Comparison of the optical coherence tomography-angiography (OCT-A) vascular measurements between molecularly confirmed MODY and age-matched healthy controls.

AIMS: Previous structural, vascular density, and perfusion studies have mostly comprised type 1 and type 2 diabetes, even in the absence of retinopathy. The current study aimed to compare macular vessel density (VD) measurements between maturity-onset diabetes of the young (MODY) patients and controls. METHODS: The macular VD of superficial, deep retina, and choriocapillaris (CC), and central macular thickness (CMT), foveal avascular zone (FAZ), FAZ perimetry, VD of the total retina at 300 µm around the FAZ (FD), and acirculatory index (AI) measurements were taken and analyzed via OCT-A (RTVue XR 100-2 Avanti, AngioVue) and were compared between molecularly confirmed MODY (glucokinase (GCK) variants) patients and healthy controls. RESULTS: Twenty-five MODY patients and 30 healthy controls were included in the study. The mean plasma hemoglobin A1c level in the MODY group was 6.39 ± 0.38. The mean age was 13.8 ± 2.1 in the MODY group and was 12.6 ± 2.5 years among controls. There was no significant difference in terms of the age, superficial and deep retinal VD, FAZ, FAZ perimetry, CMT, FD, or AI between the groups. Compared to the healthy controls, a slight but significant increase in the CC-VD was detected in the MODY group, but only in the parafoveal and perifoveal regions (p = 0.034, p = 0.009). CONCLUSION: The significant CC-VD increase in the MODY group might be associated with hyperglycemia and/or relatively poor and vulnerable peripheral vascular CC perfusion compared to the central. Previous thickness and VD results of childhood or adolescent diabetes were distributed in a wider range, suggesting that various factors, including some not yet clearly defined, may affect the choroidal vasculature independently of glycemia or as a contributing factor.

Conspicuous Peripheral Retinal Hemorrhages with a Relatively Preserved Posterior Pole in Immune Thrombocytopenic Purpura.

BACKGROUND: Immune thrombocytopenic purpura (ITP) is a rare auto-antibody mediated disease of isolated thrombocytopenia (<100,000/µL) with normal haemoglobin levels and leukocyte counts. Only a small number of ITP cases have been reported with accompanying ophthalmological findings. Herein, we report an ITP case with demonstrative retinal haemorrhages. CASE PRESENTATION: A fifty-five-year-old woman with a known history of type 2 diabetes mellitus was referred to our clinic with blurred vision. After detailed anamnesis and clinical assessment, she was diagnosed as primary ITP in haematology department, and systemic steroid (1.5mg/kg) therapy was initiated. During her follow-up, a concomitant peripheral facial paralysis (PFP) emerged. In the course of follow-up, her platelet counts increased gradually, the retinal haemorrhages regressed partially, and the PFP recovered completely. CONCLUSION: ITP is a rare haematologic disease that sometimes manifests with additional systemic involvements, and this disease should be remembered in the differential diagnosis of unusual retinal haemorrhages, which might be the only presenting feature.

Macula and optic disc vessel density analyses in systemic lupus erythematosus with optical coherence tomography angiography.

To evaluate the optical coherence tomography angiography (OCT-A) findings in patients with systemic lupus erythematosus (SLE). Twenty-eight eyes of 28 patients with SLE and 27 eyes of 27 age and sex matched healthy controls were enrolled in this cross-sectional study. The vessel densities in the macula and optic disc were evaluated using the OCT-A (Optovue, Inc., Freemont, CA). Foveal retinal thickness, retinal vascular density in superficial capillary plexus (SCP), deep capillary plexus, and choriocapillaris, foveal avascular zone (FAZ), acircularity index, foveal vessel density (FD), and non-flow area in the superficial retina, the capillary and all-vessels density in the peripapillary area and the inside-disc area were automatically measured using Angiovue software of OCT-A and compared between the groups. The foveal, parafoveal and perifoveal retinal vessel densities in the superficial and deep capillary plexus and choriocapillaris were similar between groups. FAZ area, FAZ perimetry, acirculatory index, FD and non-flow area did not show a statistically significant difference. The vessel density in the inside disc area was significantly lower in patients with SLE (46.3 ±â€…3.8%) compared to the control group (49.1 ±â€…4.8%) (P = .02). Our results demonstrate significant decrement in vessel density in the inside-disc area in patients with SLE. The lower vessel density measurement in the inside-disc area might be associated with early neurologic vascular impairment in SLE. Further studies are required to determine the clinical relevance of this finding.

Comparison of the vessel density measurements of the macula, optical disc, and peri-papillary region between ocular hypertension patients and healthy controls.

OBJECTIVE: To evaluate Optical Coherence Tomography Angiography (OCT-A) findings in patients with Ocular Hypertension (OHT) and compare them with healthy individuals. METHODS: Thirty-four patients with ocular hypertension (OHT) and 22 healthy individuals were included in the study. Foveal thickness, retinal vascular density in superficial and deep capillary plexus and choriocapillaris, foveal avascular zone (FAZ), acircularity index (AI), foveal vessel density (FD), non-flow area, capillary and all vessel densities in the peripapillary area and the disc were automatically measured using the Angiovue software of OCT-A and compared between groups. RESULTS: The comparison of the macular OCT-A findings did not reveal a significant difference between the two groups in terms of central macular thickness, superficial and deep capillary plexus vessel density (p>0.05). The foveal avascular zone width was significantly higher in OHT subjects compared to the control group (0.30±0.08 µ and 0.25±0.11 µ, respectively; p = 0.04). The comparison of optic nerve OCT-A findings revealed that the whole-field vessel density (wVD) (p = 0.007), peripapillary vessel density (pVD) (p = 0.001), inferior, superior and temporal radial peripapillary capillary plexus vessel density (p = 0.006, p = 0.008, p = 0.02) and the mean retinal nerve fiber layer thickness (p = 0.02) were significantly lower in the OHT group. CONCLUSIONS: Our findings suggest that the decrement in the optic disc vascular density and foveal avascular zone width was significantly higher in OHT subjects. The possible effect or role of these microvascular changes in terms of glaucoma development should be examined through further studies.

Vogt Koyanagi Harada syndrome in a 15-year-old girl, steroids side effects and recurrences.

BACKGROUND: Vogt-Koyanagi-Harada Syndrome is rare in childhood and is usually seen between the 2nd and 5th decades. We present a 15-year-old girl with findings of incomplete Vogt-Koyanagi-Harada Syndrome. CASE: In the first visit, anterior chamber inflammation, vitritis, serous retinal detachment and papillitis were observed in her both eyes. She also had neurological symptoms such as a headache. During the systemic treatment period, some of the side effects related to steroids emerged. Additionally, the symptoms and findings of the disease relapsed while the steroid dose was reduced. CONCLUSION: Early diagnosis and selection of an individualized appropriate treatment provided good clinical and visual results without any serious complications in our case.

Fluorescein angiographic characteristic in predominantly classic and occult types of neovascular age-related macular degeneration treated with ranibizumab.

BACKGROUND AND PURPOSE: Randomised-controlled clinical trials (the ANCHOR and MARINA) examined the intravitreal anti-vascular endothelial growth factor (anti-VEGF) efficacy for eyes having fluorescein angiographic classic and occult (OCC) neovascular lesions. No significant difference in the treatment response between the lesion types was observed. Fundus fluorescein angiography and optical coherence tomography (OCT) are complementary devices that provide information about neovascular age-related macular degeneration (n-AMD). The aim of this retrospective study was to compare the clinical aspects of fluorescein angiographic characteristics in predominantly classic (PDC) and OCC subtypes of n-AMD treated with intravitreal ranibizumab. METHODS: Treatment-naive fluorescein angiographic OCC-n-AMD and PDC-n-AMD patients, who received monthly intravitreal ranibizumab for 3 months after baseline, and were followed-up with pro re nata injections between March 2013 and February 2018, were included. Means of the visual acuity (VA), central macular thickness (CMT), and intravitreal injection and visit numbers of the groups were compared throughout 24 months. RESULTS: We included 41 eyes of PDC-n-AMD patients and 36 eyes of OCC-n-AMD patients. The mean ages were 74.5 ± 10.6 and 71.9 ± 9.4, respectively. The baseline, and 3-, 6-, 12-, 18-, and 24-month VA results of the OCC group were significantly better than those in the PDC. However, VA gain in the PDC group at 3, 6, and 12 months was significantly higher than that in the OCC group. The mean of baseline CMT of the PDC (353 ± 118 µm) was significantly higher than that in the OCC group (293 ± 64 µm). No significant differences in terms of the number of visits or injections, or CMT change from the baseline values between groups were observed. CONCLUSION: The OCC-n-AMD patients had better baseline and follow-up VA and CMT means than the PDC-n-AMD patients. However, the PDC-n-AMD patients are expected to benefit more than the OCC-n-AMD patients in terms of VA gains.

Survey of hygiene, behaviours, and awareness regarding contact lens wear with conventional and novel questions.

Clinical relevance: Contact lens (CL) compliance studies are beneficial to identify potential risks, take necessary measures, and issue warnings on time.Background: Understanding CL compliance is crucial to ensure that individuals maintain healthy and appropriate CL wear. The present study aims to assess knowledge and awareness about soft CLs; the degree of non-compliance among soft CL wearers; and the associations of these with demographic features.Methods: A comprehensive questionnaire composed of 44 questions was used to assess the demographic characteristics, hygiene care, habits, knowledge, opinions, awareness and experiences of 109 soft CL wearers. The survey was conducted between September 2019 and March 2020.Results: The mean non-compliance rates were: 35.4% and 11.2% for hand-washing and hand-drying; 77.8% for rubbing/rinsing of the CLs; and 37.0%, 50.5%, and 31.6% for swimming, showering, and overnight sleeping with the CLs in, respectively. Women were more compliant in manual cleaning and rinsing of the CL storage cases with lens solution (p = 0.037). Swimming behaviour with inserted CLs was significantly high among CL users with over 5 years of experience (p = 0.03). Additionally, 85% of the wearers generally found the soft CLs comfortable, and 69.8% found the cost of the soft CLs reasonable. Over 90% of the wearers spent between 0 to 5 min per day to insert and remove the soft CLs.Conclusion: Women presented with more frequent CL use, and were significantly more compliant in terms of cleaning the CL storage cases. Regardless of gender, the non-compliance of swimming with inserted CLs was significantly high among CL wearers with over five years of experience. A majority of the results of the investigated non-compliances did not depend on gender, income level, age-group, duration of CL use, and education level.

Multimodal imaging in iodate-induced toxic retinopathy.

INTRODUCTION: Iodine deficiency is a leading cause of preventable physical and mental retardation. Potassium iodate is used for iodine supplementation to prevent iodine deficiency. We herein report a case of toxic retinopathy following intentional ingestion of potassium iodine. CASE PRESENTATION: A 41-year-old male presented with a 5-day history of blurred vision in both eyes. His visual acuity (VA) was hand motion and his pupillary reactions were sluggish bilaterally. The fundus examination revealed bilaterally diffuse retinal pigment epithelium atrophy and secondary pigmentary changes at the posterior pole, but his peripheral fundus was relatively spared. Choroidal thinning, punctate hyperreflective dots along the retinal pigment epithelium layer, and outer retinal atrophy were the optical coherence tomography findings, which were consistent with widespread areas of retinal pigment epithelium window defects observed on fundus fluorescein angiography. The visual evoked potential test showed no response in the right eye and revealed a delay in the latency and a decrease in the amplitude of the P100 wave in the left eye. Wave b responses of the photoreceptors could not be observed in the patient's electroretinogram. After a vitamin supplementation protocol consistent with the literature, at the 4-month follow-up visit his visual acuity had improved to 0.3 in the right eye and counting fingers in the left eye. CONCLUSION: Potassium iodate toxicity is a cause of serious retinal and choroidal damage and results in severe vision loss. Hydration, hemodialysis, and antioxidants can be helpful to minimize the complications.

Analysis of optical coherence angiography in cystoid macular oedema associated with gyrate atrophy.

BACKGROUND: To evaluate the relationship between superficial, deep foveal avascular zone (FAZ) and foveal cyst areas in eyes with cystoid macular oedema (CMO) associated with gyrate atrophy of the choroid and retina (GA). METHODS: This is a retrospective collaborative multicenter study of optical coherence tomography-angiography (OCTA) images in GA. Superficial and deep FAZ and foveal cyst were measured using Image J by two independent experts. Values were corrected for myopia magnification. These values were compared with age-matched controls from normative data. RESULTS: Twenty-three eyes from 12 patients with GA and CMO were included in the study. The mean ± standard deviation age was 22 ± 19.7 years, mean Snellen spectacle-corrected visual acuity of 20/70 with mean myopia of 5.7 ± 4.1 dioptres. Qualitatively, no focal occlusion of superficial and deep capillary plexus was noted. Mean superficial FAZ area (0.484 ± 0.317 mm2), deep FAZ area (0.626 ± 0.452 mm2), and foveal cyst area (0.630 ± 0.503 mm2) were significantly larger than superficial and deep FAZ areas in controls of same age range (p < 0.001). Macular cyst area correlated with superficial FAZ area (R = 0.59; p = 0.0057) and more strongly with deep FAZ area (R = 0.69; p < 0.001). CONCLUSIONS: The superficial and deep FAZ area in GA-associated CMO were noted to be significantly larger than in controls. It seems that RPE dysfunction leads to foveal cyst enlargement displacing the capillary plexus with resultant enlarged superficial and deep FAZ area.

Investigating the role of BEST1 and PRPH2 variants in the molecular aetiology of adult-onset vitelliform macular dystrophies.

Objective: To determine the clinical relevance and frequency of BEST1 and PRPH2 mutations in a clinically diagnosed adult-onset vitelliform macular dystrophy (AVMD) group with Caucasian ethnicity. Methods: The study comprised 24 patients who had been diagnosed with AVMD via indirect fundus ophthalmoscopy and presented with a dome-shaped appearance between the retinal pigment epithelium and photoreceptors on their spectral-domain optical coherence tomography. They had lesion hyper- autofluorescence on their fundus autofluorescence images and were also investigated for BEST1 and PRPH2 mutations for a probable molecular aetiology. Results: No pathogenic or likely pathogenic mutation was detected in the BEST1 and PRPH2 genes of any of the clinically diagnosed AVDM patients. A heterozygous NM_000322.5:c.938C>T (p.Pro313Leu) variant of the PRPH2 gene was detected in 2 non-consanguineous patients. According to current guidelines, this variant was classified as a 'variant of uncertain significance'. Conclusion: In conclusion, AVMD is a genotypic and phenotypic heterogeneous disease. The genetic aetiology could not be explained by sequencing BEST1 and PRPH2 genes in the AVMD patients; however, the variant of PRPH2 could be a cause of predisposition relevant to the phenotype.

Optical coherence tomography-angiographic vascular densities in Familial Mediterranean Fever (FMF) Patients with M694V Mutations.

Familial Mediterranean fever (FMF) is a hereditary auto-inflammatory disease with accompanying findings of amyloidosis and vasculitis. M694V is one of the most common mutations associated with amyloidosis. This study compared the macular optical coherence tomography angiography measurements in FMF patients who were genetically verified to carry the M694V mutation of the MEFV gene to those in healthy controls. The vessel densities (VDs) of superficial (SVP) and deep vascular plexus (DVP) of the retina, and choriocapillaris, foveal avascular zone (FAZ) perimetry, foveal VD 300µ around the FAZ (FD-300), acirculatory index (AI) and non-flow area were measured with  optical coherence tomography angiography (OCT-A). The FMF and control groups were matched for age and gender. Compound heterozygous pathogenic variants were excluded. Thirty-eight FMF patients with M694V mutations (28 heterozygous and 10 homozygous) and 40 healthy controls were included. The two groups were similar with the regard to age and gender (P=0.88 and P=0.49, respectively). None of the investigated parameters, including the vessel densities of the SVP and DVP, and choriocapillaris, FAZ perimetry, FD-300, AI, and non-flow area showed a statistically significant difference between the FMF and control groups. The macular vessel density measurements and FAZ parameters of FMF patients with M694V mutations do not differ from age- and sex-matched healthy controls.

Pupil Diameter, Corneal Thickness, and Anterior Chamber Alterations Following Topical Olopatadine Hydrochloride 0.1%: A Single-Masked Randomized Controlled Clinical Study.

Purpose: Olopatadine hydrochloride 0.1% is one of the known primary topical treatments in ocular allergy. Although olopatadine is a worldwide used medication, the changes in pupil diameter, cornea, and anterior chamber associated with its use have not been studied in detail. In this prospective study, we aimed to determine the amount of mydriasis and explore the possible corneal and anterior chamber alterations after 0.1% topical olopatadine. Methods: A total of 77 eyes from 77 ocular-allergy diagnosed patients between 18 and 40 years were investigated in this prospective study. Thirty-nine eyes of 39 patients received topical olopatadine, and 38 eyes of 38 patients received sterile distilled water, randomly. Pentacam (Oculus Optikgeräte GmbH, Wetzlar, Germany) topography was used to assess the pupil and anterior chamber measurements at baseline and after 45 min of olopatadine or sterile distilled water instillation. Results: The differences between the baseline and 45th-min measurements for corneal thickness, anterior chamber depth, angle, and volume did not reach a statistical significance in the olopatadine or control groups. The pupil diameter significantly increased from 3.19 ± 0.62 to 3.36 ± 0.62 mm in the olopatadine group (P < 0.001), and remained relatively unchanged in the control group (P = 0.06). Conclusion: Olopatadine 0.1% does not lead to a significant change in corneal topography or anterior chamber parameters. However, it causes a slight but statistically significant increase in pupil diameter.

Regression of macular edema with topical brinzolamide and nepafenac alone and identification of a novel gyrate atrophy mutation.

Gyrate atrophy is a rare metabolic autosomal recessive disorder caused by ornithine aminotransferase enzyme deficiency that leads to characteristic progressive, degenerative chorioretinal findings. Patients complain mostly of low vision, night blindness, and peripheral vision loss. Posterior subcapsular cataract, myopia, choroid neovascularization, and intraretinal cysts may be accompanying factors related to vision loss. We encountered a patient with vision loss secondary to posterior subcapsular cataract and intraretinal cysts. After treatment with topical brinzolamide and nepafenac (and without any diet mo dification and/or supplementation), we observed 143- and 117-mm macular thickness resolutions with 2 and 1 Snellen lines of visual gain in his right and left eyes, respectively. Also, we detected a novel homozygous mutation in the ornithine aminotransferase gene: c.1253T>C (p.Leu418Pro). Carbonic anhydrase inhibitors and/or non-steroid anti-inflammatory drugs can control macular edema in patients with gyrate atrophy-associated intraretinal cysts. The genetic variants may also be a determinant in the responsiveness to the therapy type.

Does Long-term Soft Contact Lens Wear Affect Corneal and Anterior Chamber Parameters?

OBJECTIVES: To assess the long-term effects of soft contact lenses (SCL) on the cornea and anterior chamber by topography. MATERIALS AND METHODS: Thirty-nine eyes of 22 healthy patients were included in this prospective study. Changes in corneal and anterior chamber parameters before and after 12 months of daily SCL use (Air Optix Aqua, Air Optix Aqua for Astigmatism, Acuvue Oasys and Acuvue Oasys for Astigmatism) were evaluated with Pentacam (Oculus, Germany). RESULTS: Best corrected visual acuity with toric SCL was significantly better compared to spectacles in the toric SCL group (0.98±0.34 vs 0.94±0.72, p=0.004). None of the corneal (horizontal and vertical keratometry, corneal volume, anterior and posterior corneal astigmatism, corneal pachymetry of apex and thinnest location) and anterior chamber (anterior chamber depth, volume and angle) parameters showed a statistically significant change after long-term daily wear of SCLs. CONCLUSION: The results of this study revealed that long-term wear of current high oxygen permeable and relatively low modulus silicone hydrogel SCLs does not impact cornea and anterior chamber morphology or volumetric parameters. Furthermore, toric silicone hydrogel SCLs can provide better visual performance than spectacles.

Comparison of the refractive measurements with hand-held autorefractometer, table-mounted autorefractometer and cycloplegic retinoscopy in children.

PURPOSE: To evaluate the performance of the hand-held and table-top autorefractokeratometer in measuring refractive errors by comparing them with cycloplegic retinoscopy. METHODS: Included in the study were 112 eyes of 112 pediatric patients whose mean age was 6.78 ± 2.61 years (range, 2 to 12 years). The refractive errors of all the eyes were measured with and without cycloplegia using a hand held autorefractokeratometer (Retinomax K-plus 3), table top autorefractokeratometer (Canon RK-F1) and performing cycloplegic retinoscopy. The spherical equivalent, cylindrical axis and keratometer values were statistically compared. RESULTS: The mean spherical equivalent obtained from the Retinomax K-plus 3 was significantly less hyperopic than that of Canon RK-F1 (p = 0.004) before cycloplegia. When the Bland Altman analysis was performed in comparisons of spherical equivalent values measured with the Retinomax K-plus 3, Canon RK-F1 and cycloplegic retinoscopy, it was seen that almost all of the differences between the measurements remained within the range of ±2 standard deviation. Good agreement was found between Retinomax K-plus 3 and Canon RK-F1 for the Jackson cross-cylinder values at axis 0° and 45°; keratometer values respectively. CONCLUSIONS: The refractive error components were highly correlated between the two instruments and cycloplegic retinoscopy.