Diagnostic accuracy of OCT angiography for macular neovascularization in central serous chorioretinopathy: A systematic review and meta-analysis.

Identifying macular neovascularization (MNV) in eyes with central serous chorioretinopathy (CSC) has important implications for its management. Optical coherence tomography angiography (OCTA) is increasingly used for this purpose. Here, we systematically reviewed the literature and conducted meta-analysis to determine the diagnostic accuracy of OCTA for detecting MNV in eyes with CSC. We systematically searched the literature in 12 databases for relevant studies from database inception until 18 November 2023. Eligible studies had eyes with CSC with MNV and CSC without MNV. Index test was OCTA. Reference test was retinal dye angiography. Study selection and data extraction were performed in duplicate, and study was evaluated using the Quality Assessment of Diagnostic Accuracy Studies 2. Our main outcome of interest was the sensitivity and specificity of OCTA for detecting MNV in CSC. Pooled diagnostic test accuracy estimates were computed using MetaDTA. Of 177 records screened, seven fulfilled the eligibility criteria for our study. These studies summarized data from a total of 1061 eyes. Summary estimate sensitivity and specificity to diagnose MNV in eyes with CSC using OCTA was 92.9% (95% CI: 81.7%-97.5%) and 99.4% (95% CI: 84.1%-100.0%), respectively. The main source of bias across studies was the reference standard, as four studies used multimodal imaging including OCTA for the reference standard. OCTA alone is excellent for detecting MNV in CSC compared to retinal dye angiography or multimodal imaging. Using OCTA first before considering retinal dye angiography could potentially save an important number of retinal dye angiographies.

PHARC syndrome which an ultra-rare syndrome with retinitis pigmentosa and cataracts: case report and review of the literature.

BACKGROUND: PHARC syndrome (MIM:612674) is a rare neurodegenerative disorder characterized by demyelinating polyneuropathy, hearing loss, ataxia, retinitis pigmentosa, and cataracts (PHARC). The syndrome is caused by mutations in the ABHD12 gene, which encodes αß-hydrolase domain-containing protein 12 related to endocannabinoid metabolism. PHARC syndrome is one of the rare diseases; so far, only 51 patients have been reported in the literature. METHODS: We evaluated the 25-year-old male patient referred to us due to vision loss, cataracts, and hearing loss. Ophthalmological examinations and genetic analyses were performed using targeted next-generation sequencing. RESULTS: In the genetic analysis, the patient was diagnosed with PHARC syndrome by detecting homozygous (NM_001042472.3): c.871del (p.Tyr291IlefsTer28) novel pathogenic variation in the ABHD12 gene. Following the molecular diagnosis, he was referred to the neurology department for reverse phenotyping and sensorimotor demyelinating polyneuropathy was detected in the neurological evaluation. CONCLUSIONS: In this study, we report a novel variation in ABHD12 gene in the first Turkish-origin PHARC patient. We present this study to contribute genotype-phenotype correlation of PHARC syndrome and emphasize the importance of molecular genetic diagnosis in order to determine the appropriate clinical approach. This report is essential for expanding the phenotypic spectrum in different populations and understanding the genotype-phenotype correlation of PHARC syndrome via novel pathogenic variation in the ABHD12 gene.

Real-World Outcomes of Intravitreal Anti-Vascular Endothelial Growth Factor Treatment for Diabetic Macular Edema in Türkiye: MARMASIA Study Group Report No. 1.

Objectives: This study aimed to report the demographic and clinical characteristics of diabetic macular edema (DME) patients treated with intravitreal injection (IVI) of anti-vascular endothelial growth factors (anti-VEGF) and provide an overview of outcomes during routine clinical practice in Türkiye. Materials and Methods: This retrospective, real-world study included 1,372 eyes (854 patients) treated with a pro re nata protocol by 21 ophthalmologists from 8 tertiary clinics on the Asian side of the Marmara region of Türkiye (MARMASIA Study Group). Five cohort groups were established by collecting the patients' baseline and 3, 6, 12, 24, and 36-month follow-up data, where each subsequent cohort may include the previous. Changes in best-corrected visual acuity (BCVA, approximate ETDRS letters) and central macular thickness (CMT, µm), number of visits and IVI, and rates of anti-VEGF switch and intravitreal dexamethasone implant (IDI) combination were evaluated. Results: The 3, 6, 12, 24, and 36-month cohorts included 1372 (854), 1352 (838), 1185 (722), 972 (581), and 623 (361) eyes (patients), respectively. The mean baseline BCVA and CMT were 51.4±21.4 letters and 482.6±180.3 µm. The mean changes from baseline in BCVA were +7.6, +9.1, +8.0, +8.6, and +8.4 letters, and in CMT were -115.4, -140.0, -147.9, -167.3, and -215.4 µm at the 3, 6, 12, 24, and 36-month visits (p<0.001 for all). The median cumulative number of anti-VEGF IVI was 3.0, 3.0, 5.0, 7.0, and 9.0, respectively. The overall anti-VEGF switch and IDI combination rates were 18.5% (253/1372 eyes) and 35.0% (480/1372 eyes), respectively. Conclusion: This largest real-life study of DME from Türkiye demonstrated BCVA gains inferior to randomized controlled trials, mainly due to the lower number of IVI. However, with the lower baseline BCVA and higher IDI combination rates in our cohorts, these gains were relatively superior to other real-life study counterparts.

Predictive Factors of Complications and Visual Outcomes after Pediatric Cataract Surgery: A Single Referral Center Study from Türkiye.

Objectives: To evaluate the predictive factors of complications and visual acuity outcomes in pediatric cataract patients. Materials and Methods: This retrospective, observational clinical study included 80 eyes of 50 patients treated for pediatric cataracts between 2010 and 2020. The eyes were divided into Group I (congenital cataracts, n=38) and Group II (developmental cataracts, n=42). Group II was also divided into Group IIA (aphakic, n=21) and Group IIB (pseudophakic, n=21). The effects of the age, laterality, cataract morphology, intraocular lens implantation, preoperative nystagmus/strabismus, and intraoperative anterior hyaloid rupture on complications and final best-corrected visual acuity (BCVA; logMAR) were evaluated. Results: The median (interquartile range) age and follow-up time were 28 (5-79) months and 60 (29-84) months, respectively. There was a significant difference in mean final BCVA between Group I (0.79±0.46) and Group II (0.57±0.51) (p=0.047); however, no difference was observed between Group IIA and Group IIB (p=0.541). Having congenital cataract (p=0.045), preoperative nystagmus/strabismus (p=0.042), total/mature cataract (p<0.001), and postoperative complications (p=0.07) were significantly associated with final BCVA. However, in multivariate analysis, only total/mature cataract (ß: 0.52, p<0.001) and having any complication (ß: 0.24, p=0.018) were associated with final BCVA. Congenital cataract and intraoperative anterior hyaloid rupture were the only significant risk factors of postoperative complications on univariate (p=0.027 and p=0.003, respectively) and binary logistic regression analysis (odds ratio [OR]: 2.95 [95% confidence interval: 1.07-8.15], p=0.036 and OR: 4.28 [95% confidence interval: 1.55-11.77], p=0.005, respectively). Conclusion: Total/mature cataract and the presence of any postoperative complication adversely affected the final BCVA. Having a congenital cataract and intraoperative anterior hyaloid membrane rupture increased the risk of complications.

Choroidal vascularity index as an activity criterion and a treatment response measure in chronic central serous chorioretinopathy.

PURPOSE: This study aimed to evaluate the choroidal vascularity index (CVI) as an activity criterion in chronic central serous chorioretinopathy (CSC) and as a measure of treatment response after full-dose-full-fluence photodynamic therapy (fd-ff-PDT). METHODS: This fellow-eye-controlled, retrospective cohort study included 23 patients with unilateral chronic CSC treated with fd-ff-PDT (6 mg/m2 ; 50 µcm2 ; 83 s). Subfoveal choroidal thickness (SFCT, µm) and CVI (%) of the affected and fellow eyes at baseline as well as at 1, 3 and 6 months after fd-ff-PDT were compared. RESULTS: The patients' mean age was 43.4 ± 7.3 years, and 18 (78.3%) were male. CVI was comparable between the affected and fellow eyes at baseline (66.09 ± 1.56 vs. 65.84 ± 1.57, p = 0.59). However, it became significantly lower in the affected eyes 1 (64.45 ± 1.68 vs. 65.87 ± 1.19, p = 0.002), 3 (64.21 ± 2.08 vs. 65.71 ± 1.59, p = 0.009) and 6 (64.47 ± 2.19 vs. 65.62 ± 1.52, p = 0.045) months after fd-ff-PDT. The mean SFCT and the mean CVI were significantly decreased in the affected eyes at all follow-up visits compared with baseline after fd-ff-PDT (p < 0.001). CONCLUSION: At baseline, CVI was comparable between affected and fellow eyes. Therefore, its use as an activity criterion in chronic CSC patients is questionable. However, it was significantly decreased in fd-ff-PDT-treated eyes, supporting its role as a measure of treatment response in chronic CSC.

A Useful Method for the Practice of Pneumatic Retinopexy: Slit-Lamp Laser Photocoagulation through the Gas Bubble.

This study aimed to demonstrate the laser retinopexy method through the gas bubble under a slit-lamp biomicroscope using a wide-field contact lens to treat rhegmatogenous retinal detachment (RRD) with pneumatic retinopexy (PR) and report its anatomical and functional results. This single-center, retrospective case series included RRD patients treated with PR using sulfur hexafluoride (SF6). The demographics, preoperative factors, and anatomical and functional outcomes were collected from the patient files. The single-procedure success rate of PR at postoperative 6th months was 70.8% (17/24 eyes), and the final success rate after secondary surgeries was 100%. The BCVA was better in the successful PR eyes at postoperative 3rd (p = 0.011) and 6th month (p = 0.016) than in failed eyes. No single preoperative factor was associated with PR success. The single-procedure success rate of PR using the laser retinopexy method through the gas bubble with a wide-field contact lens system seems comparable to the PR literature.

Staphylococcus hominis: a rare cause of endophthalmitis / Staphylococcus hominis: uma causa rara de endoftalmite

ABSTRACT Staphylococcus hominis (S. hominis) is a coagulase-negative Staphylococci and an infrequent cause of endophthalmitis. Due to its ability to produce biofilm, especially in diabetic patients, strains may acquire antibiotic resistance. We present two cases of S. hominis endophthalmitis, one with acute endophthalmitis after intravitreal bevacizumab injection and one with chronic endophthalmitis following undiagnosed penetrating ocular trauma. Although there are only four published S. hominis endophthalmitis cases in the literature, to the best of our knowledge, there has been no previously published case after intravitreal bevacizumab.

RESUMO Staphylococcus hominis (S. hominis) é um estafilococo coagulase-negativo e uma causa pouco frequente de endoftalmite. Devido à sua capacidade de produzir biofilme, especialmente em pacientes diabéticos, cepas dessa bactéria podem adquirir resistência a antibióticos. Este relato apresenta dois casos de endoftalmite por S. hominis: um de endoftalmite aguda após injeção intravítrea de bevacizumabe e outro de endoftalmite crônica após trauma ocular penetrante não diagnosticado. Embora existam apenas quatro casos de endoftalmite por S. hominis publicados na literatura, até onde sabemos não houve nenhum caso publicado anteriormente após bevacizumabe intravítreo.

Skin-Only Versus Skin-Plus-Orbicularis Resection Blepharoplasty: An Elaborated Analysis of Early- and Long-Term Effects on Corneal Nerves, Meibomian Glands, Dry Eye Parameters, and Eyebrow Position.

PURPOSE: To evaluate the early- and long-term effects of 2 different blepharoplasty techniques on corneal nerves, meibomian gland morphology, clinical parameters of dry eye disease (DED), and eyebrow position. METHODS: This prospective, interventional study included age-sex-matched blepharoplasty patients who had a skin-only resection (24 eyes of 12 patients; Group-S) or a skin-plus-orbicularis muscle resection (24 eyes of 12 patients; Group-M) procedure. Preoperative and postoperative parameters of in vivo corneal confocal microscopy (IVCCM; corneal nerve fiber density [CNFD], nerve branch density [CNBD], and nerve fiber length), meibomian gland area loss (MGAL), DED (Schirmer I test and noninvasive tear breakup time), and eyebrow heights (lateral [LBH] and central [CBH]) were evaluated and compared between the intervention groups ( ClinicalTrials.gov , NCT05528016). RESULTS: Compared with baseline, the CNBD of Group-S (19.91 ± 7.66 vs. 16.05 ± 7.28 branches/mm 2 , p = 0.049) and CNFD of Group-M (19.52 ± 7.45 vs. 16.80 ± 6.95 fibers/mm 2 , p = 0.028) was significantly decreased at postoperative first week. However, in both groups, IVCCM parameters returned to baseline values at postoperative first month and first year ( p > 0.05). A significant MGAL increase was observed in Group-S (18.47 ± 5.43 vs. 19.94 ± 5.31, p = 0.030) and Group-M (18.86 ± 7.06 vs. 20.12 ± 7.01, p = 0.023) at the postoperative first year, demonstrating meibomian gland atrophy. Only significant changes were observed in Group-M in LBH (16.17 ± 2.45 vs. 16.67 ± 2.28 mm, p = 0.044) and CBH (17.33 ± 2.35 vs. 17.96 ± 2.31 mm, p = 0.004) at postoperative first year. CONCLUSIONS: Blepharoplasty with or without orbicularis resection seems to have similar effects on IVCCM, DED, and MGAL parameters. However, incorporating an orbicularis muscle resection in a blepharoplasty operation could slightly elevate the eyebrow position.

In Vivo Corneal Confocal Microscopy in Multiple Sclerosis: Can it Differentiate Disease Relapse in Multiple Sclerosis?

PURPOSE: This study aims to investigate the role of in vivo corneal confocal microscopy (IVCCM) in the detection of corneal inflammatory activity and subbasal nerve alterations in patients with multiple sclerosis (MS) and to further determine whether IVCCM can be used to detect (acute) disease relapse. DESIGN: Prospective cross-sectional study, with a subgroup follow-up. METHODS: This single-center study included 58 patients with MS (MS-Relapse group [n = 27] and MS-Remission group [n = 31]), and 30 age- and sex-matched healthy control subjects. Patients with a history of optic neuritis or trigeminal symptoms were excluded. Corneal nerve fiber density (CNFD), corneal nerve branch density (CNBD), corneal nerve fiber length (CNFL), and dendritic cell (DC) density were evaluated in all patients with MS and control subjects by IVCCM. Patients in the MS-Relapse group who were in remission for ≥6 months after the MS incident underwent a repeat IVCCM. RESULTS: No statistical difference was observed between the MS-Relapse and MS-Remission groups regarding age, sex, MS duration, and the number of relapses (P > .05). Compared with healthy control subjects, all subbasal nerve parameters were significantly lower (CNFD: P < .001, CNFL: P < .001, CNBD: P < .001), and the DC density was significantly higher (P = .023) in patients with MS. However, no significant difference was observed between MS-Relapse and MS-Remission groups in terms of CNFD (mean [SE] difference -2.05 [1.69] fibers/mm2 [95% confidence interval {CI} -1.32 to 5.43]; P < .227), CNFL (mean [SE] difference -1.10 [0.83] mm/mm2 [95% CI -0.56 to 2.75]; P < .190), CNBD (mean [SE] difference -3.91 [2.48] branches/mm2 [95% CI -1.05 to 8.87]; P < .120), and DC density (median [IQR], 59.38 [43.75-85.0] vs 75.0 [31.25-128.75]; P = .596). The repeat IVCCM in relapse patients (n = 16 [59.3%]) showed a significant increase in CNFD (P = .036) and CNBD (P = .018), but no change was observed in CNFL (P = .075) and DC density (P = .469). CONCLUSION: Although increased inflammation and neurodegeneration can be demonstrated in patients with MS compared with healthy control subjects, a single time point evaluation of IVCCM does not seem to be sufficient to confirm the occurrence of relapse in patients with MS. However, IVCCM holds promise for demonstrating early neuroregeneration in patients with MS.

Staphylococcus hominis: a rare cause of endophthalmitis.

Staphylococcus hominis (S. hominis) is a coagulase-negative Staphylococci and an infrequent cause of endophthalmitis. Due to its ability to produce biofilm, especially in diabetic patients, strains may acquire antibiotic resistance. We present two cases of S. hominis endophthalmitis, one with acute endophthalmitis after intravitreal bevacizumab injection and one with chronic endophthalmitis following undiagnosed penetrating ocular trauma. Although there are only four published S. hominis endophthalmitis cases in the literature, to the best of our knowledge, there has been no previously published case after intravitreal bevacizumab.

Juvenile Xanthogranuloma Presented with Buphthalmos and Corneal Clouding in Neonatal Period: A Case Report.

Aim: To report an ocular juvenile xanthogranuloma (JXG) case presented with buphthalmos, corneal cloudiness, and normal intraocular pressure (IOP) in the neonatal period and treated with Ahmed glaucoma valve (AGV) implantation. Background: JXG is a rare disorder predominantly seen in infants, but the neonatal presentation is extraordinary. Although spontaneous hyphema is a common presenting sign in JXG, buphthalmos and corneal opacity in the neonatal period were reported only in one case, which had high IOP values at presentation. Case presentation: Sixteen-day-old male patient presented with buphthalmos, diffuse corneal clouding, and 11 mm Hg of IOP value in the right eye. IOP increased to 28 mm Hg three weeks later, and spontaneous hyphema developed, which did not respond to antiglaucomatous medications and topical corticosteroids. AGV was implanted, and the IOP decreased to 13 mm Hg postoperatively. In the follow-ups, numerous firm yellowish nodules were noticed on the patient's skin during the examination under general anesthesia. Histopathological examination of the skin nodules was compatible with the diagnosis of JXG. Lens subluxation and phacodonesis were developed during the follow-up and were managed with pars plana lensectomy. After a silent period of 3 months, epithelial ingrowth was determined around the side port entrance. Unfortunately, the ingrowth did not respond to cryotherapy and resulted in phthisis bulbi. Pathological evaluation of the enucleated phthisic eye revealed posterior segment involvement. Conclusion: Ocular JXG can be present with buphthalmos, corneal opacity, and normal IOP values without any skin lesions in the neonatal period. Neonatal presentation of JXG may be associated with limited medical therapy response and aggressive disease course. Clinical significance: This case report introduces the second ocular JXG case, which presented with buphthalmos and corneal cloudiness, and the third pathologically proven posterior segment involvement of JXG in the literature. How to cite this article: Dericioglu V, Sevik MO, Eraslan M, et al. Juvenile Xanthogranuloma Presented with Buphthalmos and Corneal Clouding in Neonatal Period: A Case Report. J Curr Glaucoma Pract 2022;16(2):128-131.

Effect of age on primary balloon dacryocystoplasty and probing success in congenital nasolacrimal duct obstruction.

PURPOSE: To compare the success rates of balloon dacryocystoplasty (BDP) and probing as a primary procedure in congenital nasolacrimal duct obstruction (CNLDO) and investigate the effect of age on both procedures. METHODS: A total of 135 patients (171 eyes) with simple and incomplete complex CNLDO were included in this retrospective study; complete complex CNLDO cases were excluded. The success rates for primary BDP (118 eyes) and for probing (53 eyes) were compared overall and among the age groups; Group 1 (12-24 months old), Group 2 (25-36 months old), and Group 3 (> 36 months old). RESULTS: Mean age of the patients was 41.5 ± 27.2 months for primary BDP, and 21.8 ± 10.8 months for probing (p < 0.001). Overall success rates for primary BDP and probing were 81.1% (43/53) and 76.3% (90/118), respectively (p = 0.481). Success rates for BDP and probing among age groups were 93.8% and 85.3% in Group 1 (p = 0.360), 93.3% and 50.0% in Group 2 (p = 0.012), and 63.6% and 27.3% in Group 3 (p = 0.052), respectively. Cox regression analysis showed that the median ages were 18 months for probing and 36 months for primary BDP. The Poisson regression model showed that for every 1-month increase in patients' age, the success rate of probing decreased by 9.7%. CONCLUSION: Probing success decreased to a point where different treatment options such as primary BDP can be discussed with the patients' parents after 18 months of age. The success of BDP decreased after 36 months, while it maintained a high success rate between 24 and 36 months as primary treatment.

Clinical outcomes with a low add multifocal and an extended depth of focus intraocular lenses both implanted with mini-monovision.

OBJECTIVES: To compare the visual acuity, contrast sensitivity (CS), spectacle needs, photic phenomena, and quality of life parameters of patients bilaterally implanted with a low add multifocal (MIOL) or an extended depth of focus (EDOF) intraocular lens (IOL), both with intended mini-monovision. METHODS: In this prospective, randomized, comparative study, patients were randomized to receive either Tecnis +2.75 D (ZKB00) (MIOL Group, n = 15) or Tecnis Symfony (ZXR00) (EDOF Group, n = 14) for bilateral implantation with mini-monovision (-0.50 D). Binocular logMAR uncorrected visual acuities (UVA), monocular defocus curves, CS with CSV 1000-E, and Pelli-Robson Test (PRT), spectacle needs and quality of life parameters with NEI RQL-42 questionnaire were evaluated at postoperative 1, 3, and 6 months. RESULTS: Results of MIOL and EDOF Groups at postoperative month 6 are as follows: distance (6 m) UVA -0.03 ± 0.05 and -0.05 ± 0.06 (p = 0.938), intermediate (60 cm) UVA, 0.04 ± 0.08 and -0.03 ± 0.07 (p = 0.046); near (40 cm) UVA, 0.22 ± 0.08 and 0.15 ± 0.07 (p = 0.046); near spectacle needs, 26.7% and 14.3% (p > 0.05), respectively. Better visual acuity was achieved in the EDOF Group between the defocus range of -0.50 and -1.75 D (p < 0.05). No significant difference was found regarding photic phenomena and CS evaluated with CSV 1000-E between the two IOL groups at 6 months after surgery (otherwise there are differences at 1 and 3 months in favor of EDOF). However, EDOF Group performed better in mesopic CS evaluated with PRT (p < 0.05). CONCLUSIONS: When implanted with mini-monovision better binocular uncorrected visual performance at intermediate and near distances achieved with EDOF than low add MIOL.

TRANSCORNEAL ELECTRICAL STIMULATION THERAPY MAY HAVE A STABILIZATION EFFECT ON MULTIFOCAL ELECTRORETINOGRAPHY FOR PATIENTS WITH RETINITIS PIGMENTOSA.

PURPOSE: To assess the effects of transcorneal electrical stimulation (TES) on several measures of visual function in retinitis pigmentosa. METHODS: This prospective, randomized, fellow-eye-controlled study includes 30 eyes of 15 patients with retinitis pigmentosa. Each patient's eyes were randomly selected as treatment (TE) and control eye (CE), and 30 minutes/week TES was applied for 6 months. Patient evaluations were performed before and after TES, including comprehensive ophthalmological examination, visual fields, full-field and multifocal electroretinography, microperimetry, and optical coherence tomography. All parameters were compared before and after TES and between TE and CE. RESULTS: After TES, the mean signal amplitudes in multifocal electroretinography were stabilized in TE. The mean signal amplitudes in CE decreased in every ring, reaching significance in the fifth ring (847.15 ± 393.94 and 678.77 ± 282.66 nV, P = 0.039, before and after TES, respectively). The changes in the mean signal amplitudes of TE and CE were -0.38 ± 295.53 and -185.15 ± 332,62 nV in second (P = 0.046), 36.69 ± 326.4 and -143.38 ± 317,41 nV in fourth (P = 0.028), and -17.46 ± 333.07 and -168.38 ± 297,14 nV in fifth rings (P = 0.046), respectively. The decrease in the mean signal amplitudes between 2° and 20° midperipheral retina was significantly less in TE (-33.59 ± 225,1 nV) than CE (-205.56 ± 345.1 nV) (P = 0.011). There were no significant changes in other parameters. CONCLUSION: The progression in multifocal electroretinography might be stabilized with TES. Further studies with larger sample sizes and a longer follow-up are needed to conclude that TES reduces retinitis pigmentosa progression.

Choroidal vascularity index changes during the Valsalva manoeuvre in healthy volunteers.

PURPOSE: To evaluate the effects of the Valsalva manoeuvre (VM) on the choroidal vascularity index (CVI) in healthy volunteers. METHODS: This prospective, cross-sectional study included 60 eyes of 30 healthy volunteers. Enhanced depth imaging-optical coherence tomography scans of both eyes involving the fovea were taken, and a 1500 µm subfoveal choroidal area was selected for image binarization with open-access Fiji software. The binarized image was segmented into the stromal area (SA) and luminal area (LA), and CVI was calculated as the ratio (%) of LA to the total choroidal area (TCA). CVI, subfoveal choroidal thickness (SFCT), IOP, systolic and diastolic blood pressure were evaluated at rest and during the VM. RESULTS: During the VM, a mean ± standard deviation increase in LA (0.02 ± 0.05 mm2 , p < 0.001) and CVI (1.72 ± 2.83%, p < 0.001) was observed, whereas SA (-0.02 ± 0.05 mm2 , p < 0.001) decreased. There was no significant change in TCA (0.00 ± 0.03 mm2 , p = 0.55) or SFCT (1.05 ± 10.92 µm, p = 0.46). There was a moderate positive correlation between the spherical equivalent refractive error (SE) and SFCT both at rest and during VM (r58  = 0.49, p < 0.0005 and r58  = 0.49, p < 0.0005, respectively). However, there was no significant correlation between SE and CVI either at rest or during VM (p = 0.11 and 0.06, respectively). In a multiple linear regression analysis, CVI was only associated with SFCT; however, SFCT was also associated with SE, both at rest and during VM (p < 0.001). CONCLUSION: Valsalva manoeuvre increases CVI by choroidal vascular dilation as demonstrated by an increase in LA and a decrease in SA. Researchers should be careful about unintentional VM during examinations.

Straatsma Syndrome: Should Visual Prognostic Factors Be Taken into Account? A Case Report.

Straatsma syndrome is the triad of myelinated retinal nerve fibers, myopia, and amblyopia and may be associated with strabismus, nystagmus, hypoplastic optic nerve, and heterochromia iridum. The degree of anisometropia, presence of strabismus, extent of myelination, and macular involvement have been reported to be associated with poor visual acuity after occlusion therapy for amblyopia in this syndrome. Here we present two cases of Straatsma syndrome with different responses to occlusion therapy and discuss their treatment responses according to prognostic factors for post-occlusion visual acuity.

Resolution of cystoid macular edema with topical carbonic anhydrase inhibitor in a patient with retinal dystrophy associated with Cohen syndrome.

Background: Cohen Syndrome (CS) is an autosomal recessive multisystemic disorder characterized by various ophthalmologic findings, including retinal dystrophy and associated cystoid macular edema (CME), in which there was no known effective treatment approach.Material and Methods: We describe a CS patient with a homozygous c.62 T > G, p.(Leu21*) mutation in the VPS13B gene with a topical carbonic anhydrase inhibitor (CAI; brinzolamide %1, thrice daily) responding CME.Case Description: A seven-year-old girl with an established diagnosis of CS was referred with a primary complaint of nyctalopia. On ophthalmologic examination, bilateral decreased visual acuity and normal-appearing macula with mild optic disc pallor were present. However, the detailed evaluation revealed bilateral blunted foveal reflexes, barely visible foveal pigmentation, and slightly attenuated retinal vessels without any peripheral retinal pigmentary changes in dilated fundus examination, and CME on optical coherence tomography. Bilateral topical brinzolamide thrice daily was initiated for CME. Visual acuity increased, and CME was resolved except for minimal schisis at the inner nuclear layer level at the third-month follow-up visit and remained stable up to one-year follow-up. CME reappeared after five months of self-discontinuation of the patient's therapy but resolved again with treatment resumption.Conclusion: We report the first case of CME secondary to rod-cone dystrophy associated with CS showing improvement in anatomy and visual acuity with a topical CAI.

The effect of COVID-19 pandemic restrictions on neovascular AMD patients treated with treat-and-extend protocol.

PURPOSE: To investigate the adherence rate of neovascular age-related macular degeneration (nAMD) patients in treat-and-extend (TAE) protocol to their anti-vascular endothelial growth factor (anti-VEGF) intravitreal injection (IVI) appointments and to evaluate the functional and anatomical outcomes of the patients who attended and did not attend their IVI appointments during the coronavirus disease 2019 (COVID-19) restriction period (RP). METHODS: The patients with nAMD having IVI appointments between March 16 and June 1, 2020 (RP in Turkey) were included in this retrospective study. For adherence analysis, the patients who attended (Group 1, n = 44) and who did not attend (Group 2, n = 60) their IVI appointment visits during the RP (VRP) were evaluated according to their last visit before the RP (V0). For outcome analysis, the patients who attend VRP and have follow-up (Group 1a, 46 eyes) and who did not attend VRP but later attended for follow-up (Group 2a, 33 eyes) were evaluated for functional (best-corrected visual acuity, BCVA [logMAR]) and anatomical (optical coherence tomography [OCT] disease activity) outcomes at the first visit after RP (V1) and last visit within six months after RP (V2). Patients received a complete ophthalmologic evaluation with anti-VEGF (Aflibercept) IVI administration at all visits. RESULTS: The adherence rate of the patients to VRP was 42.3% (44/104). The patients in Group 1 were significantly younger (mean ± SD years, 71.0 ± 8.1 vs. 74.7 ± 8.0, p = 0.024), had better median [IQR] BCVA at their first presentation (0.30 [0.54] vs. 0.61 [1.08], p = 0.023) and V0 (0.40 [0.48] vs. 0.52 [0.70], p = 0.031), and had less hypertension (36.4% vs. 58.3%, p = 0.044) than Group 2. The mean ± SD delay of planned IVI at VRP in Group 2a was 13.9 ± 6.2 weeks. Disease activity in OCT was significantly higher in Group 2a than Group 1a at V1 (60.6% vs. 32.6%, p = 0.025). In Group 2a, the median (IQR) BCVA was significantly worse at V1 (0.70 [0.58]) and V2 (0.70 [0.59]) than V0 (0.52 [0.40], p = 0.047 and p = 0.035, respectively). CONCLUSIONS: More than half of the scheduled nAMD patients in TAE protocol missed their IVI visits during the RP, which resulted in a delay of their treatments. The delay of IVI treatment in those patients resulted in an increase in OCT disease activity and a decrease in BCVA.