Efficacy and safety of dexamethasone intravitreal implant in patients with retinal vein occlusion resistant to anti-VEGF therapy: a 12-month prospective study.

Purpose: To evaluate the safety and efficacy of repeated intravitreal dexamethasone implant (Ozurdex) injections administrated on an "as-needed" protocol for retinal vein occlusion patients with macular oedema, previously subjected to at least five anti-vascular endothelial growth factor (VEGF) injections with poor or no response. Methods: Prospective interventional case series of 13 branch retinal vein occlusion (BRVO) and 10 central retinal vein occlusion (CRVO) patients with persistent macular oedema (>250 µm) after at least five anti-VEGF injections. Exclusion criteria included: baseline visual acuity worse than 1.5 logMAR, previous intravitreal implant, history of vitreoretinal surgery, manifest glaucoma or ocular hypertension, epiretinal membrane, retinal neovascularization, massive retinal or macular ischaemia, vitreous haemorrhage or severe lens opacity, previous laser photocoagulation treatment. Each patient received an initial intraocular dexamethasone implant and the procedure was repeated at 6 months "as needed." Patients were followed up at months 2, 4, 6, 8, 10 and 12 with spectral domain optical coherence tomography and best corrected visual acuity measurements. Exclusion criteria included: baseline visual acuity worse than 1.5 logMAR, previous intravitreal implant, history of vitreoretinal surgery, manifest glaucoma or ocular hypertension, epiretinal membrane, retinal neovascularization, retinal or macular ischaemia, vitreous haemorrhage or severe lens opacity, previous laser photocoagulation treatment. Patients on topical or systemic corticosteroid therapy (during the last 3 months), and known steroid responders as well as diabetic patients were also excluded. Results: In the BRVO group, the mean central retinal thickness (CRT) and best corrected visual acuity (BCVA) significantly improved from 482.92 ± 139.99 µm (0.55 ± 0.12 logMAR) at baseline, to 369.31 ± 119.72 µm (0.43 ± 0.18 logMAR) at 6 months (p = 0.011/p = 0.019). At 12 months CRT was 295.82 ± 135.48 µm (p = 0.026) and BCVA 0.29 ± 0.17 logMAR (p = 0.002). Minimum CRT values were achieved at 3.45 months after the first injection, and 2.46 months after the second injection (197.00 ± 84.27 and 180.00 ± 76.89 µm, respectively). Best BCVA values were achieved at a mean of 4 ± 0.853 months after the first injection, and 4 months after the second injection (0.219 ± 0.129 and 0.222 ± 0.078 logMAR, respectively). In the CRVO group, neither the mean CRT nor BCVA improved significantly at 6 months: from 669.70 ± 203.20 µm (0.80 ± 0.231 logMAR) at baseline, to 586.20 ± 237.63 µm (0.740 ± 0.268 logMAR) at 6 months (p = 0.131/p = 0.333). At 12 months CRT was significantly improved: 549.90 ± 191.26 µm (p = 0.047), but BCVA lacked significant improvement: 0.690 ± 0.285 logMAR (p = 0.072). Minimum CRT values were achieved at a mean of 2 months after the first injection, and also 2 months after the second injection (261.60 ± 121.31 and 280.00 ± 177.43 µm, respectively). Best BCVA values were achieved at a mean of 2 months after the first injection, and 2 months after the second injection and were 0.390 ± 0.173 and 0.385 ± 0.233 logMAR, respectively. Cataract progression was a rare event (2/23 eyes), while transient steroid-induced ocular hypertension (5/23 eyes) was managed successfully with IOP-lowering medication Conclusion: Dexamethasone implant should be considered as an effective and safe alternative in patients with BRVO and CRVO who have failed anti-VEGF therapy. Shortening the re-injection interval especially for CRVO cases should be considered.

Long-Term Results of Combination Treatment with Single-Dose Ranibizumab plus Photodynamic Therapy for Retinal Angiomatous Proliferation.

PURPOSE: To present functional and anatomic outcomes of combination therapy with ranibizumab and photodynamic therapy (PDT) in a series of retinal angiomatous proliferation (RAP) cases. METHODS: A total of 17 eyes of 17 patients with RAP were included. Thirteen eyes were treatment naïve and 4 were already on ranibizumab monotherapy. Combined treatment with single-dose ranibizumab and PDT was performed on all treatment-naïve cases. Follow-up was performed every month for 6-38 months, using optical coherence tomography and indocyanine green angiography when necessary. RESULTS: Overall, 6 out of 7 treatment-naïve cases with stage I or II disease manifested complete angiographic resolution of the hot spot with a single injection of ranibizumab plus PDT. In stage III disease, 7 out of 10 eyes showed persistent leakage immediately after the combined treatment. The rate of hot spot occlusion was found to correlate with the stage of disease (p = 0.05). There was a significant improvement in posttreatment BCVA for all patients regardless of disease stage (p = 0.02), which was more evident in the earlier stages. Improvement was also greater for treatment-naïve patients (p = 0.03). The posttreatment improvement in BCVA was strongly correlated with hot spot occlusion (p < 0.001). CONCLUSIONS: Single-dose modified anti-VEGF treatment in combination with PDT may provide long-term regression at the initial stages of RAP.

CAPILLARY NONPERFUSION AND PHOTORECEPTOR LOSS IN BRANCH RETINAL VEIN OCCLUSION: Spatial Correlation and Morphologic Characteristics.

PURPOSE: To evaluate the photoreceptor layer in eyes with branch retinal vein occlusion associated with macular ischemia, using a method of en face optical coherence tomography (OCT) representation of the ellipsoid zone. METHODS: Customized macular OCT scans of 9 patients (10 eyes) with branch retinal vein occlusion and macular ischemia were exported and subsequently postprocessed (removal of vascular and cystic spaces' shadows, segmentation, and alignment to the retinal pigment epithelium). The ellipsoid band was then isolated, aligned, and used to produce an en face OCT image. Areas with photoreceptor loss (hyporeflective ellipsoid) were compared with ischemic areas as identified in an early-phase fluorescein angiography. RESULTS: The areas of capillary nonperfusion (as detected in fluorescein angiography) were closely associated with disruption of the ellipsoid zone (depicted as areas of low reflectance in the en face reconstruction of the OCT images). The ellipsoid zone disruption had a patchy appearance and either sharp or fuzzy borders, depending on the grade of the loss of reflectance. CONCLUSION: En face OCT reconstruction and subsequent representation of ellipsoid zone revealed a close association between capillary nonperfusion and photoreceptor disruption in eyes with branch retinal vein occlusion. It seems that the deep capillary plexus plays an important role on the metabolic demands of outer retina and, consequently, an ischemia at the level of deep capillary plexus has significant impact on the integrity of the photoreceptors.

Case report: Aqueous and Vitreous amino-acid concentrations in a patient with maple syrup urine disease operated on rhegmatogenous retinal detachment.

BACKGROUND: Maple syrup urine disease (MSUD) is a rare metabolic disorder, affecting the metabolism of branched chain amino-acids (Valine, Leukine, Isoleukine). We present a rare case of rhegmatogenous retinal detachment (RRD) in a MSUD patient. CASE PRESENTATION: We performed amino acid analysis of aqueous humour, vitreous and serum samples obtained during surgery from a 24 year old female MSUD patient successfully operated on RRD. Serum values for a-amino-butyric acid, valine, isoleucine, leucine, tyrosine, phenylalanine, ornithine and histidine were low, while values for citrulline, methionine and lysine were borderline low, all attributed to the patient's special diet. Serum glutamate was above normal, probably due to the breakdown of glutamine to glutamate. In the aqueous and vitreous the amino acids implicated in MSUD (Valine, Leukine Isoleukine), were within normal range. Glutamate was absent in the vitreous and presented low levels in the aqueous. Glutamate has been reported to play an important role in retinal damage. Elevated glutamate levels have been reported in vitreous specimens from patients subjected to vitrectomy or buckling surgery for RRD. In MSUD, glutamate has been implicated in the pathogenesis of brain damage. Low levels of glutamate have been observed in the cerebellum of experimental MSUD animals, as well as postmortem brain tissue from a child that died of leucine intoxication. The reduction was attributed to the elevation of a-ketoisocaproic which reverses the net direction of nitrogen flow. It could be argued that this could impact on amino acid concentration in aqueous and vitreous fluids. CONCLUSIONS: Although no definite conclusions can be drawn by this extremely rare case, the low vitreous and aqueous levels of Glutamate is an interesting finding. Further studies are needed to provide a better insight in the role of amino acids as neurotransmitters in the human eye in health and disease.

ß-Thalassemia and ocular implications: a systematic review.

BACKGROUND: Beta-thalassemia is a severe genetic blood disorder caused by a mutation in the gene encoding for the beta chains of hemoglobin. Individuals with beta-thalassemia major require regular lifelong Red Blood Cell transfusions to survive. Ocular involvement is quite common and may have serious implications. METHODS: Extensive review of observational studies on beta-thalassemia, to determine the prevalence and spectrum of ocular abnormalities, by clinical examination and multimodal imaging, and to investigate risk factors for their development. RESULTS: Frequency of ocular involvement differs among various studies (41.3-85 %, three studies). Ocular findings in beta-thalassemia may correlate to the disease itself, iron overload or the chelating agents used. Beta-thalassemia ocular manifestations include ocular surface disease, as demonstrated by tear function parameters (two studies). Lens opacities are present in 9.3-44 % (five studies). Lenticular opacities and RPE degeneration correlated positively with use of desferrioxamine and deferriprone respectively (two studies). Ocular fundus abnormalities characteristic of pseudoxanthoma elasticum (PXE), including peau d'orange, angioid streaks, pattern dystrophy-like changes, and optic disc drusen are a consistent finding in seven studies. Patients with PXE-like fundus changes were older than patients without these fundus changes (two studies). Age (two studies) and splenectomy (one study) had the strongest association with presence of PXE-like fundus changes. Increased retinal vascular tortuosity independently of the PXE-like fundus changes was found in 11-17.9 % (three studies), which was associated with aspartate amino transferase, hemoglobin and ferritin levels (two studies). Fundus autofluorescence and electrophysiological testing (ERG and EOG) may indicate initial stages or more widespread injury than is suggested by fundus examination (two studies). CONCLUSIONS: Beta-thalassemia may present with various signs, both structural and functional. Pseudoxanthoma elasticum like fundus changes are a frequent finding in patients with b-thalassemia. These changes increase with duration or severity of the disease. Retinal vascular tortuosity may be an additional disease manifestation related to the severity and duration of anemia and independent of the PXE-like syndrome. Patients with long-standing disease need regular ophthalmic checkups because they are at risk of developing PXE-like fundus changes and potentially of subsequent choroidal neovascularization.

Scleral Buckling versus Vitrectomy for Retinal Detachment Repair: Comparison of Visual Fields and Nerve Fiber Layer Thickness.

PURPOSE: To compare visual field loss and retinal nerve fiber layer (RNFL) defects in cases of rhegmatogenous retinal detachment (RRD) treated with scleral buckle (SB) versus pars plana vitrectomy (PPV) and C3F8 injection. METHODS: This was a prospective, comparative interventional study of 50 eyes with primary RRD, treated with PPV (25 eyes) or SB (25 eyes). All measurements took place at least 9 months following successful and uncomplicated surgical treatment. The visual field total deviation (TD) values for preoperative attached and detached areas were calculated and compared separately. The optic nerve head morphology was studied with Heidelberg retinal tomography (HRT), and the RNFL using spectral-domain optical coherence tomography. RESULTS: The preoperative detached areas demonstrated more affected TD values (in dB) compared to the preoperative attached areas (-6.9 ± 5.2 vs. -4.3 ± 3.3 for the SB group and -9.6 ± 5.2 vs. -7.8 ± 5.1 for the PPV group; p = 0.001) in both groups. The preoperative attached areas of the SB group showed better TD values (calculated mean values) compared to the preoperative attached areas of the PPV group (-4.3 ± 3.3 vs. -7.8 ± 5.1, p = 0.007). The RNFL and HRT values showed no statistically significant difference between the two groups. CONCLUSIONS: It seems that the preoperative detached retina, despite successful reattachment, suffers permanent damage as a result of the detachment, irrespective of the method of treatment. In the PPV group, the postoperative functionality of the preoperative attached areas was detected to be worse compared to the postoperative functionality of the preoperative attached areas of the SB group. We postulate that this fact could be attributed to an additional traumatizing factor (possibly fluid-air exchange or gas injection) in patients with RRD treated with PPV.

Prototype posterior chamber phakic IOL - 35 year follow up.

Purpose: To present a case involving a rarely seen prototype posterior chamber phakic IOL (PC-pIOL) in a highly myopic patient with bilateral cataract. Observations: A 64-year-old male presented to our clinic with poor vision in both eyes. Clinical examination revealed bilateral mature cataract, phacodonesis as well as a PC-pIOL implanted 35 years ago to address his high myopia. The visual acuity (VA) was 20/200 in the right eye and no light perception in the left eye. PC-pIOL extraction as well as 23G pars plana vitrectomy (PPV) and fragmentation surgery was scheduled for the right eye. The left eye was treated conservatively. Successful extraction of the PC-pIOL was performed while it was easy to remove. It was a bow-tie shaped lens with a collar-stud-like button in the middle which extended anteriorly into the anterior chamber through the pupil. PPV with lens fragmentation was successful and the patient was left aphakic in order to avoid the placement of a zero diopter IOL. Final best corrected VA was 20/25 one month post-surgery. Conclusions and importance: Removal of this rarely seen pIOL was performed without difficulty while excellent VA was achieved. Aphakia following complete vitrectomy represented a viable option in this case. Furthermore, we highlight the clinical manifestations associated with this IOL more than three decades after implantation.

UGH Syndrome Resolution after IOL Explantation and Concomitant Carlevale IOL Implantation.

OBJECTIVE: To examine the use of Carlevale IOL placement in patients with UGH, and to evaluate surgical outcomes. DESIGN: In this retrospective study, 28 patients with UGH syndrome that were subjected to IOL explantation and concomitant Carlevale IOL implantation were included in the study. METHODS: Information about VA, IOP, number of glaucoma medication, need for glaucoma surgery, presence of hemorrhage and inflammation were recorded up to 6 months after the procedure. RESULTS: We found a statistically significant increase in mean visual acuity and complete resolution of uveitis in all patients. Mean IOP and the mean number of glaucoma medications were significantly decreased postoperatively, while 14% of patients required additional glaucoma surgery. CONCLUSIONS: IOL explantation and concomitant Carlevale IOL implantation may provide a viable solution for UGH syndrome resolution, increases visual acuity, and decreases the need for glaucoma medication.

Optical Coherence Tomography Angiography Changes in Patients Diagnosed With Acute Coronary Syndrome: A Systematic Review and Meta-Analysis.

We conducted a systematic review and meta-analysis to assess the association between optical coherence tomography angiography (OCTA) parameters and acute coronary syndrome (ACS). Two independent reviewers searched the electronic databases (MEDLINE (Medical Literature Analysis and Retrieval System Online), Scopus, Embase (Excerpta Medica Database), Cochrane Library, ClinicalTrials.gov, and World Health Organization International Clinical Trials Registry Platform) from inception until April 2023. According to the inclusion criteria of this review, eligible were observational studies, randomized control trials, and registry/database studies that included the eyes of adult ACS patients and assessed OCTA parameters within the macula. The pooled standardized mean differences (SMD) between patients diagnosed with ACS and healthy controls with a confidence interval (CI) of 95% were calculated using the Hartung-Knapp-Sidik-Jonkman random-effects method. The heterogeneity was assessed by I2 and the Cochran Q and a random effects model was applied. Seven studies were eligible and included in our systematic review (n = 898), of which three were included in the meta-analysis (n = 341). The pooled SMD in the superficial vascular plexus (SVP), deep vascular plexus (DVP), and foveal avascular zone (FAZ) were -0.46 (95% CI: -0.94 to 0.01, p = 0.05, I2 = 0%, three studies), -0.10 (95% CI: -3.20 to 3.00, p = 0.75, I2 = 67%, two studies), and 0.43 (95% CI: -1.22 to 2.09, p = 0.38, I2 = 92%, three studies), respectively. Our findings suggest that there are no differences in OCTA metrics between ACS patients and healthy individuals.

Late PAMM-Like Lesions in a Patient with HIV Retinopathy.

This report describes a case of a newly diagnosed 49-year-old HIV patient, who presented with decreased visual acuity and retinal lesions characterized by ischemia at the level of the deep retinal capillary plexus, documented with optical coherence tomography (OCT), OCT angiography, fluorescein angiography, and visual fields testing. These lesions closely resembled the morphologic and clinical characteristics of late paracentral acute middle maculopathy. The presence of these lesions suggests that HIV microangiopathy can potentially affect both superficial and deep retinal capillary plexuses.

Impact of flight and equivalent short-term high-altitude exposure on ocular structures and function.

Background: Exposure to high-altitude conditions during flight or similar activities affects many aspects of visual function, which is critical not only for flight safety but for any altitude-related activity. We aimed to summarize the available literature pertaining to ocular changes during flight or equivalent short-term high-altitude exposure (e.g., hypobaric chamber, effortless ascent lasting ≤ 24 h) and to highlight future research priorities. Methods: Using the PubMed/MEDLINE and Web of Science/ISI Web of Knowledge databases with structured search syntax, we conducted a systematic review of the literature spanning a 40-year period (January 1, 1983, to October 10, 2023). Articles pertaining to ocular changes during flight or flight-equivalent exposure to altitude were retrieved. The reference lists of retrieved studies were also searched, and citations of these references were included in the results. Results: Of 875 relevant PubMed and ISI publications, 122 qualified for inclusion and 20 more were retrieved from the reference lists of initially selected records, for a total of 142 articles. Reported anterior segment changes included deterioration in tear film stability and increased dry eye incidence, increased corneal thickness, discomfort and bubble formation in contact lens users, refraction changes in individuals with prior refractive surgery, decreased intraocular pressure, and alterations in pupillary reaction, contrast sensitivity, and visual fields. Photoreceptor-visual pathway changes included alterations in both photoreceptors and neuro-transduction, as evidenced in dark adaptation, macular recovery time, reduction in visual field sensitivity, and optic neuritis (likely an element of decompression sickness). Retinochoroidal changes included increases in retinal vessel caliber, retinal blood flow, and choroidal thickness; central serous chorioretinopathy; and retinal vascular events (non-arteritic ischemic optic neuropathy, high-altitude retinopathy, and retinal vein occlusion). Conclusions: The effect of short-term high-altitude exposure on the eye is, in itself, a difficult area to study. Although serious impairment of visual acuity appears to be rare, ocular changes, including tear film stability, contact lens wear, central corneal thickness, intraocular pressure, contrast sensitivity, stability of refractive surgeries, retinal vessels, visual fields, and macula recovery time, should be considered in civilian aviators. Our report provides guidance to climbers and lowlanders traveling to altitude if they have pre-existing ocular conditions or if they experience visual symptoms while at altitude. However, key outcomes have been contradictory and comprehensive studies are scarce, especially those pertaining to the choroid and retina. Such studies could not only deepen our understanding of high-altitude ocular pathophysiology, but could also offer valuable information and treatment possibilities for a constellation of other vision-threatening diseases.

Correlation Between Coronary and Retinal Microangiopathy in Patients With STEMI.

Purpose: To investigate the morphological and functional correlation between microvascular retinal changes in optical coherence tomography angiography (OCTA) and the microvascular coronary circulation in patients with ST elevation myocardial infarction (STEMI) coronary heart disease (CHD). Methods: A total of 330 eyes from 165 participants (88 cases and 77 controls) were enrolled and imaged. Superficial capillary plexus (SCP) and deep capillary plexus (DCP) vascular density was measured in the central (1 mm) and perifoveal (1-3 mm) areas and in the superficial foveal avascular zone (FAZ) and choriocapillaris (3 mm). These parameters were then correlated to the left ventricular ejection fraction (LVEF), and the number of affected coronary arteries. Results: Decreased vessel densities in the SCP and DCP and choriocapillaris were positively correlated to the LVEF values (P = 0.006, P = 0.026, and P = 0.002, respectively). No statistically significant correlation between the SCP and DCP central area or FAZ area was found. Regarding the number of affected vessels, significant negative correlations were revealed for the SCP and DCP central vessel densities (P < 0.001 and P < 0.001, respectively) and the SCP perifoveal vascular density (P = 0.009). Conclusions: OCTA vascular indices are significantly correlated with morphological and functional parameters in patients with STEMI CHD. SCP vascular density especially seems to be a promising biomarker for the extent of both macrovascular damage (number of affected coronary arteries) and microvascular damage, as mirrored in the decreased LVEF at admission. Translational Relevance: OCTA vascular indices offer a valuable insight into the microvascular status of coronary circulation.

Impact of Cataract Surgery on IOP and Ocular Structures in Normotensive Patients and Primary and Exfoliation Open-Angle Glaucoma Patients.

Purpose: The aim of this study was to evaluate and compare the changes in Intraocular Pressure (IOP) and other ocular parameters: the Anterior Chamber Angle (ACA), Anterior Chamber Volume (ACV), and Anterior Chamber Depth (ACD) during phacoemulsification surgery in Greek patients with normotensive eyes and those with well-controlled Open-Angle Glaucoma (OAG). Additionally, parameters such as the Corneal Thickness (CCT), Axial Length (AL), Central Macular Thickness (CMT), and Retinal Nerve Fibre Layer (RNFL) were also examined. Patients and Methods: This was a prospective observational case-control study that included 50 phakic eyes, 25 normotensive (Group 1), and 25 with OAG: 15 Primary Open-Angle Glaucoma (POAG) and 10 Exfoliation Glaucoma (EXG) (Group 2). Ophthalmic assessment included IOP measurements, ocular biometry, and anterior and posterior segment optical coherence tomography evaluation of the aforementioned ocular parameters, prior and 6 months after phacoemulsification surgery. Results: At the 6 months post-operative review, a greater IOP reduction was recorded in eyes with OAG, in comparison to normotensive ones (5.3mmHg and 1.6 mmHg respectively). In addition, a significant but similar increase was recorded in the values of the ACA, ACV, and ACD of both groups between the pre- and the post-op period. Furthermore, the CCT and AL values remained unaltered. Finally, there was a non-statistically significant change in the mean CMT and the mean average RNFL of both groups. Conclusion: Eyes with OAG tend to undergo a greater reduction in IOP post-phacoemulsification surgery, in comparison to normotensive eyes. This reduction may not be solely attributed to ocular anatomical changes after phacoemulsification surgery but may also be due to the remodeling of the trabecular meshwork and the ciliary body. This may be especially true in the case of OAG eyes, which already start off with a compromised trabecular endothelium prior to surgery.

Periostin as a biomarker in chronic rhinosinusitis: A contemporary systematic review.

BACKGROUND: The role of periostin, a matricellular protein encoded by the POSTN gene, in chronic rhinosinusitis with nasal polyposis (CRSwNP) is reviewed. Periostin is considered a potential biomarker of endotype and may be useful for evaluating response to treatment. METHODS: Search terms in PubMed and Web of Science (1990-March 2022) included: ((periostin) OR (POSTN)) AND ((sinusitis) OR (nasal polyp) OR (CRSwNP) OR (CRS). The primary outcomes were differences in tissue, serum, and nasal lavage between CRSwNP and CRS without NP (CRSsNP) or controls. Associated factors reported to affect periostin expression, data regarding participants' clinical characteristics, disease endotypes, laboratory methods, and samples' origin were also pooled. Studies on <10 patients were excluded. RESULTS: Out of 101 records harvested through database searching, 29 prospective cross-sectional or case-control studies were eligible for review and qualitative analysis. Tissue sample origin, concurrent infection, current and past medication, primary or recurrent disease, allergic rhinitis, and smoking status should be considered as confounding factors for periostin levels. Periostin and POSTN messenger RNA (mRNA) levels were consistently and significantly higher in CRSwNP than CRSsNP and controls. Despite the distinctly different inflammation patterns among CRSwNP endotypes, periostin-related remodeling patterns seemed to be similar. CONCLUSION: Tissue and serum periostin levels, and POSTN expression appear elevated in CRSwNP, especially in eosinophilic inflammation, compared to CRSsNP and controls. Disease severity and comorbidities are also reflected in periostin and POSTN values. Carefully designed prospective studies may establish the role of periostin as a biomarker in CRSwNP and allow its incorporation in clinical practice.

Erdheim-Chester disease: a comprehensive review from the ophthalmologic perspective.

Erdheim-Chester disease (ECD) is a rare clonal histiocytic neoplasm with less than 1200 documented cases to date. The disease is life-threatening and difficult to recognize, although increasing awareness as well as the integration of clinical, imaging, pathology information, and genetic studies have led to a recent exponential increase in new reported cases. ECD affects multiple organs and systems, including skeletal, neurologic, and cardiovascular. Pulmonary, retroperitoneal, and cutaneous lesions have also been reported in various combinations. Until the discovery that more than half of ECD patients harbor the BRAF-V600E mutation or other mutations in the mitogen-activated protein kinase (MAPK) and RAS pathways, Interferon-a was the first-line treatment. Nowadays BRAF and MEK-inhibitors targeted therapies are the mainstay of treatment. Ophthalmologic involvement occurs in 25% -30% of ECD cases, usually in the form of orbital involvement presenting with exophthalmos and ophthalmoplegia. Other ophthalmologic manifestations include palpebral xanthelasmas, anterior uveitis and vitritis, optic disk edema, choroidal infiltration, recurrent serous retinal detachment, retinal drusen-like deposits and retinal pigment epithelial changes. ECD patients can also present with ocular symptoms as a result of adverse effects of the treatment regimens. In some cases with smoldering or protean symptoms, the emergence of eye manifestations triggered the diagnosis. Ophthalmologists have to be aware of the disease, recognize the constellation of ECD symptoms, and contribute to the diagnosis, treatment, and follow-up of ECD patients.

Scleral fixation of Carlevale intraocular lens: A new tool in correcting aphakia with no capsular support.

PURPOSE: To report the clinical outcomes of the use of a novel specially designed scleral fixated intraocular lens, the Carlevale intraocular lens (carlevale IOL, Soleko, Italy) for the correction of aphakia in the absence of capsular support of variable etiology. METHODS: This retrospective, non-comparative study included 169 eyes of 169 consecutive patients who underwent 3-port pars plana vitrectomy and scleral fixation on Carlevale IOL. Inclusion criteria were at least 6 months' follow-up period, patients > 18 years old who underwent vitrectomy and Carlevale IOL placement for aphakia and inadequate capsular support. RESULTS: The median follow up period of 9 months (range 6-18 months). Mean post-operative BCVA at the last follow-up visit was 20/25 (0.09 ± 0.1 LogMAR), improving from a mean baseline BCVA of 20/80 (0.58 ± 0.49 LogMAR), a statistically significant change (p = 0.0001). Regarding the post-operative complications, a transient rise in the IOP was observed in 28 patients (16.5%) and mild vitreous hemorrhage was observed in the immediate post-operative period in eight eyes (4.7%) and it spontaneously resolved within 3 weeks. All patients demonstrated good IOL position at the end of the follow-up without IOL capture. None of the patients required re-operation. CONLCUSION: The present study represents the largest to date in evaluating the use of carlevale IOL in patients with aphakia and inadequate capsular support. The technique is safe and provides excellent post-operative IOL fixation without IOL capture in any of the patients studied.

Choroidal thickness in patients with systemic arterial hypertension: a systematic review and meta-analysis.

Background: The global burden of hypertension is constantly increasing with adverse cardiovascular and ocular sequelae. The association between elevated blood pressure and choroidal thickness (CT), as assessed via optical coherence tomography (OCT), is poorly understood. Objectives and Design: Studies including hypertensive adults and normotensive controls undergoing OCT were evaluated for inclusion in this meta-analysis. The primary endpoint was CT difference between hypertensive and normotensive adults. Data Sources and Methods: We conducted a systematic review and after searching 1011 results from MEDLINE, ClinicalTrials.gov, medRxiv and Cochrane Library, six studies were deemed eligible and were pooled according to a random-effect model. Results: A statistically significant reduction in choroidal thickness was found in hypertensive adults (n = 454) as compared with normotensive controls (n = 365) [mean difference: -0.77; 95% confidence intervals: (-1.20, -0.34); p = 0.0004]. The main limitations of this meta-analysis are the relatively small population included and the high statistical heterogeneity (I 2 = 87%) among the various studies. Of note, after excluding one study the heterogeneity was markedly reduced. Conclusion: Choroidal thickness is reduced among hypertensive subjects compared with normotensive controls. This finding mandates further examination in the context of long-term clinical outcomes.

A fingerprint hidden inside the eye. A unique pattern of outer retina splitting as seen on en-face OCT and OCT-angiography.

A splitting of the outer plexiform retinal layer in a saw-like hyporeflective pattern in addition to partially formed concentric circles centred at the foveola were observed using en-face OCT and OCT-angiography in a 27-year-old female patient with rhegmatogenous retinal detachment and a 50-year-old female patient with Vogt-Koyanagi-Harada chorioretinopathy.