Hedgehog Signal Defect Leading to Familial Exudative Vitreoretinopathy-Like Disease and Gastrointestinal Malformation.

Objectives: The aim of the study was to present a new genetic association presenting with gastrointestinal tract malformations (GTMs) and familial exudative vitreoretinopathy (FEVR)-like disease and review the genetics of Hedgehog signaling. Materials and Methods: Three neonates were diagnosed with FEVR-like retinal vascular disease upon routine ophthalmological examination during hospitalization in the neonatal surgical intensive care unit for GTMs. Genetic analysis of the neonates was performed. Results: Gestational age of the neonates was 39, 38, and 39 weeks and birth weights were 3,500, 3,600, and 3,300 grams, respectively. All six eyes of the three infants were treated by laser photocoagulation. Recurrence was not seen in any of the eyes. Genetical analysis of all the neonates diagnosed with FEVR-like disease revealed defects in the Hedgehog pathway. Conclusion: FEVR is a genetically well-defined retinal vascular disease. The current study is the first to show an association between FEVR-like retinal vascular disease and GTMs. This study demonstrates the importance of the Hedgehog pathway in retinal vascular and gut development.

Effect of pseudoexfoliation syndrome on lamina cribrosa morphology after uneventful phacoemulsification.

AIM: To evaluate the behavior of the lamina cribrosa (LC) following successful phacoemulsification (PE) surgery in non-glaucomatous PXS cases with cataract. METHODS: A total of 25 bilateral PXS and 56 non-PXS cataract patients were included in this prospective, interventional, and controlled study. The lamina cribrosa thickness (LCt) and anterior laminar depth (ALD) were measured horizontally and vertically from two sections using EDI-OCT. The measurements were taken preoperatively, and the LCt and ALD measurements were repeated at week one (W1), month one (M1), month two (M2), and month three (M3) postoperatively. RESULTS: The baseline horizontal and vertical LCt values were found to be significantly thinner in the PXS group (p = 0.001, p = 0.006 respectively). The horizontal section ALD values were 463.32 ± 98.82 µm and 383.88 ± 85.8 µm (p < 0.001) for the PXS and non-PXS groups, respectively, and this significance continued during follow-up. The vertical ALD values were 469.64 ± 117.83 µm and 390.3 ± 98.1 µm (p = 0.002) in the PXS and non-PXS groups, respectively. There was no significant change in the PXS group for the depth following PE, but a statistically significant depth increase response was observed in the non-PXS group during follow-up. CONCLUSION: It is possible to conclude that the bending capacity of the lamina cribrosa may be lower than seen in the normal population, but we do not know how this lack of flexibility will affect the eye.

The effect of home education on myopia progression in children during the COVID-19 pandemic.

OBJECTIVES: To evaluate the effect of the COVID-19 pandemic restrictions on myopia progression (MP) in school age children. METHODS: A total of 115 children aged 8-17 years with a diagnosis of myopia who had been followed-up for at least three years, were included in this study with a retrospective and single-centre design. The subjects' age, the history of myopia in the family, the time spent in front of a screen, the digital devices used during home education (computer, tablet, smartphone, television), the time spent in open air (hours/day), the refractive error (RE) (spherical equivalent value) detected before the home education period and the changes in the myopia over the years, were recorded. RESULTS: The mean age was 12.06 (±2.29) years. Only the right eyes were included. The glasses use duration was 3.57 (±0.74) years. The annual MP amount 0.49 (±0.26), 0.41 (±0.36) and 0.54 (±0.43) dioptres (D) for the 2017, 2018 and 2019 years before home education, respectively, (p > 0.05), and 0.71 (±0.46) D in 2020, during home education. The increase in MP amount in 2020 compared to the 2019 and 2018 years was statistically significant (p < 0.003). MP was statistically significantly less in children who participated in open-air activities for 2 h a day and those who lived in detached houses (p = 0.004, p = 0.006, respectively). CONCLUSION: During home confinement, education programmes of school children should be designed while taking into account preventive measures for MP, in particular for allowing children to spend at least 2 h of outdoor time per day.

The Use of Fundus Autofluorescence in Dry Age-Related Macular Degeneration

Fundus autofluorescence (FAF) has been a well-known imaging method for quite some time. However, with developing technologies and novel imaging devices, FAF is being used more often to diagnose and monitor retinal diseases. The density of lipofuscin (LF) and other fluorophores in the retina have a determining role in FAF images. In dry age-related macular degeneration (AMD), hyperautofluorescence is seen in cases of increasing LF in the retina pigment epithelium, whereas hypoautofluorescence is detected in decreasing LF resulting from geographic atrophy. In recent years, studies have shown that FAF images provide prognostic information in patients with AMD. This review aims to highlight the importance of FAF imaging in dry AMD.

Impact of ocular and systemic risk factors on progression of geographic atrophy in age-related macular degeneration.

BACKGROUND: Geographic atrophy (GA) corresponds to an end-stage form of age-related macular degeneration and the leading cause of irreversible vision loss. The aim of this study was to investigate the role of fundus autofluorescence (FAF) patterns surrounding GA, baseline GA size, the presence of reticular pseudodrusen (RPD), the condition of the fellow eye and the presence of systemic vascular diseases (diabetes, atherosclerosis, systemic hypertension) on the progression of GA due to age-related macular degeneration. METHODS: Seventy-five eyes of 52 patients with GA were included into this retrospective study. Digital FAF images were achieved using scanning laser ophthalmoscopy. Areas of GA were measured with the Region Finder software program on an Heidelberg Retinal Angiograph 2. FAF patterns around GA were classified and the presence of RPD and systemic diseases were noted. The median follow-up of the patients was 2.6 years (interquartile range, 1-9.2). RESULTS: The median progression rate of the banded pattern (median 0.97 mm2/year) was significantly higher than the other patterns (median 0.85 mm2/year) (p = 0.03). Eyes with RPD had a significantly higher progression rate (median 1.21 mm2/year) than those without RPD (median 0.79 mm2/year) (p = 0.007). The presence of systemic diseases was not related with lesion progression rates. CONCLUSIONS: The results demonstrate that the banded pattern and the presence of RPD accelerate the progression of GA and function as predictive factors.

Favorable outcomes in the treatment of aggressive posterior retinopathy of prematurity.

OBJECTIVES: To report the results of intravitreal ranibizumab injection as primary therapy in aggressive posterior retinopathy of prematurity, the process of the disease, and the additive treatments performed. METHODS: This retrospective case review included 15 eyes of 8 premature babies with aggressive posterior retinopathy of prematurity who were initially treated with intravitreal ranibizumab injection. The documented data were gestational age, birth weight, gender, postmenstrual age at intravitreal ranibizumab injection, zone of retinopathy of prematurity, reactivation time of disease, iris neovascularization, retinal hemorrhage, anatomical outcome, and additional treatment. RESULTS: Median gestational age at birth was 26 (range, 23-27) weeks, birth weight was 730 (range, 550-970) g, and postconceptional age at aggressive posterior retinopathy of prematurity diagnosis and intravitreal ranibizumab injection was 35 (range, 33-35) weeks. Intravitreal ranibizumab injection was performed as primary treatment. Two eyes necessitated a second intravitreal ranibizumab injection. The reactivation of retinopathy of prematurity was 5 (range, 3-7) weeks after intravitreal ranibizumab injection. Recurrence of the disease in Zone II was treated with laser photocoagulation. A favorable outcome was obtained in all eyes (100%). CONCLUSION: Aggressive posterior retinopathy of prematurity is a serious, rapidly progressing form of retinopathy of prematurity that requires quick and proper management. This study indicates that primary treatment with ranibizumab and laser photocoagulation on recurrence provide favorable anatomical outcomes.

The relationship of central corneal thickness with the status of diabetic retinopathy.

BACKGROUND: To compare central corneal thickness (CCT) values measured by three different devices: slit-scanning topography (SST), ultrasonic pachymetry (UP), and optical coherence tomography (OCT) in diabetic eyes and compare the CCT values in patients with and without diabetic retinopathy. METHODS: Ninety-six patients with diabetes mellitus were included in this prospective study and divided into two groups according to the presence of diabetic retinopathy, as Group I with retinopathy and Group II without. The CCT of 96 eyes was measured by three different devices; SST (Orbscan II), UP and OCT. The results of CCT measurements with three different devices were compared. Also, the intergroup differences in CCT measurements were evaluated. RESULTS: The CCT was statistically insignificantly different between the two groups. Although the three methods of CCT measurements correlated well with each other, SST showed significantly (p < 0,0001) higher CCT results compared to both UP and OCT. CONCLUSIONS: According to our results, neither the duration of DM nor the presence of diabetic retinopathy did have a significant effect on the CCT. The CCT values obtained with three devices were all in correlation. However, the results of SST were significantly higher compared to the other two. Our findings emphasize the value anterior segment OCT in CCT measurements, since it is a non-contact method and correlate very well with UP.

Impact of Platelet Count in Retinopathy of Prematurity.

Objectives: The aim of the study was to investigate the risk factors for retinopathy of prematurity (ROP), including platelet count. Materials and Methods: This retrospective study analyzed 137 infants in 3 subgroups: no ROP; mild ROP, and severe ROP requiring laser treatment (type 1 ROP). A retrospective review of records was performed and statistical analysis of possible risk factors for ROP including platelet count was evaluated by using logistic regression. Results: Birth weight (BW), gestational age (GA), and low platelet count in the first week after birth were significant risk factors for developing ROP (p=0.038, 0.02, and 0.004, respectively). BW, GA, ventilation, and lower platelet count were associated with progression to type 1 ROP (p=0.004; 0.027, and 0.021, respectively). Conclusion: Lower platelet count in the first week after birth is a risk factor for ROP development in addition to the previously established factors of ventilation need, low BW, and low GA.

Effect of diluted povidone iodine in adenoviral keratoconjunctivitis on the rate of subepithelial corneal infiltrates.

AIM: To evaluate the clinical characteristics of adenoviral keratoconjunctivitis, the management modalities, as well as the incidence of subepithelial corneal infiltrates (SEI). METHODS: Patients with characteristic clinical symptoms and signs, who presented to our clinic within the first week of symptoms and received the diagnosis of adenoviral keratoconjunctivitis between January 2013 and April 2016, were included in the study. A total of 211 patients were included in the study. Patients were evaluated for the incidence of clinical signs, late complications, management preferences, and the effect of diluted povidone-iodine (d-PVP-I) 2%. RESULTS: Patients' mean age was 33.03±14.76y. We observed an increase in the number of cases according to the years. At presentation and/or early follow-up, the clinical signs were conjunctival hyperemia (100%), conjunctival follicules (79.1%), edema of the eyelids (39.3%), chemosis (16.1%), pseudomembrane formation (16.6%), and corneal epitheliopathy (29.9%). During late follow-up 13.3% patients developed conjunctival subepithelial fibrosis, and 39.8% developed SEI. A significant decrease in the incidence of SEI development was observed in patients who used d-PVP-I 2% (P=0.032; 33.3% vs 45.9%, respectively in patients who received d-PVP-I 2% and who did not). CONCLUSION: Adenoviral keratoconjunctivitis has a tremendous effect on patient's comfort and abilities in short-term. Additionally, almost half of the patients develop visual problems related to SEI. According to our clinical experience, using d-PVP-I 2% in the first days of adenoviral keratoconjunctivitis might be helpful in reducing the risk of SEI as a complication.

Effect of Latanoprost on Choroidal Thickness.

PURPOSE: The purpose of this study was to evaluate the changes in choroidal thickness during the use of latanoprost eyedrops which shift fluid to the suprachoroidal space and change choroidal perfusion. MATERIALS AND METHODS: This prospective study included 34 eyes of 17 bilateral glaucoma or ocular hypertensive patients who had no previous hypotensive therapy. Each patient had both eyes treated with latanoprost. Each patient also underwent a complete ophthalmological examination and optical coherence tomography measurement. Choroidal thickness measurements by optical coherence tomography were carried out on the first visit before latanoprost use and at time intervals of 7 and 30 days of therapy. RESULTS: The patients' mean age was 62.3±11 years (range, 30 to 83 y). Of 17 patients, 7 were female individuals. There were no significant differences in central choroidal thicknesses at the fovea, or at each point within the horizontal nasal and temporal quadrants between visits (P<0.05 for all comparisons). CONCLUSIONS: The current study showed that choroidal thickness does not change after latanoprost therapy. However, the choroidal changes of anterior part of the eye can be effected by the latanoprost therapy. Future studies investigating the anterior part of the eye and focusing on both choroidal perfusion and choroidal thickness may give additional information concerning the mechanism of action.

Influence of glycosylated hemoglobin on the choroidal thickness.

PURPOSE: The aim of the study reported here was to assess the influence of serum HbA1c levels on foveal choroidal thickness in diabetic patients. METHODS: A total of 122 eyes from 122 patients who had type 2 DM were studied in this prospective, cross-sectional study. Patients were divided into three groups: 43 patients (43 eyes) without diabetic retinopathy (NDR), 39 patients (39 eyes) with diabetic retinopathy and no macular edema (DR/ME-), 40 patients (40 eyes) with diabetic retinopathy and macular edema (DR/ME+). Central foveal thicknesses and subfoveal choroidal thicknesses were noted. Subfoveal choroidal thickness measurement was taken perpendicularly from the outer part of the retinal pigment epithelial layer to the line corresponding to the choroidal-scleral junction. Serum glycosylated hemoglobin (HbA1c) levels were evaluated. RESULTS: No significant differences in age, gender, pseudophakia were observed between the groups. There was no significant difference in HbA1c value among the three diabetic groups. Mean foveal choroidal thickness was 270.09 ± 42.41 in NDR group, 243.18 ± 30.21 in DR/ME- group and 250.90 ± 40.06 in DR/ME+ group. Mean foveal choroidal thickness in NDR group was significantly different from DR/ME- group. There was no statistically significant difference between the other groups among foveal choroidal thicknesses. There was no correlation between HbA1c and foveal choroidal thickness. Multiple regression analyses of two diabetic retinopathy groups showed no significant correlation between foveal choroidal thickness and HbA1c and also no significant correlation between foveal choroidal thickness and duration of diabetes. CONCLUSIONS: Serum HbA1c levels were not correlated with foveal choroidal thickness. Choroidal thickness is effected by several factors as axial length, age and systemic diseases.

Measurements of Retinal Nerve Fiber Thickness and Ganglion Cell Complex in Neurofibromatosis Type 1, with and Without Optic Pathway Gliomas: A Case Series.

PURPOSE: The aim of this study was to investigate differences in retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) thicknesses in neurofibromatosis 1 (NF1) cases, with and without optic pathway gliomas (OPGs). MATERIALS AND METHODS: In total, 33 eyes of 33 subjects were evaluated in this prospective observational case series. Twenty-one patients with a diagnosis of NF1 were enrolled. Patients with NF1 and OPGs were included in Group 1 (n = 9), and patients with NF1 without OPGs were included in Group 2 (n = 12). The control group (Group 3) was comprised of 12 age- and sex-matched subjects with no history of ophthalmic or systemic diseases. All of the subjects underwent complete ophthalmic examinations, including best-corrected visual acuity (BCVA), slit lamp microscopy, and indirect ophthalmoscopy. Additionally, optical coherence tomography (OCT) measurements were obtained. RESULTS: There were no statistically significant between-group differences in age and sex (p = 0.227 and 0.986, respectively). The average RNFL thickness in Group 1 (NF1 patients with OPGs) was significantly lower than in Groups 2 and 3 (86.6 ± 22.5, 107.4 ± 6.65, and 108.4 ± 5.05 µm, respectively; p = 0.001). Furthermore, the average GCC thickness in Group 1 was significantly lower than in Groups 2 and 3 (78.6 ± 16.3, 94.8 ± 3.55, and 94.9 ± 3.82 µm, respectively; p < 0.001). CONCLUSIONS: Both RNFL and GCC thicknesses were significantly lower in NF1 patients with OPGs. The use of OCT to quantify damage to the visual pathway may enable earlier detection of OPGs in NF1 patients.

Causes of visual impairment in patients with ocular toxoplasmosis.

INTRODUCTION: The aim of this study was to report the causes of visual impairment in patients with the classic clinical presentation of ocular toxoplasmosis (OT). METHODOLOGY: Eight patients with OT underwent standardized ophthalmologic examination and fundus imaging. Macula and the lesions that could be visualized were evaluated by spectral domain optical coherence tomography (SOCT) at presentation. The scan acquisition protocols for SOCT included a radial line scan through the retinochoroiditis lesion, radial line macular scan, and horizontal volume scans at the macula. RESULTS: The mean age of the five (62.5%) women and three (37.5%) men was 25.7±7.6 years. The mean logMAR ETDRS best-corrected visual acuity was 0.45 (Snellen equivalent, 20/50). SOCT findings of macula were normal in seven patients, and one patient had decreased retinal thickness from a healed chorioretinitis at the fovea. Of eight patients, two had 3+ vitreous haze, four had 2+ vitreous haze, and two had 1+ vitreous haze at presentation. OCT scans revealed vitreous hyperreflective dots in all patients with different densities in different radial scans. Hyperreflective dots were denser in macular scans of eyes in which the active lesion was closer to the fovea. CONCLUSIONS: In this study, visual impairment in majority of the patients was found to be related to vitreous cells and flare. Dense vitritis on macula scans and visual impairment were seen in the patients who had an active lesion closer to the fovea. SOCT may provide objective data of the cellular load of the eyes with posterior segment inflammation.

Retinal nerve fiber layer thickness in a healthy Turkish population measured by optical coherence tomography.

BACKGROUND: Normative optical coherence tomography (OCT) data is required for different subsets of the population as ethnic differences in retinal nerve fiber layer (RNFL) thickness have been reported. An OCT database for the normal Turkish population is not commercially available. OBJECTIVES: Quantify peripapillary RNFL thickness in a Turkish population. DESIGN: Cross-sectional, descriptive study. SETTING: Numune Training and Research Hospital, Adana, Turkey. SUBJECTS AND METHODS: RNFL measurements performed by Spectral OCT with a circular scan with 3.4 mm circle were centered around the optic disc of the right eye of each subject. Correlation of the effects of age and gender on RNFL thickness was analyzed. MAIN OUTCOME MEASURE(S): Correlation and measurements of RNFL. Retinal nerve fiber layer thick- ness measurements were obtained for the entire circumference of the optic nerve head. RESULTS: In 307 healthy subjects consisting of 135 (44%) males and 172 (56%) females, with a mean (SD) age of 35.1 (9.6) years (range, 20-50 years), RNFL was superior: 132.2 (16.63) micro m, inferior: 139.1 (14.53) micro m, nasal: 96.2 (14.23) micro m, temporal: 79.8 (10.7) micro m. The mean (SD) RNFL thickness for the group was 111.5 (9.3) micro m. There was no significant difference between the average RNFL thicknesses of age groups nor between males and females (P=.1, P=.42), and no significant correlation with respect to age (P=.430, r=-.04). There was a statistically significant difference between the inferior quadrants of the two genders (P=.04). CONCLUSIONS: RNFL thickness did not vary significantly with gender. A weak negative but statistically nonsignificant correlation between average RNFL thickness and age was found. LIMITATIONS: The participants were young adults or at early middle age so age effects on RNFL thickness may not be explained. The data does not reflect the entire Turkish population, which is not homogeneous.

Serous Macular Detachment Secondary to Optic Pit: Surgical Treatment and Long Time Results.

32-year-old Turkish male patient presented with an optic disk pit and serous macular detachment in the left eye. Spectral domain optical coherence tomography revealed serous macular detachment and retinoschisis. After vitrectomy the retina gradually flattened and vision was gradually improved. We aimed to report a case of serous macula detachment secondary to optic pit and long term result of surgical treatment.

Vitreous incarceration after ranibizumab injection: an ultrasound biomicroscopy study.

BACKGROUND AND OBJECTIVE: The aim of the study was to evaluate vitreous incarceration at the injection site using ultrasound biomicroscopy (UBM) after intravitreal injection of ranibizumab. PATIENTS AND METHODS: The study included 39 eyes of 34 patients who underwent intravitreal injection of 0.05 mL of ranibizumab. UBM (HiScan; Optikon, Rome, Italy) with a 50 MHz probe was performed 1 day after surgery to determine the existence of vitreous incarceration at the injection site. RESULTS: The mean patient age was 59.7 (± 10.1) years, and 58.9% were female. Vitreous incarceration into the pars plana site was detected in six eyes (15.3%) the day after intravitreal injection. CONCLUSION: Vitreous incarceration at the injection site after intravitreal injection is thought to lead to a higher risk for complications such as infection, retinal breaks, retinal detachment, vitreous hemorrhage, and fibrovascular proliferation at the injection site. To understand the clinical importance of vitreous incarceration, further long-term prospective studies are necessary.

A frontal lobe meningioma in a child leading to visual loss.

Objective. Meningiomas are benign primary meningeal tumors and are seen rare in children and adolescents. Clinical Presentation and Intervention. A 15-year-old Turkish boy reported a 1-month history of headache and blurred vision in both eyes. His visual acuity was 0.3 in both eyes with papilledema. Magnetic resonance imaging showed a 77 × 97 × 77 mm intracranial-extra-axial frontal lesion which compresses the chiasm. He was diagnosed with intracranial meningioma and referred to neurosurgery clinic. Conclusion. Ophthalmologists should be aware of the fact that papilledema and low vision can be caused by an intracranial tumor which compresses optic chiasm.

Metabolic syndrome as a risk factor for elevated intraocular pressure.

OBJECTIVE: The aim of this study was to investigate the association between intraocular pressure and metabolic syndrome by comparing central corneal thicknesses. METHODS: One hundred sixty-two subjects were enrolled in this cross-sectional study, with 89 subjects in a metabolic syndrome group and 73 subjects in a control group. Ophthalmological examinations, including intraocular pressure and central corneal thickness measurements, were performed on each subject. Serum fasting glucose, triglyceride and HDL cholesterol levels were measured, and waist circumference, systolic and diastolic blood pressure of all patients were recorded. RESULTS: Participants with metabolic syndrome had a significantly higher intraocular pressure than those without metabolic syndrome (p = 0.008), and there was no statistically significant difference between the central corneal thicknesses of the two groups (p = 0.553). Most of the metabolic syndrome components were associated with higher intraocular pressure (p < 0.05). CONCLUSIONS: There is a relationship between metabolic syndrome and intraocular pressure, but no association between metabolic syndrome and central corneal thicknesses. Intraocular pressure is affected by central corneal thicknesses, and intraocular pressure is used to correct according to the central corneal thicknesses measurement. To our knowledge, this is the first study that determines the positive relationship between metabolic syndrome and intraocular pressure by comparing the central corneal thicknesses of the groups.

Evaluation of retinal nerve fiber layer thickness in vernal keratoconjunctivitis patients under long-term topical corticosteroid therapy.

PURPOSE: The aim of this study was to evaluate the retinal nerve fiber layer (RNFL) thickness in vernal keratoconjunctivitis (VKC) patients who were under long-term topical corticosteroid therapy. METHODS: Thirty-six eyes of 36 VKC patients with clear cornea and normal videokeratography and 40 eyes of 40 age- and gender-matched normal children were included in the study. Clinical and demographic characteristics of the patients were noted and detailed ophthalmological examination was performed. Visual acuity (VA), spherical equivalent (SE), axial length (AL) and RNFL thickness measurements were compared between the groups. To correct ocular magnification effect on RNFL, we used Littmann's formula. RESULTS: All VKC patients had history of topical corticosteroid use and the mean duration of the topical corticosteroid use was 23.8 ± 9.09 months. There was no significant difference between the groups in terms of intraocular pressure (IOP). VKC group had significantly worse VA, greater SE and AL and thinner mean global, superior and inferior RNFL thickness. There were significant negative correlations between the duration of topical corticosteroid use and the mean global, superior and temporal RNFL thickness in VKC group. After correction of magnification effect, VKC group still had thinner mean global, superior and inferior RNFL thickness, and significant difference between the groups in inferior RNFL thickness did not disappear. CONCLUSION: Significant RNFL thickness difference between the groups suggests a possible effect of long-term corticosteroid use in VKC patients. Because visual field (VF) analysis in pediatric patients is difficult to perform and IOP may be illusive, RNFL thickness measurements in addition to routine examinations in VKC patients may help clinicians in their practice.