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Purpose: To assess the possible association between MIR200B variations and sight-threatening diabetic retinopathy (STDR). Methods: A total number of 141 diabetes mellitus patients were enrolled in the study and divided into two groups including 76 patients diagnosed with STDR assigned to the case group, and 65 subjects without STDR considered in the control group. Peripheral blood specimens were used to extract the DNA content, and the primary MIR200B encoding sequence was amplified using a polymerase chain reaction. Then, the amplified DNA was sequenced by the Sanger method. The sequences were compared to the MIR200B reference sequence to find sequence variations. RNAfold, miRVaS, and Mfold bioinformatics web servers were employed to predict the potential effects of the identified variations on RNA structure. Results: Two MIR200B gene variants were identified. Although both variations were found more frequent in cases than controls, statistical analysis of allelic and genotypic features did not reach statistical significance. Conclusions: In silico analysis showed mild changes in MIR200B secondary structure and increased free energy in the presence of one of the identified variants (g.1167183G>A; rs72563729). Increasing the sample size in future studies may help a more accurate interpretation of the allelic association of MIR200B variations with STDR.

Abstract Purpose: To report a case of adult-onset asthma and periocular xanthogranuloma (AAPOX) in a patient with intermediate uveitis and a history of Hodgkin's lymphoma (HL). Case Report: A 51-year-old man with a past medical history of HL presented with blurred vision, asthma, and bilateral yellowish eyelid lesions. The eyelid lesions and asthma appeared 10 years after being diagnosed with HL. Physical examination was significant for multiple subcutaneous and firm eyelid masses in addition to the presence of pre-auricular and submandibular lymphadenopathies. Ophthalmic examination revealed bilateral intermediate uveitis and mild macular edema. Further systemic evaluations, including laboratory testing and imaging, were normal. Excisional biopsy of the eyelid lesions was performed and the histopathologic examination was consistent with the diagnosis of AAPOX. Conclusion: The presence of AAPOX in a patient with intermediate uveitis and history of HL suggests that immunological dysfunction may play a role in the pathogenesis of adult orbital xanthogranulomatous disease.

Abstract Purpose: To report the first case of ocular syphilis in an Iranian patient and discuss its diagnostic challenges. Case Report: A man in his mid-70s presented with progressive bilateral visual and auditory decline. He had previously lived in a Southeast Asian country for 10 years. Prior steroid therapies entailed no inflammation subsidence. His visual acuity at presentation was light perception OU. Funduscopic findings included severe vitritis, severe optic atrophy, diffuse retinal vascular occlusion, and diffuse retinal atrophy OU. Angiography demonstrated diffuse areas of retinal and choriocapillaris atrophy with no active choroiditis. Scaly cutaneous lesions were noted on his palms and soles – atypical findings of secondary syphilis. Serum analysis revealed an underlying syphilis infection. The cerebrospinal fluid sample was reactive to anti-syphilis antibodies, securing a neurosyphilis diagnosis. Two weeks of antibiotic therapy resulted in cutaneous lesions resolution and relative visual improvement despite extensive baseline retinal atrophic damage. Conclusion: Ocular syphilis can mimic numerous ocular inflammatory scenarios. In cases of ocular inflammation that is unresponsive to steroids, reconsidering alternative diagnoses, especially infections with the highest clinical relevance, is necessary. We stress the importance of acquiring patients' sexual history, regardless of cultural barriers and the rarity of the entity in some regions.

Abstract Background To study the clinical utility of broad-range real-time Polymerase Chain Reaction (PCR) assay in patients suspected for infectious uveitis and to analyze the clinical relevance. Methods Medical records of patients with uveitis were assessed in whom PCR analysis of intraocular fluids was performed between January 2018 and February 2021. Intraocular samples were investigated for cytomegalovirus (CMV), Epstein-Barr virus (EBV), varicella zoster virus (VZV), herpes simplex viruses type 1 and 2 (HSV1,2), human T-lymphotropic virus type 1 (HTLV-1), Toxoplasma gondii and also for bacterial 16 S and fungal 18 S/28S ribosomal DNA (rDNA). Results Aqueous paracentesis and vitreous sampling was done for 151 (81.2%) and 35 (18.8%) patients, respectively. Most of the patients had panuveitis (61.3%). PCR results were positive in 69 out of 186 patients (37%) according to the following order: CMV (18 cases), VZV (18 cases), fungal 18s/28s rDNA (17 cases), HSV (9 cases), bacterial 16s rDNA (3 cases), HTLV-1 (2 cases), and Toxoplasma gondii (2 cases). PCR positivity rate was 5.8% in patients with undifferentiated panuveitis. EBV was not detected at all. Initial treatment was changed in 38 patients (20%) based on PCR results. The overall sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of PCR test for aqueous samples was 82%, 91%, 96%, and 87%, respectively. No significant adverse effect related to sampling was reported. Conclusion PCR analysis of intraocular fluids in patients with suspected infectious uveitis plays an important role in confirming diagnosis or changing treatment with good predictive value. However, routine PCR test in patients with undifferentiated panuveitis in order to rule out possible underlying infectious etiology had low benefit.

Abstract Purpose To evaluate the choroidal structure in patients with inherited retinal diseases (IRDs) by investigating the choroidal vascularity index (CVI). Methods The present study was conducted on 113 IRD patients and 113 sex- and age-matched healthy individuals. Patients’ data was extracted from the Iranian National Registry for IRDs (IRDReg®). Total choroidal area (TCA) was determined between retinal pigment epithelium and choroid-scleral junction,1500 microns on either side of the fovea. Luminal area (LA) was considered as the black area corresponding to the choroidal vascular spaces, following Niblack binarization. CVI was calculated as the ratio of the LA to the TCA. CVI and other parameters were compared among different types of IRD and the control group. Results The IRD diagnosis included retinitis pigmentosa (n = 69), cone-rod dystrophy (n = 15), Usher syndrome (n = 15), Leber congenital amaurosis (n = 9), and Stargardt disease (n = 5). Sixty-one (54.0%) individuals of each of the study and control groups were male. The average CVI was 0.65 ± 0.06 in the IRD patients and 0.70 ± 0.06 in the control group (P

Abstract Background To investigate the adjunctive effect of an intravitreal ROCK inhibitor (fasudil) in combination with intravitreal bevacizumab (IVB) on refractory macular edema secondary to retinal vein occlusion (RVO). Methods In this prospective interventional case series, 17 eyes of 17 patients (10 men, 7 women) with refractory RVO-related macular edema underwent three consecutive intravitreal injections of bevacizumab plus fasudil. Monthly evaluation was continued up to 12 months and IVB injection was performed if needed during the follow-up. Changes in the best corrected visual acuity (BCVA) was the primary outcome measure. The secondary outcome measures included central macular thickness (CMT) changes and any adverse events. Results BCVA significantly improved (mean change: −0.15 LogMAR; P = 0.017) after 3 consecutive intravitreal injections of fasudil in combination with bevacizumab. CMT significantly decreased (mean change: −206 µm; P = 0.028). The anatomical and functional improvement was maintained during the 12 month follow-up. No adverse effects were noticed. Conclusion Intravitreal ROCK inhibitors may break the resistance to anti-VEGF therapy and improve the RVO induced macular edema via affecting the VEGF-independent pathways.

Purpose: To report a case of non-paraneoplastic autoimmune retinopathy (npAIR) treated with intravenous immunoglobulin (IVIG). Case report: A 12-year-old boy presented with progressive visual field loss, nyctalopia, and flashing for three months. He had suffered from common cold two weeks before the onset of these symptoms. On the basis of clinical history and paraclinical findings, he was diagnosed with npAIR, and IVIG without immunosuppressive therapy was started. During the one-year follow-up period after the first course of IVIG, flashing disappeared completely. Visual acuity remained 10/10, but nyctalopia did not improve. Multimodal imaging showed no disease progression. Conclusion: Although established retinal degenerative changes seem irreversible in npAIR, IVIG may be a suitable choice to control the disease progression.

Purpose: To report the genetic analysis of an Iranian Bietti crystalline dystrophy (BCD)-affected family, and to review previously reported mutations in the gene and assess the distribution of affected amino acids in the encoded protein. Methods: The eleven exons of CYP4V2 were sequenced in the DNA of the proband of the Iranian BCD family. The putative disease-causing variation was screened in all affected and non-affected members. BCD causing CYP4V2 mutations previously reported in the literature were compiled, and positions of amino acids affected by nonsense and missense mutations were mapped onto the primary structure of the CYP4V2 protein. Results: C.1219G > T in CYP4V2 that causes p.Glu407* was identified as cause of BCD in the Iranian family. The mutation segregated with disease status. Clinical presentations were similar among affected members, except that one patient presented with retinal macular hole. Twelve nonsense and 47 missense mutations in CYP4V2 were compiled. Inspection of distribution of amino acids affected by the mutations suggested non-random distribution and clustering of affected amino acids in nine regions of the protein, including regions that contain the heme binding site, the metal binding site, and a region between these binding sites. The most C-terminus proximal nonsense mutation affected position 482. Conclusions: This study presents results of the genetic analysis of an Iranian BCD family. Protein regions affected by mutations within the nine mutation clusters include regions well conserved among orthologous proteins and human CYP4 proteins, some of which are associated with known functions. The findings may serve to identify reasonable candidate gene region targets for gene editing therapy approaches. Keywords: p.Glu407*, Bietti crystalline dystrophy, BCD, CYP4V2, CYP4V2 mutations

Purpose: To report a case of unilateral retinal detachment with a large subretinal macrocyst, representing an atypical presentation of ocular toxoplasmosis. Case Report: A healthy 30-year-old woman presented with a two month history of progressive visual loss in her right eye. Funduscopy revealed vitreous condensations, total retinal detachment with a large subretinal orange-red cystic mass and multiple retinal breaks sealed with surrounding retinal scars. B-scan echography showed a large subretinal cyst with non-homogenous tissue echogenicity. Pars plana deep vitrectomy and complete cyst removal were performed. Histopathologic examination of the excised cyst revealed intraretinal toxoplasma cysts containing bradyzoites. Analysis of intraocular fluids by polymerase chain reaction (PCR) and serologic tests also supported the diagnosis. After six months, the retina was completely attached with no signs of inflammation. Conclusion: Toxoplasma retinochoroiditis should be considered in the differential diagnoses of retinal detachment with subretinal cyst.

Purpose: To compare the anterior segment indices measured by two Scheimpflug camera machines; Galilei and Pentacam. Methods: In this observational case series, the anterior segment indices of myopic healthy subjects seeking for refractive surgery were measured by Pentacam and Galilei on the same day. Analyzed parameters were anterior and posterior best fit spheres (BFS), axial curvature, true corneal power, central corneal thickness (CCT), anterior chamber (AC) depth, AC volume, AC angle, and pupil diameter. Results: This study included 176 eyes of 88 participants. Mean radius of the anterior BFS was 7.79 ± 0.34 mm versus 7.75 ± 0.39 mm measured by Pentacam and Galilei, respectively (r = 0.877, P < 0.001). Corresponding values for the mean radius of posterior BFS were 6.42 ± 0.32 and 6.47 ± 0.38 mm, respectively (r = 0.879, P < 0.001). Anterior corneal mean power was 43.8 ± 1.9 diopters (D) with Pentacam and 43.8 ± 2.4 D with Galilei (r = 0.905,P < 0.001). Posterior corneal mean power was measured − 6.3 ± 0.3 and − 6.3 ± 0.4 D using Pentacam and Galilei, respectively (r = 0.873, P < 0.001). True corneal power was 43.9 ± 1.9 D with Pentacam and 43.5 ± 2.3 D with Galilei (r = 0.909, P < 0.001). CCT was 537 ± 44 and 553 ± 51 μm measured by Pentacam and Galilei, respectively (r = 0.796, P < 0.001). AC depth measurements using Pentacam and Galilei were 3.29 ± 0.4 and 3.3 ± 0.38 mm (P < 0.001), respectively; AC volume was 207 ± 50 and 129 ± 39 mm3≥ (P = 0.004), and AC angle was 39.7 ± 9.2 and 54.2 ± 5.2 degrees (P = 0.051), respectively. Average pupil diameter was measured 3.91 ± 1.77 mm by Pentacam and 3.34 ± 0.89 mm by Galilei (P = 0.018). Conclusions: There was a significant correlation between the Pentacam and Galilei in all measured parameters except AC angle, AC volume, and average pupil diameter.

Purpose: To compare the efficacy and side effects of loteprednol versus fluorometholone after myopic photorefractive keratectomy (PRK). Methods: One hundred and twenty four eyes of 62 patients who underwent PRK were enrolled in this study. One eye of each subject was randomized to receive loteprednol 0.5% and the fellow eye was given fluorometholone 0.1%. Patients were followed up for three months. Results: There was no significant difference in uncorrected distance visual acuity (UDVA), corrected distance visual acuity (CDVA), manifest refraction, corneal haze, intraocular pressure (IOP), and ocular discomfort and redness between groups at the final visit. At 3 months postoperatively, 20/25 or better UDVA was achieved in 95% of the loteprednol group and 92% of the fluorometholone group (P > 0.05). There was neither visually significant corneal haze nor ocular hypertension (IOP rise > 10 mmHg or IOP > 21 mmHg) in any group. Conclusion: The efficacy and side effects of loteprednol 0.5% and fluorometholone 0.1% after myopic PRK are comparable.

Purpose: To describe a 42-year-old man, a known case of Bietti crystalline dystrophy who underwent surgery for unilateral full thickness macular hole. Case Report: clinical features, color fundus photographs, and optical coherence tomography, electroretinography, and electrooculography findings of the patient are reported. His visual acuity improved from counting fingers to 20/50 after pars plana deep vitrectomy with internal limiting membrane (ILM) peeling and gas injection. Conclusion: Macular hole can occur in Bietti crystalline dystrophy and the post-surgical outcome is good.

Purpose: To compare the corneal power measurements obtained using different topographic instruments after myopic photorefractive keratectomy (PRK). Methods: Patients with myopia who were candidates for corneal refractive surgery were sequentially included. Pre-PRK and six months post-PRK corneal powers were measured using Javal manual keratometer, Orbscan II, Galilei, Tomey TMS4, and EyeSys 2000 topographers. Measured values were compared with those obtained using the clinical history method (cHM). Results: This study included 66 eyes of 33 patients. The lowest keratometric measurements were obtained using the Galilei topographer (42.98 ± 1.69 diopters, D) and the highest measurements were obtained using the Javal manual keratometer (43.96 ± 1.54 D) preoperatively. The same order was observed postoperatively. Effective refractive power (EffRP) measured using EyeSys was most similar to the values obtained using cHM (Icc, intraclass correlation coefficient = 0.951), followed by the total corneal power measured using the Galilei system (Icc = 0.943). The values obtained using the adjusted EffRP formula (EffRP – 0.015*Δ Refraction – 0.05) were more consistent with the values obtained using cHM (Icc = 0.954) compared to those obtained with the adjusted average central corneal power formula measured using the Tomey system (Icc = 0.919). Conclusion: Post-PRK corneal powers measured using the adjusted EffRP formula were the most similar to values obtained using cHM.