Radius-Maumenee syndrome-associated glaucoma, a therapeutic challenge: A case report.

INTRODUCTION: Radius-Maumenee syndrome is a rare cause of open-angle glaucoma, secondary to elevated episcleral venous pressure (EVP) without any orbital or systemic abnormalities. CASE PRESENTATION: We present a case of a male patient in his mid-sixties, who presented with bilateral dilated episcleral vessels, bilateral glaucoma, chorioretinal folds in both maculae and choroidal effusion in his left eye. Our case highlights the differentials that should be considered and the systematic investigations that should be performed. We describe the clinical, optical coherence tomography and angiography findings of this patient and propose a potential pathophysiological mechanism leading to the propensity for perioperative complications. CONCLUSION: Radius-Maumenee syndrome should be a diagnosis of exclusion. Secondary glaucoma can remain refractory to medical treatment and filtering surgery carries the risk intra-operative or post-operative uveal effusions.

Associations of ARMS2 and NR3C2 genes polymorphisms with central serous chorioretinopathy in a Greek population.

BACKGROUND: Central serous chorioretinopathy (CSCR) is characterized by serous detachment of the central neurosensory retina and it is one of the most common retinal disorders. Various genetic polymorphisms have been associated with CSCR development. METHODS: The aim of our study was to investigate the potential association between ARMS2 (rs10490924) and NR3C2 (rs2070951 and rs5522) genes polymorphisms and CSCR development in a well defined Greek cohort for the first time in literature. We enrolled, in our case-control study, 48 CSCR patients and 137 controls. The ARMS2 (rs10490924) and NR3C2 (rs2070951 and rs5522) genes polymorphisms were analyzed using Polymerase Chain Reaction (PCR) assays. RESULTS: In our study, we found significant associations between ARMS2rs10490924 and NR3C2rs2070951 single nucleotide polymorphisms and CSCR development. Specifically, the GTrs10490924 genotype frequency of the ARMS2 gene was found to be significantly associated with risk of CSCR and T allele of rs10490924ARMS2 gene was also found to increase risk for CSCR. The genotype frequency GC and CC of rs2070951NR3C2 gene were observed more frequently in CSCR patients than controls and C allele of rs2070951NR3C2 gene was also observed more frequently in CSCR patients than controls. Rs5522 of NR3C2 gene polymorphism was not found to be significantly associated with CSCR. CONCLUSION: Our findings showed, for the first time in a Greek population, that SNPs in the ARMS2 and NR3C2 genes are significantly associated with risk of CSCR. The results of this study support the involvement of extracellular matrix (ARMS2 gene) and mineralocorticoid receptor (MR) in the pathogenesis of CSCR.

Α Novel Sutureless Pterygium Excision Surgery Using Human-Derived Dehydrated Amniotic Membrane.

There are many surgical techniques available for the management of pterygium. Excision with limbal conjunctival autograft is currently the most popular surgical procedure and has been shown to have low recurrence rate and fewer complications. The fixation of limbal conjunctival autograft is performed with either sutures or fibrin glue. The use of sutures results in longer operating time and intense postoperative irritation and epiphora. We describe a new technique for pterygium excision using a human amniotic membrane-derived dry matrix (Omnigen®; Nottingham, UK: Nu-Vision Biotherapies). The use of Omnigen instead of conjunctival autograft in pterygium surgery is a new promising technique and results in shorter operation time, easier graft fixation, reduction in complications, and less postoperative discomfort. This is a simple technique for pterygium surgery with dry amniotic membrane matrix and without the need for anchoring limbal sutures or an assistant holding the graft whilst it is glued in place.

Multifocal Pattern Dystrophy Simulating Fundus Flavimaculatus: Multimodal Imaging for Early Diagnosis.

Multifocal pattern dystrophy simulating fundus flavimaculatus (MPDSFF) is a clinical entity characterized by several clinicopathological, angiographic, tomographic, and electrophysiological findings. A 58-year-old caucasian female patient presented with bilateral floaters and metamorphopsia. Best-corrected visual acuity (VA) was 6/6 in both eyes and intraocular pressure was 14 and 15 mm Hg, respectively. Fundus examination, optical coherence tomography (OCT), autofluoresence (AF), fluorescein angiography (FA) and pattern Electroretinogram were employed for the diagnosis of this case. Clinical and imaging findings were consistent with MPDSFF. Noticeable progression was observed in OCT scans 6 months following the baseline visit, while no significant changes were observed over the following 12 months. Prognosis of VA in MPDSFF patients may remain relatively good even in the presence of considerable anatomic changes. Disease progression may be slow and significant reduction in VA may present only secondary to a choroidal neovascular membrane. Patient follow-up should include OCT scans, PERG, and AF in addition to VA and dilated fundus examination every 6-12 months. As relevant literature is limited and no effective treatment modality has been employed for this clinical entity, the identification of the cellular death pathway in pattern dystrophies may lead to an applicable management approach.

Glueless and Sutureless Multi-Layer Amniotic Membrane Transplantation in a Patient With Pending Corneal Perforation.

Ocular graft-versus-host disease (GVHD) is a severe complication of allogenic hematopoietic stem cell transplantation (HSCT). It is a term used to describe a spectrum of signs and symptoms including ocular surface inflammation, dry eye syndrome, lacrimal and meibomian gland dysfunction. We present a case of a 73-year-old man with chronic myeloblastic leukaemia and chronic GVHD. On examination, severe corneal thinning was detected in his left eye. We performed multi-layer amniotic membrane patching of the affected area, in an ambulatory setting, without using sutures or glue, but only a bandage contact lens to keep amniotic membranes attached. Three months post-amniotic-membrane-patching symptoms improved, corneal integrity was maintained, and corneal thickness increased significantly. Multi-layer amniotic membrane patching without glue and sutures may be sufficient enough to prevent further deterioration of corneal thinning and can be safely performed as an outpatient procedure, reducing the need for tectonic corneal transplantation.

Large Retinectomies for Retinal Detachment Complicated by Proliferative Vitreoretinopathy: Anatomical and Functional Outcome of Silicone Oil versus Perfluoropropane Gas.

PURPOSE: To assess the anatomic and functional outcomes of eyes undergoing vitrectomy and large retinectomy for the management of retinal detachment (RD) complicated by advanced proliferative vitreoretinopathy (PVR). METHODS: A total of 66 eyes of 63 patients with RD complicated by PVR were treated with vitrectomy and 180° or more retinectomy and silicone oil (n=46) or perfluoropropane gas (n=20) were used as intraocular tamponades. RESULTS: Retinal reattachment was achieved in 89.39% (59 eyes) of the cases. The mean follow-up was 33.7 months (range 12-76 months). In gas-filled eyes, the final anatomic success rate was 85% (17 eyes) and in silicone oil-filled eyes was 91.3% (42 eyes) (p=0.46). After the initial retinectomy, the total anatomic success rate was 80.3% (53/66 eyes), 70% in gas-filled eyes (14/20 eyes) and 84.79% in silicone oil-filled eyes (39/46 eyes) (p=0.19). Visual acuity (VA) improved in 37 (56.06%) eyes, remained the same in 19 (28.78%) eyes and became worse in 10 (15.15%) cases. Best corrected VA was ≥20/200 in 22 eyes 33.33%. The final VA was associated with the preoperative VA (r=0.68), the preoperative status of the macula influence significantly the final visual acuity (p<0.0001) and there is statistically significant difference in the final visual acuity between eyes with and without anatomic success (p<0.05). CONCLUSION: The large circumferential retinectomies can be beneficial in eyes with RD complicated by advanced PVR. No significant difference was found in anatomic success rate, and rate of complications between eyes with silicone oil tamponade and long acting gas undergoing large retinectomy. Regarding the final BCVA, slight difference was found in favor of gas-filled eyes.

Histone deacetylase inhibitors as a new anticancer option: How far can we go with expectations? delivery systems.

Histone modification that occurs through the process of acetylation plays a key role in the epigenetic regulation of gene expression. The balance between histone deacetylases (HDACs) and histone acetyltransferases controls this process. Histone deacetylase inhibitors (HDACIs) can induce cancer cell cycle arrest, differentiation and cell death, reduce angiogenesis and modulate immune response. Therefore, HDAIs represent a group of enzymes that can be used for the development of pharmaceutical agents against a variety of malignant diseases. The mechanisms of their anticancer effect depend on many factors. HDACIs vorinostat, romidepsin and belinostat have been approved for some T-cell lymphomas and panobinostat for multiple myeloma. Other HDACIs are tested in clinical trials for the treatment of hematological and solid malignancies. The results of such studies are promising but further larger studies are needed.

Refractive Nightmares Revisited: Calcification of a Multifocal Intraocular Lens.

BACKGROUND/AIM: Cataract is the leading cause of reversible blindness and visual impairment worldwide. Although cataract surgery using phacoemulsification and intraocular lens (IOL) implantation is one of the commonest surgical procedures, IOL opacification remains a potential complication that can affect the visual outcome of the operation. CASE REPORT: A 50-year-old female patient presented to our clinic complaining of glare and blurry vision in her right eye over the previous 6 weeks. She had undergone bilateral refractive lens exchange elsewhere 9 months earlier. Her unaided distance visual acuity was 8/10 in the right eye and 10/10 in the left. On slit-lamp examination, we observed the presence of in-the-bag, multifocal, hydrophobic acrylic IOLs bilaterally. Specifically, calcified deposits within the substance of the IOL were observed. CONCLUSION: To our knowledge, this is the first case of spontaneous calcification of a hydrophobic multifocal IOL and all cataract/refractive surgeons should be aware of this rare complication.

The Role of Histone Deacetylase Inhibitors in Uveal Melanoma: Current Evidence.

Uveal melanoma is the most common intraocular malignancy in adults, representing approximately 3% of all melanoma cases. Despite progress in chemotherapy, radiation and surgical treatment options, the prognosis and survival rates remain poor. Acetylation of histone proteins causes transcription of genes involved in cell growth, DNA replication and progression of cell cycle. Overexpression of histone deacetylases occurs in a wide spectrum of malignancies. Histone deacetylase inhibitors block the action of histone deacetylases, leading to inhibition of tumor cell proliferation. This article reviewed the potential therapeutic effects of histone deacetylase inhibitors on uveal melanoma. MEDLINE database was used under the key words/phrases: histone deacetylase, inhibitors, uveal melanoma and targeted therapies for uveal melanoma. A total of 47, English articles, not only referring to uveal melanoma, published up to February 2018 were used. Valproic acid, trichostatin A, tenovin-6, depsipeptide, panobinostat (LBH-589), vorinostat (suberanilohydroxamic acid) entinostat (MS-275), quisinostat, NaB, JSL-1, MC1568 and MC1575 are histone deacetylase inhibitors that have demonstrated promising antitumor effects against uveal melanoma. Histone deacetylase inhibitors represent a promising therapeutic approach for the treatment of uveal melanoma.

Depression in Choroidal Melanoma Patients Treated with Proton Beam Radiotherapy.

AIM: To determine depression in patients with choroidal melanoma (CM) treated with proton beam radiotherapy. PATIENTS AND METHODS: This was a cross-sectional study including 50 patients with CM (50% males, mean age=49.88±6.34 years) and 46 age- and sex-matched healthy controls (52% males, mean age=48.60±8.05 years). Participants completed the Patient Health Questionnaire-9 (PHQ-9) and the Zung Self-Rating Depression Scale (SDS) questionnaires. RESULTS: There was a considerable difference in visual acuity as logarithm of the minimum angle of resolution (logMAR) between the patient and control groups (1.16±0.97 and 0.04±0.05 logMAR, respectively, p<0.0001). Both PHQ-9 and SDS scores differed significantly between the two groups (10.18±4.68 and 8.07±4.90, p=0.04; and 47.94±12.56 and 39.91±8.80, p=0.004, respectively). Scores appeared to be positively correlated with logMAR visual acuity (Spearman rho=0.700, p<0.0001 for PHQ-9; and 0.767, p<0.0001 for SDS), and they were also correlated to each other (Spearman rho=0.759, p<0.0001). CONCLUSION: Patients with CM having undergone proton beam therapy seem to be more depressed compared to a sample of healthy individuals, and the level of depression is correlated with their visual acuity.

Tear Film Osmolarity in Subjects with Acute Allergic Rhinoconjunctivitis.

BACKGROUND/AIM: Acute allergic rhinoconjuctivitis is the most common form of ocular allergies. The pathogenetic mechanisms are based on an immunoglobulin E (IgE)-mediated hypersensitivity reaction. On the other hand, tear osmolarity has been suggested to be an index of ocular surface damage and inflammation. These data were the motive to investigate the levels of tear osmolarity in subjects with acute allergic rhinoconjuctivitis, before and after administration of artificial tears. PATIENTS AND METHODS: Forty-five subjects with acute allergic rhinoconjuctivitis were randomly divided into three groups, based on the type of artificial tears that they received: Group A (Thera tears), Group B (Wet therapy) and Group C (Tears Naturale free). The eye drops were administered six times a day for 60 days and all subjects underwent grading of subjective symptoms and clinical examination at baseline and at the end of the treatment. RESULTS: The diagnosis of severe eye disease, which was based on ocular surface disease index (OSDI; Allergan, Inc, Irvine, CA, USA) and tear osmolarity values, concerned all patients at baseline. Although the administration of artificial tears significantly ameliorated the symptoms and the ocular variables in all groups, the results were better in the first group. Tear osmolarity was strongly and negatively correlated with tear film breakup time (BUT) and Schirmer I test at 2 months. Contrariwise, symptoms were eliminated, when tear osmolarity was decreased. CONCLUSION: Acute allergic rhinoconjuctivitis is characterized by tear hyperosmolarity, which can be rehabilitated with the administration of hypotonic artificial tears.

Trabeculectomy with or without Anterior Chamber Maintainer: A Study on Intraocular Pressure, Endothelial Cells, and Central Corneal Thickness.

PURPOSE: To compare the impact of trabeculectomy and trabeculectomy with an anterior chamber (AC) maintainer on intraocular pressure (IOP), endothelial cell density (ECD), and central corneal thickness (CCT) in patients with primary open angle glaucoma (POAG). METHODS: The two groups consisted of 36 (trabeculectomy, Group A) and 42 (trabeculectomy with AC maintainer, Group B) patients with POAG. IOP, CCT, and ECD were measured one day prior to surgery, one month, six months, and 12 months postoperatively. RESULTS: No complications were observed. The mean decrease of mean IOP from baseline to 12 months was statistically significant for both groups (all p < 0.0001), but more significant in Group B (p = 0.01). ECD decrease was significant in both Groups (all p < 0.01), but more significant in Group A (p < 0.0001). CCT decrease was also greater in Group A (p < 0.0001). The surgical procedure's duration was significantly longer in Group B than in Group A (p < 0.0001). CONCLUSIONS: Both techniques seem to be effective and safe options for patients with POAG. Using an AC maintainer might add some safety for the ECD and a bit greater IOP reduction; however, the classic technique, which is of less complexity and less time-consuming, has comparable results.

Postoperative eccentric macular holes after vitrectomy and internal limiting membrane peeling.

The purpose of this study was to describe the incidence, clinical characteristics, and outcome of eccentric macular holes presenting after vitrectomy and internal limiting membrane (ILM) peeling for the treatment of macular pathology and discuss the pathogenesis of holes formation. A retrospective, noncomparative, interventional case-series study of five patients who developed eccentric macular holes postoperatively following vitrectomy in 198 consecutive patients who underwent ILM peeling for idiopathic macular hole and epiretinal membrane formation between 2008 and 2015. Five patients (2.5 %) developed full-thickness eccentric macular holes postoperatively. Three patients presented with a single eccentric macular hole, one patient had an eccentric hole after a failed idiopathic macular hole surgery and one patient developed four eccentric macular holes. The mean diameter of the holes was 584 µm (range 206-1317 µm) and the average time of holes formation after vitrectomy was 27.7 weeks (range 1-140 weeks). Postoperative best-corrected visual acuity ranged from "counting fingers" to 20/25. The eyes with the holes distant from the fovea had the best final visual acuity. No further intervention was attempted and no complications occurred. The mean follow-up time was 26.8 months. The postoperative macular holes after vitrectomy and ILM peeling were variable in number, size, and time of appearance but remained stable and were not associated with any complications. The pathogenesis of macular holes is most consistent with contraction of the residual ILM or secondary epimacular proliferation probably stimulated by ILM peeling.

Polymorphism Analysis of GSTM1 and OPA1 Genes in Greek Patients with Primary Open-angle Glaucoma.

BACKGROUND: Glaucoma is a heterogenous group of optic neuropathies leading to progressive degeneration of the optic nerve and vision loss. Over the past decades, disease-causing genes have been identified and multigenic inheritance theory has-beens investigated for many cases of glaucoma. The purpose of this study was to investigate the distribution of mutations in glutathione S-transferase M1 (GSTM1) and optic atrophy 1 (OPA1) genes in a series of patients of Greek origin. PATIENTS AND METHODS: This was a case-control study of 106 patients with primary open-angle glaucoma (POAG) and 120 healthy controls of Greek origin, surveyed for polymorphisms with potential correlation to POAG. A DNA sample from each individual was genotyped for GSTM1 and OPA1 (rs166850, rs10451941) polymorphisms. RESULTS: GSTM1 null genotype carriers seem to have an increased risk of developing POAG (odds ratio=1.86, 95% confidence interval=1.07-3.21; p=0.03). The results indicate that the OPA1 genotype (rs166850 and rs10451941 polymorphisms) is not significantly associated with POAG. CONCLUSION: The GSTM1 null genotype might be associated with increased risk of development of POAG in the Greek population. No significant correlation was found between OPA1 polymorphisms and POAG.

Estimation of depression prevalence in patients with Stargardt disease using PHQ-9 and Zung scores.

PURPOSE: To evaluate the psychological impact and depression prevalence in patients with Stargardt disease. METHODS: We conducted a case-control study including 39 patients with Stargardt disease and 32 age- and sex-matched healthy controls. All participants underwent a complete ophthalmologic examination and completed the Patient Health Questionnaire-9 (PHQ-9) and the Zung Depression Inventory questionnaire. Results were analyzed using IBM SPSS 22.0 software. RESULTS: The patient group consisted of 19 men and 20 women with mean age of 36.9 ± 5.4 years and control group of 19 men and 13 women with mean age of 42.5 ± 10.1 years. The mean values of PHQ-9 and Zung scores for patients and healthy individuals were 10.9 ± 4.9, 46.7 ± 11.1, 6.7 ± 5.4, and 41.1 ± 8.5, respectively. There were statistically significant differences between the 2 groups in PHQ-9 scores (independent samples t test: p = 0.001), but not in Zung scores (Mann-Whitney test: p = 0.053). The PHQ-9 and Zung scores appeared to be moderately but significantly correlated (Pearson coefficient 0.44, p<0.0001). In addition, PHQ-9 score seems to be raised along with age, whereas both scores are low when best-corrected visual acuity is high. CONCLUSIONS: Patients with Stargardt disease exhibited more depressive symptoms compared to healthy individuals according mainly to PHQ-9 scores. Moderate depression was significantly correlated visual function decline.