with complete and reliable data., Results: Convergence led to a thickening of the total anterior eye wall (5.9 ± 1.4 μm) and forward movement (10 ± 2 μm) of the nasal anterior scleral surface (both p < 0.001). Larger changes were found at 18° than at 9° convergence and in more peripheral nasal scleral regions. There was a significant association between total wall thickening and forward movement of the scleral surface. Refractive group was not a significant main effect, but there were significant interactions between refractive group and the thickness changes with convergence in different scleral regions., Conclusion: During convergence, the biomechanical forces acting on the eye lead to nasal anterior scleral thickening and forward movement of the nasal scleral surface., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Objective: The aim of this study was to evaluate the effects of goniotomy on patients with primary angle-closure glaucoma who underwent "phacoemulsification and visco-goniosynechialysis.", Materials and Methods: This prospective randomized clinical trial was carried out on 63 eyes of 61 patients (48-85 years) with primary angle-closure glaucoma who were enrolled. The subjects were randomly assigned into two groups. In the first group (32 eyes) and the second group (31 eyes), the patients underwent "phacoemulsification + visco-goniosynechialysis + goniotomy" and "phacoemulsification + visco-goniosynechialysis," respectively. Intraocular pressure and antiglaucoma medication were evaluated (1 week, as well as 1, 3, and 6 months after the surgery). Anterior segment optical coherence tomography parameters (Casia, Tomey, USA) and goniotomy were evaluated preoperatively and 6 months after the surgery., Results: The mean intraocular pressure lowering the effects in the "phacoemulsification + visco-goniosynechialysis + goniotomy" group was higher than that in the "phacoemulsification + visco-goniosynechialysis" group (6.93 and 4.6, respectively). Furthermore, the difference in intraocular pressure between the two groups was significant at 3 months (P = 0.014) and 6 months (P = 0.021) after the surgery. There was no difference in anterior segment optical coherence tomography findings before the intervention; however, after the intervention, the anterior segment optical coherence tomography indices were significantly different between the two groups. Moreover, the difference in "phacoemulsification + visco-goniosynechialysis + goniotomy" indices was more than the changes in the "phacoemulsification + visco-goniosynechialysis" group., Conclusion: The results of this study showed that phacoemulsification + visco-goniosynechialysis + goniotomy is a more effective surgery than phacoemulsification + visco-goniosynechialysis alone in undercounted primary angle-closure glaucoma.

Purpose: To identify the genetic cause in a four-generation Chinese family with Axenfeld-Rieger syndrome (ARS)., Methods: The family members received clinical examinations of the eye, tooth, periumbilical skin, and heart. Sanger sequencing and whole-exome sequencing (WES) were performed to screen potential mutations. The genomic deletion region around the PITX2 gene was estimated from single nucleotide polymorphism (SNP) data from WES and then confirmed with "quantitative PCR (qPCR) using a set of primers. The DNA breakpoint was further identified with long-range PCR and Sanger sequencing., Results: Symptoms including anterior segment dysplasia of the eye (iris dysplasia, multiple pupils, and posterior embryotoxon), dental dysplasia, and periumbilical skin redundancy were present in all of the affected individuals. Three of them had glaucoma. Corneal abnormalities (inferior sclerocornea, corneal endothelial dystrophy, and central corneal scar) were seen in most of the affected individuals. Cataract, limited eye movement, electrocardiographic abnormalities, intellectual disability, and recurrent miscarriages were observed in some of the affected individuals. No mutations in the coding and exon-intron adjacent regions of the PITX2 and FOXC1 genes were identified with Sanger sequencing. According to the SNP data from WES, we suspected that there might be a deletion region (at most 1.6 Mb) around the PITX2 gene. With the use of qPCR and long-range PCR, we identified a 53,840 bp deletion (chr4: 111,535,454-111,588,933) spanning PITX2 and PANCR . The genomic deletion cosegregated with the major ARS symptoms observed in the family members., Conclusions: With the help of WES, qPCR, and long-range PCR, we identified a genomic deletion encompassing PITX2 and the adjacent noncoding gene PANCR in a Chinese family with ARS. The clinical features of the affected individuals are reported. This work may broaden understanding of the phenotypic and mutational spectrums related to ARS., (Copyright © 2020 Molecular Vision.)

Purpose: Studies examining the anterior chamber angle and angle closure disease often compare quantitative angle information obtained using anterior segment optical coherence tomography (ASOCT) with one of several ordinal scales derived using gonioscopy. We test the assumption that the ordinal gonioscopic angle grades have equal step sizes and can be analysed using metric statistics., Methods: The medical records of 214 consecutive patients who were referred for assessment of the anterior chamber angle were prospectively examined using gonioscopy and ASOCT (Spectralis Optical Coherence Tomography, OCT, www.heidelbergengineering.com). Anterior chamber angle parameters (angle opening distance, AOD, and trabecular-iris space area, TISA at 500 and 750 microns) were extracted from ASOCT images using a semi-automated segmentation algorithm written on MATLAB (www.mathworks.com). We first matched the quantitative values for each gonioscopic grade (0-4, from no structures visible to ciliary body visible) and described the frequency distributions to determine separability. We then applied a grade-agnostic clustering algorithm to determine the concordance between algorithm-clustered groups (using solely quantitative data) and those obtained using gonioscopy., Results: The frequency distributions of the quantitative ASOCT parameters for each angle grade were mostly non-parametric and displayed unique distribution characteristics, with a floor effect seen for grade 0 and the lack of a ceiling effect seen for grades 3 and 4. Although we found significant differences in quantitative values across the five angle grades using the frequency distributions, some pairwise comparisons were indistinguishable (such as grades 0 and 1, and grades 3 and 4) due to the overlaps in distributions. On average, differences in quantitative values were consistent between gonioscopic grade steps, but there remained substantial variability that confounds prediction of change between ordinal steps. The clustering algorithm showed approximately 10% of cases with the same group assignment as that of the gonioscopic grade, improving slightly to 30% when the top 5% of quantitative data were excluded from analysis., Conclusions: Our results do not necessarily support the assumption that the ordinal scales used in gonioscopy can be interpreted using an interval scale. We highlight the need for better methods of describing the course and risk of angle closure spectrum disease to identify disease progression and conversion, where gonioscopy remains the gold standard., (© 2020 The Authors Ophthalmic & Physiological Optics © 2020 The College of Optometrists Ophthalmic & Physiological Optics.)

Segmental flow in the human trabecular meshwork is a well-documented phenomenon but in depth mechanistic investigations of high flow (HF) and low flow (LF) regions are restricted due to the small amount of tissue available from a single donor. To address this issue we have generated and characterized multiple paired HF and LF cell strains. Here paired HF and LF cell strains were generated from single donors. Cells were characterized for growth and proliferation, as well as gene and protein expression of potential segmental region markers. Cells isolated from HF and LF regions have similar growth and proliferation rates. Gene expression data reveals vascular cell adhesion protein 1 (VCAM1), thrombospondin 2 (THBS2), and tissue inhibitor of metalloproteinase 1 (TIMP1) are potential markers of LF cells in vitro. Protein expression of VCAM1, THBS2 and TIMP1 are complex and may reflect the dynamic nature of the TM. Initial protein expression levels of these genes is either similar between HF and LF cells (VCAM1, THBS2), or higher in HF compared to LF in some strains (TIMP1). However, after long term culture LF cells express higher levels of VCAM1, TIMP1 and THBS2 protein compared to HF cells. HF and LF cell strains are a powerful new tool that enable understanding segmental flow allowing for multiple experiments on the same genetic background., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Objective: The main purpose of this article is to demonstrate the co-occurrence of Axenfeld-Rieger anomaly and neuropsychiatric problems as clinical signs of genetically determined cerebral small vessel disease in two patients., Case Study: We report on two adolescent individuals with ocular anterior segment dysgenesis (Axenfeld-Rieger anomaly) presenting with neuropsychiatric symptoms. Both patients underwent cerebral magnetic resonance imaging showing white matter T2-hyperintensities involving different brain regions, suspective of cerebral small vessel disease. Genetic analysis revealed pathogenic mutations in the FOXC1 gene (patient 1) and the COL4A1 gene (patient 2), respectively., Conclusion: We report on the co-occurrence of ocular anterior segment dysgenesis (Axenfeld-Rieger anomaly) and neuropsychiatric symptoms as clinical signs of genetically determined cerebral small vessel disease in two patients. In both patients, the cerebral lesions involved the frontotemporal regions, brain regions that control social behavior as well as executive and cognitive function, highlighting the fact that neuropsychiatric symptoms may be early clinical presentations of cerebral small vessel disease. We further provide a review of monogenic causes of pediatric cerebral small vessel disease, emphasizing the links to childhood-onset neuropsychiatric disease., Competing Interests: Dr. Ziegler reports personal fees from Biogen, personal fees from Avexis, and personal fees from PTC, outside the submitted work., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: The surgical management of glaucoma associated with Axenfeld-Rieger Syndrome (ARS) is poorly described in the literature. The goal of this study is to compare the effectiveness of various glaucoma surgeries on intraocular pressure (IOP) management in ARS., Methods: Retrospective cohort study at a university hospital-based practice of patients diagnosed with ARS between 1973 and 2018. Exclusion criterion was follow-up less than 1 year. The number of eyes with glaucoma (IOP ≥ 21 mmHg with corneal edema, Haabs striae, optic nerve cupping or buphthalmos) requiring surgery was determined. The success and survival rates of goniotomy, trabeculotomy±trabeculectomy (no antifibrotics), cycloablation, trabeculectomy with anti-fibrotics, and glaucoma drainage device placement were assessed. Success was defined as IOP of 5-20 mmHg and no additional IOP-lowering surgery or visually devastating complications. Kaplan-Meier survival curves and the Wilcoxon test were used for statistical analysis., Results: In 32 patients identified with ARS (median age at presentation 6.9 years, 0-58.7 years; median follow-up 5.4 years, 1.1-43.7 years), 23 (71.9%) patients were diagnosed with glaucoma at median age 6.3 years (0-57.9 years). In glaucomatous eyes (46 eyes), mean IOP at presentation was 21.8 ± 9.3 mmHg (median 20 mmHg, 4-45 mmHg) on 1.0 ± 1.6 glaucoma medications. Thirty-one eyes of 18 patients required glaucoma surgery with 2.2 ± 1.2 IOP-lowering surgeries per eye. Goniotomy (6 eyes) showed 43% success with 4.3 ± 3.9 years of IOP control. Trabeculotomy±trabeculectomy (6 eyes) had 17% success rate with 14.8 ± 12.7 years of IOP control. Trabeculectomy with anti-fibrotics (14 eyes) showed 57% success with 16.5 ± 13.5 years of IOP control. Ahmed© (FP7 or FP8) valve placement (8 eyes) had 25% success rate with 1.7 ± 1.9 years of IOP control. Baerveldt© (250 or 350) device placement (8 eyes) showed 70% success with 1.9 ± 2.3 years of IOP control. Cycloablation (4 eyes) had 33% success rate with 2.7 ± 3.5 years of IOP control. At final follow-up, mean IOP (12.6 ± 3.8 mmHg, median 11.8 mmHg, 7-19 mmHg) in glaucomatous eyes was significantly decreased (p < 0.0001), but there was no difference in number of glaucoma medications (1.6 ± 1.5, p = 0.1)., Conclusions: In our series, greater than 70% of patients with ARS have secondary glaucoma that often requires multiple surgeries. Trabeculectomy with anti-fibrotics and Baerveldt glaucoma drainage devices showed the greatest success in obtaining IOP control.

Background: Axenfeld-Rieger syndrome (ARS) is a congenital disease with a series of developmental abnormalities, and no case of ARS with cataract and small cornea has been reported in previous studies. In the present report, we aimed to describe the diagnosis and phacoemulsification of an ARS patient with small cornea., Case Presentation: A 58-year-old Han Chinese male patient who was referred to Eye Center of the Second Affiliated Hospital of Zhejiang University Medical College was diagnosed with ARS. Systemic and ophthalmic examination and genetic testing were performed. The slit-lamp microscopic examination of anterior segment showed obvious nuclear cataract, iris lesions, and the abnormal cornea of both eyes with small transversal and longitudinal diameters. ARS with bilateral complicated cataract and small cornea was diagnosed. Microincision-phacoemulsification in combination with intraocular lens implantation was performed on his left eye. After successful surgery of his left eye, the best-corrected visual acuity (BCVA) was obviously improved from 2 to 0.5 (LogMAR). A transient elevation of intraocular pressure (IOP) was controlled with medication., Conclusions: Through genetic testing, a known pathogenic mutation NM&#95;153427.2:c.272G > A was detected on the PITX2 gene; and an unknown mutation NM&#95;001453.2:c.1063C > T was detected on FOXC1 gene. For the ARS patient with complicated cataract, the visual acuity was increased by phacoemulsificasion in combination with microincision.

Purpose: To observe the distributions and changes in angle kappa and angle alpha preoperatively and postoperatively in patients with cataract who were implanted with a multifocal intraocular lens (mIOL)., Setting: The First Affiliated Hospital of Northwest University, Shaanxi, China., Design: Prospective nonrandomized noncomparative case series., Methods: Eyes that underwent phacoemulsification were included. The magnitudes and orientations of angle kappa and angle alpha preoperatively and postoperatively were compared, respectively., Results: The study comprised 81 eyes of 70 patients. The magnitude of angle kappa significantly decreased after phacoemulsification. No significant differences were observed between preoperative and postoperative angle kappa in orientation, as well as between preoperative and postoperative angle alpha. The correlations between preoperative and postoperative angle kappa and angle alpha were significant. There were displacement vectors for angle kappa (0.15 ± 0.10) and angle alpha (0.12 ± 0.12) after phacoemulsification. Locations of angle kappa of right and left eyes were scattered mostly in the temporal side of corneal light reflection, whereas locations of angle alpha were well organized in the horizontal position on temporal sides of corneal light reflection., Conclusions: The distribution of angle alpha was more organized compared with angle kappa. Angle kappa may change after phacoemulsification. During preoperative evaluation for patients with cataract planning for mIOLs implanted angle alpha may be a more reliable and stable factor compared with angle kappa.

Purpose: The mechanism of ocular growth eludes us and research on vitreous chamber depth (VCD) is lacking. The purpose of this study was to evaluate the role of VCD and its ratio to axial length (AL) in relation to ocular biometry., Methods: This retrospective study of patients planned for cataract surgery was performed at a tertiary center. Data regarding AL, anterior chamber depth (ACD), lens thickness (LT), and central corneal thickness (CCT) of 640 eyes was noted. Anterior segment (AS) was measured as sum of CCT, ACD, and LT, while VCD was calculated as the difference between AL and AS. Correlation of VCD and VCD: AL with ocular biometry was the primary outcome measure. Three groups were formed on the basis of AL and Pearson correlation coefficient (R) was applied., Results: Mean VCD was 15.38+/-1.14 mm. Mean VCD: AL was 0.66+/-0.02. VCD had a very strong relation with AL (R = 0.9, P < 0.001) only, whereas VCD: AL had a good--strong relation with AL (R = 0.5, P < 0.001), AS (R = 0.7, P < 0.001), ACD (R = 0.3, P < 0.001), and LT (R = 0.5, P < 0.001). The relation of VCD: AL with AS was very strong across all groups (R ≤ -0.8, P < 0.001 in all groups). 85% of eyes in group with AL <22 mm had VCD: AL <0.67, conversely 85% of eyes with AL >24.5 mm had VCD: AL >0.67., Conclusion: : We found VCD to have the strongest relation with AL. VCD: AL was more consistent and showed a strong relation to ocular biometry across all ALs. This suggests the possible utility of the ratio VCD: AL while evaluating ocular growth, refractive status, and myopia-related complications., Competing Interests: There are no conflicts of interest.

Purpose: To assess the outcomes of intra-lenticular lens aspiration (ILLA) in paediatric cases with anterior dislocation of lens., Methods: A retrospective review of medical records of cases with anterior dislocation of the lens in children (age < 16 years) that underwent ILLA between June 2017 and May 2018 was performed. Corrected distance visual acuity (CDVA), intraocular pressure (IOP), and anterior segment findings were noted at presentation and follow-up. Surgical notes were reviewed for all cases. Post-operative central corneal thickness (CCT) and central macular thickness (CMT) were recorded., Results: Eleven eyes of eight patients with a median age of ten years underwent ILLA. There were four males and four females. The median duration of symptoms was 2 months, CDVA was 1.77 logMAR, and IOP was 16 mm of Hg. Ten eyes had corneo-lenticular touch with corneal oedema, and two had raised IOP at presentation. Homocystinuria (n = 2/8), Microspherophakia (n = 2/8), Marfan syndrome (n = 1/8), Buphthalmos (n = 1/8) and Ectopia lentis et pupillae (n = 1/8) were the identifiable causes for anterior dislocation. There were no intra-operative complications in any case. Immediate post-operative corneal oedema and raised IOP was observed in nine and three cases respectively and was treated with medical therapy. The median post-operative CDVA and IOP at 6-months was 1 logMAR and 15 mm of Hg respectively. The median CCT and CMT were 516 and 248 μm respectively. Five eyes developed a central corneal descemet scar., Conclusions: ILLA is a safe and effective technique for surgical removal of an anteriorly dislocated lens in paediatric cases.

A fundamental scheme in the organization of the early visual cortex is the retinotopic representation of the contralateral visual hemifield on each hemisphere. We determined the cortical organization in a novel congenital visual pathway disorder, FHONDA-syndrome, where the axons from the temporal retina abnormally cross to the contralateral hemisphere. Using ultra-high field fMRI at 7 T, the population receptive field (pRF) properties of the primary visual cortex were modeled for two affected individuals and two controls. The cortical activation in FHONDA was confined to the hemisphere contralateral to the stimulated eye. Each cortical location was found to contain a pRF in each visual hemifeld and opposing hemifields were represented as retinotopic cortical overlays of mirror-symmetrical locations across the vertical meridian. Since, the enhanced crossing of the retinal fibers at the optic chiasm observed in FHONDA has been previously assumed to be exclusive to the pigment-deficiency in albinism, our direct evidence of abnormal mapping in FHONDA highlights the independence of pigmentation and development of the visual cortex. These findings thus provide fundamental insights into the developmental mechanisms of the human visual system and underline the general relevance of the interplay of subcortical stability and cortical plasticity., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: To examine the efficacy and safety of yttrium-aluminium garnet (YAG) laser vitreolysis for the treatment of vitreous floaters., Methods: Consecutive adult patients with symptomatic vitreous floaters who attended Chongqing General Hospital from April to December 2016 were included in this prospective study. Patients had >3 mm between the vitreous opacity and retina/lens and acceptable quality peri-papillary Optical Coherence Tomography (OCT) images (i.e., signal strength ≥5). Those with history of glaucoma, severe cataracts, vitreous haemorrhages, retinal holes and/or macular disease were excluded from the study. Best corrected visual acuity (BCVA) and non-contact intraocular pressure (IOP) were measured before and for up to 6 months post-YAG laser vitreolysis. Anterior segment photography and peripapillary retinal nerve fibre layer (RNFL) measured by OCT were taken before and 6 months post-procedure. Questionnaires on patient satisfaction were completed 6 months post-procedure., Results: No statistically significant differences in BCVA and IOP were observed before or after treatment. Anterior segment photography showed that vitreous opacities partially or completely disappeared after YAG laser treatment. The thickness of RNFL in four directions (upper, below, nasal and temporal) was not statistically significant different before or 6 months after the procedure. 75% patients reported significant improvement and 25% reported moderate improvement., Conclusion: The results of this study conducted in a cohort of 30 Chinese patients showed that YAG laser vitreolysis was a well-tolerated and effective treatment for vitreous floaters. Randomised, controlled trials involving large numbers of participants monitored over an extended follow up period are required to confirm these results.

Purpose: Optical iridectomy creates a defect in the iris to allow an alternative clear visual axis in cases of central corneal opacities occluding the pupillary axis. The purpose of this study is to evaluate the outcomes of optical sector iridectomy in children with Peters anomaly., Methods: Retrospective case series. The medical records of all patients diagnosed with Peters anomaly who underwent optical iridectomy during the years 2002-2014 were reviewed. Data collection included surgical and visual acuity outcomes., Results: Twenty-nine eyes (22 patients) were included in the study. Mean age at the time of surgery was 15.6 ± 26.3 months. Eighteen (81.8%) patients had bilateral disease. No intraoperative complications occurred. A red reflex was obtained in 28 (96.6%) eyes after surgery. Mean visual acuity improved from 2.5 ± 0.3 to 1.8 ± 0.6 in logMAR (p < 0.001). Vision improved in 21 (72.4%) eyes, remained stable in 5 (17.2%) eyes, and deteriorated in 3 (10.3%) eyes. Postoperatively visual acuity improved significantly in the patients with the bilateral disease (p < 0.05), but not in the unilateral group (p = 0.056). Mean follow-up time was 41.6 ± 43.8 months. During the follow-up period, five (17.2%) eyes were diagnosed with glaucoma, two (6.9%) eyes underwent PK, one (3.4%) eye underwent an additional sector iridectomy, and one (3.4%) eye underwent keratoprosthesis., Conclusions: In this largest series published of optical iridectomy for Peters anomaly, it was found to be a safe procedure. Improvement in visual acuity is expected, particularly in bilateral cases. The utility of optical iridectomy in unilateral cases necessitates further studies.

Importance: Corneal hysteresis (CH) is a dynamic marker of structural and functional changes in the cornea associated with intraocular pressure (IOP) and central corneal thickness, but its utility in assessing primary angle closure suspects (PACS) has not been fully elucidated., Background: This study assesses if anterior segment measurements correlate with CH in PACS patients., Design: IRB-approved retrospective review of imaging and records at a university practice., Participants: Sixty-three eyes from 37 patients diagnosed as PACS, without other ocular pathology., Methods: Every eye underwent measurements including: ocular response analyzer (ORA), anterior segment optical coherence tomography (OCT) and a clinical evaluation. ORA measurements were correlated with other anatomic parameters using a mixed effects multivariable linear regression framework., Main Outcome Measures: ORA measurements included: CH, corneal resistance factor, Goldmann IOP (IOP g ) and corneal compensated IOP (IOP cc ). Anterior segment OCT measurements included: central corneal thickness (CCT OCT ), nasal and temporal peripheral corneal thicknesses (PCT1 and PCT2), anterior chamber depth (ACD), nasal angle to temporal angle distance (ATA) and temporal and nasal angle measurements. Clinical measurements included: central corneal thickness via pachymetry, IOP measured by Goldmann applanation, axial length by A-scan ultrasound and spherical equivalent., Results: CH was negatively correlated with IOP cc, and ACD CH was positively correlated with CCT OCT , CCT p , CRF, PCT1 and PCT2. Females were found to have lower CH. In multivariable regression controlling for gender, CCT, PCT, IOP and ACD, no correlation was seen between CH and anatomic measurements., Conclusions and Relevance: CH values in PACS do not correlate with anterior segment anatomy., (© 2017 Royal Australian and New Zealand College of Ophthalmologists.)

Purpose: To investigate the effects of ambient light level variation on spectral domain anterior segment optical coherence tomography (SD-ΟCT)-derived anterior chamber angle metrics in Caucasians versus Asians., Materials and Methods: Caucasian (n = 24) and Asian participants of Chinese ancestry (n = 24) with open angles on gonioscopy had one eye imaged twice at five strictly controlled, ambient light levels. Ethnicity was self-reported. Light levels were strictly controlled using a light meter at 1.0, 0.75, 0.5, 0.25, and 0 foot candle illumination levels. SD-OCT 5-line raster scans at the inferior 270° irido-corneal angle were measured by two trained, masked graders from the Doheny Image Reading Center using customized Image-J software. Schwalbe's line-angle opening distance (SL-AOD) and SL-trabecular iris space area (SL-TISA) in different light meter readings (LMRs) between the two groups were compared., Results: Baseline light SL-AOD and SL-TISA measured 0.464 ± 0.115mm/0.351 ± 0.110mm 2 and 0.344 ± 0.118mm/0.257 ± 0.092mm 2 , respectively, in the Caucasian and the Asian group. SL-AOD and SL-TISA in each LMR were significantly larger in the Caucasian group compared to the Asian group (p < 0.05). Despite this difference in angle size between the groups, there were no statistically significant differences in the degree of change in angle parameters from light to dark (% changes in SL-AOD or SL-TISA between the two groups were statistically similar with all p-values >0.3)., Conclusion: SL-based angle dimensions using SD-OCT are sensitive to changes in ambient illumination in participants with Caucasian and Asian ancestry. Although Caucasian eyes had larger baseline angle opening under bright light conditions, the light-to-dark change in angle dimensions was similar in the two groups.

Purpose: To evaluate the effect of the Valsalva maneuver (VM) on anterior segment parameters and its association with refractive status., Method: In this prospective study, 120 eyes of 120 subjects were evaluated. Subjects were divided into three groups: myopic (n = 40 patients), hyperopic (n = 40 patients), and emmetropic (control group, n = 40 subjects). The anterior segment parameters, including keratometric, pachymetric, and anterior chamber parameters, were measured using the Sirius System (Costruzione Strumenti Oftalmici, Florence, Italy) before and during VM., Results: There was no statistically significant difference in corneal parameters including keratometric and pachymetric values at rest or during VM in myopic, hyperopic, and control groups (p > 0.05 for all). In the myopic and control groups, anterior chamber depth, anterior chamber volume (ACV), and anterior chamber angle (ACA) were significantly decreased during VM. In the hyperopic group, only anterior chamber depth was significantly decreased during VM, while ACV and ACA did not significantly change., Conclusion: The Valsalva maneuver may cause significant narrowing in anterior chamber depth, anterior chamber volume, and anterior chamber angle in the myopic and emmetropic groups, whereas it may affect only anterior chamber depth in the hyperopic group.

The PITX2 (paired-like homeodomain 2) gene encodes a bicoid-like homeodomain transcription factor linked with several human disorders. The main associated congenital phenotype is Axenfeld-Rieger syndrome, type 1, an autosomal dominant condition characterized by variable defects in the anterior segment of the eye, an increased risk of glaucoma, craniofacial dysmorphism and dental and umbilical anomalies; in addition to this, one report implicated PITX2 in ring dermoid of the cornea and a few others described cardiac phenotypes. We report three novel PITX2 mutations-c.271C > T, p.(Arg91Trp); c.259T > C, p.(Phe87Leu); and c.356delA, p.(Gln119Argfs*36)-identified in independent families with typical Axenfeld-Rieger syndrome characteristics and some unusual features such as corneal guttata, Wolf-Parkinson-White syndrome, and hyperextensibility. To gain further insight into the diverse roles of PITX2/pitx2 in vertebrate development, we generated various genetic lesions in the pitx2 gene via TALEN-mediated genome editing. Affected homozygous zebrafish demonstrated congenital defects consistent with the range of PITX2-associated human phenotypes: abnormal development of the cornea, iris and iridocorneal angle; corneal dermoids; and craniofacial dysmorphism. In addition, via comparison of pitx2M64* and wild-type embryonic ocular transcriptomes we defined molecular changes associated with pitx2 deficiency, thereby implicating processes potentially underlying disease pathology. This analysis identified numerous affected factors including several members of the Wnt pathway and collagen types I and V gene families. These data further support the link between PITX2 and the WNT pathway and suggest a new role in regulation of collagen gene expression during development.

Purpose: To evaluate the magnitude and axis orientation of the anterior, posterior, and total corneal astigmatism in normal healthy eyes of an Iranian population., Methods: In a prospective cross-sectional study, ophthalmic and anterior segment parameters of 153 healthy eyes of 153 subjects were evaluated by Galilei dual Scheimpflug analyzer. The magnitude and axis orientation [with-the-rule (WTR), against-the-rule (ATR), and oblique] of the anterior, posterior, and total corneal astigmatism measurements (ACA, PCA, and TCA) were compared according to the age, sex, and other ophthalmic parameters., Results: The mean ± SD age of the study population was 30 ± 5.9 years. The mean magnitude was 1.09 ± 0.76 diopters (D) for ACA, 0.30 ± 0.13 D for PCA, and 1.08 ± 0.77 D for TCA. Males had a significantly higher magnitude of PCA than females (p = 0.041). Most eyes had a WTR anterior astigmatism and an ATR posterior astigmatism. The WTR astigmatism had a higher mean magnitude compared to the ATR and oblique astigmatism in all the astigmatism groups, with a significant difference in the ACA and TCA groups (p < 0.05). PCA magnitude exceeded 0.50 D in only 7.8% of the subjects. ACA, PCA, and TCA were significantly correlated with each other and also had a significant correlation with the anterior and posterior maximum corneal elevation measurements (p < 0.001)., Conclusion: The results of this study although are limited due to the small number of participants and confined to our demographics, provided information regarding a population that was not described before and may be helpful in obtaining optimum results in astigmatism correction in refractive surgery or designing new intraocular lenses.

Purpose: To compare the anterior segment parameters of patients with pseudoexfoliation syndrome, patients with pseudoexfoliation glaucoma, and normal subjects., Methods: This prospective, controlled, comparative study included 150 eyes of 150 patients. The patients were divided into the pseudoexfoliation syndrome group, the pseudoexfoliation glaucoma group, and the control group (50 patients in each group). Axial length, central corneal thickness, aqueous depth, anterior chamber depth, lens thickness, K1 and K2 keratometry values, and white to white distance measurements were obtained by optical biometry and compared between the groups., Results: The mean ages of the pseudoexfoliation syndrome, pseudoexfoliation glaucoma, and control patients were 62.18 ± 6.21, 61.80 ± 6.62, and 59.40 ± 6.89 years, respectively. There were no statistically significant differences between the groups in mean age or sex ratio (p>0.05). Mean central corneal thickness was statistically significantly greater, mean aqueous depth and anterior chamber depth were statistically significantly greater, and mean lens thickness was statistically significantly less in the control group than in the pseudoexfoliation syndrome and pseudoexfoliation glaucoma groups (p<0.05). Pairwise comparisons of the pseudoexfoliation syndrome group and the pseudoexfoliation glaucoma group revealed that there were no significant differences between these two groups in central corneal thickness, aqueous depth, anterior chamber depth, and lens thickness (p>0.017)., Conclusions: Patients with pseudoexfoliation glaucoma and pseudoexfoliation syndrome had greater lens thickness, shallower aqueous depth and anterior chamber depth, and less central corneal thickness than normal subjects. None of the anterior segment parameters differed between patients with pseudoexfoliation syndrome and patients with pseudoexfoliation glaucoma.

Purpose: Peters anomaly is the most common cause of congenital corneal opacities. Although conservative management is often ineffective except in mild cases, surgical intervention in the form of penetrating keratoplasty is fraught with unpredictability and often has unacceptable postoperative outcomes. As such, there is a need to explore alternative surgical interventions that may possibly improve the postoperative visual prognosis in these patients. In this report, we present a case of type 1 Peters anomaly treated by selective endothelial removal without corneal tissue transplantation., Methods: A case report with literature review., Results: A 21-month-old child, who presented with unilateral type 1 Peters anomaly, underwent selective endothelial removal without corneal tissue transplantation for the treatment of her condition. The patient demonstrated excellent anatomical and visual recovery after the procedure over a 1-year period. Her visual acuity had improved from 20/960 preoperatively to 20/30 during the latest review. Postoperative recovery was not complicated by the development of any sight-threatening complications, and she has been successfully weaned off all topical and systemic medications., Conclusions: Selective endothelial removal can potentially be used to treat cases of type 1 Peters anomaly.

Advances in anterior segment imaging have enhanced our ability to detect keratoconus in its early stages and characterize the pathologic changes that occur. Computerized corneal tomography has elucidated the alterations in shape of the anterior and posterior corneal surfaces and alterations in thickness as the disease progresses. Automated screening indices such as the keratoconus screening index were developed to assist in detecting keratoconus in suspicious cases. In vivo assessment of keratoconic corneas has revealed that compromised corneal biomechanics can now be measured clinically. Optical coherence tomography has demonstrated alterations in corneal epithelial thickness and distribution in keratoconus, has a role in assessing Descemet's membrane detachment in acute corneal hydrops (ACH) and the depth of the demarcation line following corneal collagen cross-linking. In vivo confocal microscopy has exhibited cellular changes that occur in keratoconus and provided insight into cellular events that may be related to the development of neovascularization in ACH., (© 2017 Royal Australian and New Zealand College of Ophthalmologists.)

Purpose: To report a case of coexistent Fuchs endothelial corneal dystrophy (FECD) and keratoconus (KCN) in which there was normalization of corneal topography after Descemet membrane endothelial keratoplasty (DMEK)., Methods: Retrospective medical record review., Results: Preoperative findings revealed a best-corrected visual acuity of 20/40 with -1.00 - 2.50 × 147, topographic maximum keratometry of 50.8 D with inferior steeping, and confluent guttae in the left eye. Medical record review revealed myopic shift, but little change in keratometry or corneal thickness over the previous 3 years. The patient developed epithelial edema with contact lens trial, highlighting endothelial dysfunction and eliminating the option of contact lenses for visual rehabilitation. Combined DMEK and cataract extraction with intraocular lens implantation was undertaken. Postoperatively, best-corrected visual acuity was 20/20 with only spherical correction. Elimination of stromal edema led to flattening of maximum anterior keratometry to 46.3 D and reduction of total corneal refractive power (TCRP) by 4.4 D. There was an unanticipated postoperative refractive error of +3.75 D consistent with this normalization of corneal topography., Conclusions: This is the first case report of the role of DMEK in normalizing corneal topography in coexistent FECD and KCN. The potential impact of DMEK on anterior curvature and TCRP must be considered in intraocular lens power calculation for cataract surgery in patients with FECD and KCN.

Rationale: Axenfeld-Rieger syndrome (ARS) is a rare autosomal dominant disorder with ocular anterior segment dysgenesis and systemic anomalies., Patient Concerns: A 28-year-old Chinese Han female was referred to Beijing Tongren Eye Center for progressive decrease of the visual acuity on her right eye in the past month., Diagnoses: The patient was diagnosed as ARS with retinal detachment based on series of ophthalmic examinations performed., Interventions: A pars plana vitrectomy was performed to manage the retinal detachment., Outcomes: Her best-corrected visual acuity was slightly improved after surgery., Lessons: ARS is a developmental defect of ocular anterior segment with various clinical manifestations which might cause misdiagnosis.

Purpose: To compare central corneal backscatter obtained from Scheimpflug images between patients with insulin-dependent and non-insulin-dependent diabetes mellitus (IDDM and NIDDM, respectively) and healthy controls., Methods: Seven patients with IDDM (7 eyes), eleven patients with NIDDM (11 eyes), and sixteen healthy subjects (16 eyes) were included in this pilot study. Scheimpflug imaging system (Pentacam, Oculus Inc., Germany) was used to obtain optical sections of the cornea. Seven meridians were analyzed for each eye, oriented from 70° to 110°. Optical density values for the central 3-mm and 5-mm zones of the cornea were obtained by image analysis using external software., Results: Corneal backscatter was significantly higher in the diabetic patients than in the controls for the central 3-mm (p=0.016) and 5-mm (p=0.014) zones. No significant differences in corneal backscatter were found between the IDDM and NIDDM groups for either zone (both p>0.05). In the NIDDM group, significant correlations were observed for both central zones between corneal backscatter and age (3 mm: r=0.604, p=0.025; 5 mm: r=0.614, p=0.022) and central corneal thickness (3 mm: r=0.641, p=0.017; 5 mm: r=0.671, p=0.012); this was not found in the IDDM group (p>0.05). The presence of diabetes showed a significant effect on central corneal backscatter (Kruskal-Wallis test, p<0.001)., Conclusions: Diabetic patients showed higher values of corneal light backscatter than healthy subjects. Corneal optical density analysis may be a useful tool for monitoring and assessing the ocular changes caused by diabetes.

Purpose: Peters anomaly (PA) is known to be a primary cause of congenital corneal opacity. The aim of this study is to report the long-term clinical course and visual achievement of patients with PA who did not undergo keratoplasty., Methods: This retrospective study involved 15 eyes of 9 infants with PA less than 5 months of age at initial presentation at the Kyoto Prefectural University of Medicine who were followed up without keratoplasty for more than 6 years after presentation. In each subject, visual acuity, intraocular pressure (IOP), presence of congenital glaucoma, and change of corneal opacity were retrospectively investigated., Results: At initial presentation, the mean patient age was 2.3 months (SD: 1.3; range: 0-4 mo), and the mean follow-up period was 7.9 years (SD: 1.8; range: 6.5-11.3 yrs). Of the 9 patients, 6 were affected bilaterally. Thirteen eyes of 7 patients were found to have glaucoma. Over the follow-up period, corneal opacity gradually decreased in 4 eyes, 1 eye each of 2 unilateral patients without glaucoma and both eyes of 1 bilaterally affected patient with good IOP control during the follow-up period. Visual acuity in the unilateral case with intensive amblyopia treatment gradually increased along with decreasing corneal opacity., Conclusions: The findings of this study show that a natural decrease of corneal opacity can occur in eyes with PA. In cases of unilateral PA without IOP elevation and no enlargement of the corneal diameter, strict amblyopia treatment is vital to obtain a favorable visual prognosis.

Purpose: To evaluate the efficacy of infrared (IR) ocular thermography in screening for dry eye disease (DED)., Methods: IR ocular thermography was performed on 62 dry eye and 63 age- and sex-matched control subjects. Marking of ocular surface and temperature acquisition was done using a novel 'diamond' demarcation method. 30 static- and 30 dynamic-metrics were studied and receiver operating characteristic curves were plotted. Efficacy of the temperature metrics in detecting DED were evaluated singly and in combination in terms of their area under the curve (AUC), Youden's index and discrimination power (DP)., Results: Absolute temperature of the extreme nasal conjunctiva 5s and 10s after eye opening were best detectors for DED. With threshold value for the first metric set at 34.7°C, sensitivity and specificity was 87.1% (95% CI: 76.2-94.3%) and 50.8% (95% CI: 37.9-63.6%) respectively. With threshold value for the second metric set at 34.5°C, sensitivity and specificity was 77.6% (95% CI: 64.7-87.5%) and 61.9% (95% CI: 48.8-73.9%) respectively. The two metrics had moderate accuracy and limited performances with AUC of 72% (95% CI: 63-81%) and 73% (95% CI: 64-82%); Youden index of about 0.4 and DP of 1.07 and 1.05 respectively. None of the dynamic metrics was good detector for DED. Combining metrics was not able to increase the AUC., Conclusions: This work suggests some utility for the application of IR ocular thermography for evaluation of dry eye patients., (Copyright © 2016 British Contact Lens Association. Published by Elsevier Ltd. All rights reserved.)

Purpose: To compare the anterior segment parameters of patients with juvenile diabetes mellitus (DM) and healthy children by optical biometry., Methods: This prospective controlled clinical trial included 47 patients with juvenile type 1 DM and 50 age- and sex-matched healthy children. Central corneal thickness (CCT), aqueous depth (AD), lens thickness (LT), axial length, pupillary diameter (PD), K1 and K2 keratometry, and white to white distance (WTW) measurements were performed with optical biometry. The glycosylated hemoglobin (HbA1c) levels of the DM cases were obtained. Kolmogorov-Smirnov test, t test, χ2 test, and Pearson correlation test were used for statistical analysis., Results: The mean age of the 20 boys and 27 girls with DM was 10.91 ± 3.24 years and the mean age of the 29 healthy boys and 21 girls was 11.61 ± 3.6 years (age p = 0.42; sex p = 0.09). The mean LT was thicker (p = 0.001), the mean AD was lower (p = 0.001), and the mean PD was smaller (p = 0.001) in the DM cases and all were statistically significant. There was no significant difference between the groups for AU, CCT, WTW, or K1 and K2 (p = 0.12; p = 0.83; p = 0.54; p = 0.97; p = 0.21, respectively). We also found a significant negative correlation between HbA1c levels and PD (r = -0.37 p = 0.01)., Conclusions: Juvenile DM may affect anterior segment parameters and cause thicker LT, smaller PD, and lower AD. These effects may change the refractive status and should be considered during the examination of these children.

Purpose: To assess choroidal inflammation-related circulatory changes associated with the anterior recurrence of Vogt-Koyanagi-Harada (VKH) disease, using indocyanine green angiography (ICGA) and laser speckle flowgraphy (LSFG)., Methods: This retrospective case series included 17 eyes of 11 patients with VKH disease showing recurrent inflammatory findings in the anterior, but not posterior, segment (i.e. anterior recurrence). Indocyanine green angiography (ICGA) and LSFG were performed at the time of recurrence and one month after the initiation of corticosteroid therapy. The number and total area of hypofluorescent dark dots (HDDs) on ICGA were independently counted by three physicians and measured with ImageJ, respectively. Mean blur rate (MBR), a quantitative index of relative blood flow velocity, was calculated via the LSFG Analyzer software., Results: Hypofluorescent dark dots (HDDs) were identified on ICGA in 13 of 17 eyes (76%) with the anterior recurrence of VKH disease. The number and total area of HDDs significantly decreased from 203 ± 101 dots to 59 ± 51 dots and from 48 789 ± 24 251 pixels to 15 664 ± 13 254 pixels, respectively. The change ratio of MBR significantly increased by 17.9 ± 16.3% after the treatment. Importantly, there was no significant association between the change ratios of HDDs and MBR., Conclusions: These findings on LSFG and ICGA clearly demonstrated subclinical involvement as well as post-treatment improvement of choroidal circulation impairment due to granulomatous inflammation in eyes with the anterior recurrence of VKH disease. The present data suggest the validity of using these two examinations, capable of detecting different circulatory changes, in the management of recurrent VKH disease., (© 2016 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To evaluate the changes in anterior chamber angle (ACA) parameters in primary angle closure (PAC) spectrum eyes before and after cataract extraction (CE) and compare to the changes after laser peripheral iridotomy (LPI) using anterior segment optical coherence tomography (ASOCT)., Methods: Twenty-eight PAC spectrum eyes of 18 participants who underwent CE and 34 PAC spectrum eyes of 21 participants who underwent LPI were included. ASOCT images with 3-dimensional mode angle analysis scans were taken with the CASIA SS-1000 (Tomey Corp., Nagoya, Japan) before and after CE or LPI. Mixed-effect model analysis was used to 1) compare best-corrected visual acuity, intraocular pressure, and ACA parameters before and after CE; 2) identify and estimate the effects of potential contributing factors affecting changes in ACA parameters; and 3) compare CE and LPI treatment groups., Results: The increase in average angle parameters (TISA750 and TICV750) was significantly greater after CE than LPI. TICV750 increased by 102% (2.114 [±1.203] μL) after LPI and by 174% (4.546 [± 1.582] μL) after CE (P < 0.001). Change of TICV750 in the CE group was significantly affected by age (P = 0.002), race (P = 0.006), and intraocular lens power (P = 0.037)., Conclusions: CE results in greater anatomic changes in the ACA than LPI in PAC spectrum eyes. ASOCT may be used to follow anatomic changes in the angle after intervention., Competing Interests: I have read the journal's policy and the authors of this manuscript have the following competing interests: The CASIA SS-1000 was loaned to Dr. Feldman by the Tomey Corporation (Nagoya, Japan). Dr. Bell, Dr. Blieden, and Dr. Feldman are members of the Alcon Speaker Bureau and have received research funding from Alcon. Dr. Feldman is also a consultant for Alcon. No other authors declare any relevant conflicts of interest. This does not alter our adherence to PLOS ONE policies on sharing data and materials.

The purpose of this study is to investigate the process of corneal wound healing after penetrating injury with the change in optical intensity on anterior segment optical coherence tomography (AS-OCT) and to investigate factors associated with severity of corneal scar. Forty-seven eyes from 47 patients with repaired corneal laceration were included. AS-OCT was performed on 1day, 1week, 1, 3 and 6 months after primary repair. Internal aberrations of wound edges were observed on AS-OCT images. Parameters including height of steps, width of gaps, maximal corneal thickness, area and optical intensity of corneal wound/scar were measured. The relationship between the parameters at day 1 and the optical intensity at 6 months were analyzed. The results showed that optical intensity of corneal wound/scar increased from 124.1 ± 18.8 on day 1 postoperatively to 129.3 ± 18.7, 134.2 ± 23.4, 139.7 ± 26.5, 148.2 ± 26.4 at 1 week, 1 month, 3 months and 6 months postoperatively. Height of steps at 1 day after surgery was the only factor identified as correlated with optical intensity of corneal scar at 6 months (beta = 0.34, p = 0.024). The increase of optical intensity represents the process of fibrosis of corneal wound healing. Higher step after suturing is associated with more severity of corneal scar at last.

Anterior segment dysgenesis (ASD) encompasses a group of developmental disorders in which a closed angle phenotype in the anterior chamber of the eye can occur and 50% of patients develop glaucoma. Many ASDs are thought to involve an inappropriate patterning and migration of the periocular mesenchyme (POM), which is derived from cranial neural crest cells (NCCs) and mesoderm. Although, the mechanism of this disruption is not well understood, a number of transcriptional regulatory molecules have previously been implicated in ASDs. Here, we investigate the function of the transcription factor AP-2β, encoded by Tfap2b, which is expressed in NCCs and their derivatives. Wnt1-Cre-mediated conditional deletion of Tfap2b in NCCs resulted in post-natal ocular defects typified by opacity. Histological data revealed that the conditional AP-2β NCC knockout (KO) mutants exhibited dysgenesis of multiple structures in the anterior segment of the eye including defects in the corneal endothelium, corneal stroma, ciliary body and disruption in the iridocorneal angle with adherence of the iris to the cornea. We further show that this phenotype leads to a significant increase in intraocular pressure and a subsequent loss of retinal ganglion cells and optic nerve degeneration, features indicative of glaucoma. Overall, our findings demonstrate that AP-2β is required in the POM for normal development of the anterior segment of the eye and that the AP-2β NCC KO mice might serve as a new and exciting model of ASD and glaucoma that is fully penetrant and with early post-natal onset., Competing Interests: The authors declare no competing or financial interests., (© 2016. Published by The Company of Biologists Ltd.)

PurposeTo determine the long-term surgical and visual outcomes in Indian children with early-onset glaucoma associated with Axenfeld-Rieger anomaly (ARA).MethodsThis is a retrospective analysis of 44 eyes of 24 consecutive children with early-onset glaucoma (within 3 years of age) and ARA who underwent glaucoma surgery over a 20-year period (1991-2010) by a single surgeon. Main outcome measures were pre- and postoperative intraocular pressures (IOPs), corneal clarity, visual acuities (VAs), refractive errors, success rate, time of surgical failure, and complications.ResultsThe series consisted of 38 primary combined trabeculotomy-trabeculectomy (CTT) and 6 primary trabeculectomy procedures (Schlemm's canal could not be identified in these eyes). There was a statistically significant reduction in IOP postoperatively (27.07±4.88 vs 14.88±3.62 mm Hg; P<0.0001) with a mean reduction of 45.14%. Success probability by Kaplan-Meier survival analysis was 93% till 5 years, and then 88.1%, 82.3%, 70.5%, 56.4%, and 42.3% at year 6, 7, 8, 9, and 10, respectively. Preoperative corneal edema was present in 43/44 eyes (97.72%) and cleared in 42 eyes (97.67%). There was one case each with intraoperative hyphema and with shallow chamber postoperatively and both were successfully managed successfully. There was no incidence of endophthalmitis or any other sight-threatening complication. Data on VA were available in 34 eyes (77.3%). At final follow-up visit, 15 (44.1%) eyes had best corrected VA ⩾6/18.ConclusionsPrimary CTT is safe and effective for early-onset glaucoma associated with ARA. It leads to excellent IOP control and satisfactory visual outcome.

Purpose: To assess the effects of phacoemulsification, intraocular lens implantation, and goniosynechialysis on patients with primary angle-closure glaucoma and cataract., Methods: A total of 145 eyes of 133 consecutively recruited patients were randomly divided into Phaco group and Trab group. Ocular parameters were measured before and after surgery., Results: The mean follow-up period was 13.2±5.6 months. The visual acuity in the Phaco group was significantly improved, whereas no alternation was found in the Trab group after surgery. The intraocular pressure (IOP) had no notable difference in 2 groups before surgery. Compared with preoperative IOP, there was significant decrease after surgery. No remarkable change in the IOP was measured in the 2 groups after surgery for 12 months. The anterior chamber depth was markedly augmented in the Phaco group compared with the Trab group after surgery for 3 months. Compared with the the Trab group, no obvious change in coefficient of outflow facility was found in the Phaco group before and after surgery, whereas there was a significant increase relative to its preoperation. The angle closure decreased from (290±25) to (60±35) degrees after surgery for 12 months in the Phaco group and no significant alteration was observed in the Trab group. The reduction extent of peripheral anterior synechiae was in direct proportion to the decreasing level of angle closure in the 2 groups. The change in the corneal endothelial cell density had no significant difference between 2 groups before and after surgery., Conclusions: The treatment of phacoemulsification, intraocular lens implantation, and goniosynechialysis is safe and effective for patients with primary angle-closure glaucoma and cataract.

Purpose: To evaluate irregularity of the anterior cornea before and after Descemet stripping endothelial keratoplasty (DSEK) for bullous keratopathy and its effect on visual acuity., Methods: Corneal data were acquired using a topographic unit before and up to 12 months after DSEK. Anterior corneal elevation data were decomposed into a set of Zernike polynomials up to the 8th order within a 6.0-mm diameter region. Total higher-order aberrations (HOAs) and root mean square from the 3rd to 8th order were calculated. The effects of anterior surface irregularity on visual acuity were evaluated., Results: This clinical study comprised 20 consecutive eyes of 20 patients with bullous keratopathy. The mean corrected distance visual acuity (CDVA) was 1.00 ± 0.12 logMAR (SD) preoperatively and 0.24 ± 0.16 logMAR 12 months postoperatively. Before DSEK, corneas with bullous keratopathy had higher total HOAs compared with those in controls (p&lt;0.05). There were no significant differences in anterior surface HOAs between preoperatively and 12 months (p&gt;0.05)., Conclusions: Postoperative CDVA correlated with irregularity of the corneal anterior surface. Corneal anterior HOAs are higher in patients with bullous keratopathy than in controls, and remain higher through 12 months after DSEK. In addition to corneal transparency, regularity of the corneal anterior surface is an important factor in visual acuity after DSEK.

Purpose: To investigate the effects of dehydration due to fasting on diurnal changes of intraocular pressure, anterior segment biometrics, and refraction., Subjects and Methods: The intraocular pressures, anterior segment biometrics (axial length: AL; Central corneal thickness: CCT; Lens thickness: LT; Anterior chamber depth: ACD), and refractive measurements of 30 eyes of 15 fasting healthy male volunteers were recorded at 8:00 in the morning and 17:00 in the evening in the Ramadan of 2013 and two months later. The results were compared and the statistical analyses were performed using the Rstudio software version 0.98.501. The variables were investigated using visual (histograms, probability plots) and analytical methods (Kolmogorov-Smirnov/Shapiro-Wilk test) to determine whether or not they were normally distributed., Results: The refractive values remained stable in the fasting as well as in the control period (p = 0.384). The axial length measured slightly shorter in the fasting period (p = 0.001). The corneal thickness presented a diurnal variation, in which the cornea measured thinner in the evening. The difference between the fasting and control period was not statistically significant (p = 0.359). The major differences were observed in the anterior chamber depth and IOP. The ACD was shallower in the evening during the fasting period, where it was deeper in the control period. The diurnal IOP difference was greater in the fasting period than the control period. Both were statistically significant (p = 0.001). The LT remained unchanged in both periods., Conclusions: The major difference was shown in the anterior chamber shallowing in the evening hours and IOP. Our study contributes the hypothesis that the posterior segment of the eye is more responsible for the axial length alterations and normovolemia has a more dominant influence on diurnal IOP changes.

The article describes the potential effects of vitrectomy on clinical refraction, refraction of the cornea and its biomechanical parameters, the state of the lens, intraocular pressure, and biometric parameters of the anterior eye segment.

No study has so far evaluated the impact of coffee drinking on ocular wavefront aberration (OWA) measurements. This study presents novel findings regarding the OWA of the eye following coffee intake. We aimed to evaluate the acute changes in pupil size and OWA of the eye after single administration of coffee. A total of 30 otherwise healthy participants were included in this prospective study. All subjects drank a cup of coffee containing 57 mg caffeine. Measurements of pupil size, total coma (TC), total trefoil (TF), total spherical aberration (TSA), and total higher order aberration (HOA) were performed before and at 5 minutes, at 30 minutes, and at 4 hours after coffee drinking using a wavefront aberrometer device (Irx3, Imagine Eyes, Orsay, France). The mean age of the study population was 20.30 ± 2.74 years. Pupil size did not show a significant change during the measurements (p > 0.05). A significant increase was observed in TF and HOA measurements following coffee intake (p = 0.029 and p = 0.009, resp.). Single administration of coffee results in significant increase in TF and total HOAs in healthy subjects without any effect on pupil diameter. Ultrastructural changes in the cornea following coffee intake might be of relevance to the alterations in ocular aberrations in healthy subjects.

Purpose: The aim of this study was to evaluate the difference between intraocular pressure (IOP) measurements in children with the Icare tonomer (IT) and applanation (AT), pneumatic (PT), or digital tonometers (TT)., Design: A randomized prospective trial of children younger than age 16 attending the pediatric ophthalmology department of Manchester Royal Eye Hospital was conducted., Methods: Children had IOP measured twice, once with an IT and again with a TT, PT, or AT during the same clinic appointment., Results: Forty-four children (88 eyes) were included, with a mean [range (R)] age of 57 (2-144) months. Twelve eyes had anterior segment pathology (ASP), defined as aniridia, congenital glaucoma, or Peters anomaly. Regardless of the presence or absence of ASP, total mean difference (MD), R, positive bias (PB), and limits of agreement (LOA) between IT and other instruments were as follows: PT: MD = 3.2, R = 0 to 8, PB = 2.9, LOA = -1.0 to 6.9; TT: MD = 2.6, R = 0 to 6, PB = 0.9, LOA = -1.8 to 3.5; and AT: MD = 1.4, R = -3 to 5, PB = 0.6, LOA = -4.2 to 7.3. In eyes with ASP, IT comparisons with PT were as follows: MD = 3.9, R = 0 to 8, PB = 3.9, LOA = -0.9 to 8.8., Conclusions: In children with ASP, IOP measured with IT is higher than expected when compared with other tonometers, in some cases by up to 8 mm Hg. We found an overestimation of IOP in children using the IT with a positive bias of 4 mm Hg. We propose that the IT overestimates IOP measurements in children compared with standard tonometers. Further work should be carried out on a much larger cohort to establish a suitable correction factor for such children.

Purpose: To assess age, refractive error and gender related changes occurring in the cornea and the anterior segment of the eye using a Scheimpflug system., Methods: The study included 666 healthy eyed subjects with a mean age of 39.3±19.7 years (range: 3-85 years). All analyses were based on the right eyes of the patients as all measured parameters correlated well between the right and left eyes. Each parameter was correlated with age and the right eye's spherical equivalent (SE) using Pearson correlations. Univariate linear regression models were constructed for analyses of parameters., Results: The anterior corneal surface asphericity showed significant positive correlations whereas posterior corneal surface asphericity showed significant negative correlations with age. Anterior chamber depth (ACD), volume (ACV) and angle (ACA) showed significant negative correlations with age and SE. Age explained 25% of the variance in anterior corneal surface asphericity, 22% of variance in posterior corneal surface asphericity, 26% of variance in ACV, 27% of variance in ACD, and 19% of variance in ACA. In the SE model SE was identified to account for 25% of variance in ACV, 22% of variance in ACD, each, and 17% of variance in ACA. Significant differences were detected in anterior and posterior keratometry values, ACV, ACD and ACA among gender groups (p<0.01)., Conclusions: The cornea shows a tendency for a decrease in anterior corneal surface asphericity and an increase in posterior corneal surface asphericity with advancing age. Men have flatter corneas and women have shallower anterior chambers and narrower anterior chamber angles., (Copyright © 2015 British Contact Lens Association. Published by Elsevier Ltd. All rights reserved.)

Purpose: To explore alternative experimental protocols to investigate the biomechanical behavior of the crystalline lens and zonules using ex vivo stretching., Methods: Radial stretching tests were conducted on the anterior segment (consisting of lens, zonules, ciliary body, and sclera) of four pairs of presbyopic human donor eyes. A simple mechanical model is used to describe the behavior of the anterior segment when tested in this way. Each pair of samples was initially stretched with the ciliary body intact. One sample was retested after cutting the ciliary body radially, and the other sample was retested after removing the lens., Results: The external forces needed to stretch the sample with the ciliary body intact were significantly greater than for the tests in which the ciliary body had been cut. The forces measured with the ciliary body intact and lens in situ were comparable to the sum of the forces measured in the tests in which the ciliary body had been cut (lens in situ) and the forces measured in the tests on the intact ciliary body with the lens removed., Conclusions: When stretching tests are conducted on the anterior segment, significant circumferential tensions develop in the ciliary body. This means that the forces applied to the lens and zonules cannot be related directly to the forces applied by the external loading system. If radial cuts are introduced in the ciliary body prior to testing, however, then this difficulty does not arise.