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Este trabalho tem por finalidade discutir os desafios do ensino superior no Brasil, especificamente relativo à formação em sala de aula, percorrendo as sérias problemáticas advindas com a fragilidade do processo formativo, bem como a reflexão acerca das mudanças necessárias para seu aperfeiçoamento.

PurposeTo compare outcomes of primary trabeculectomy using either mitomycin C (MMC) alone versus MMC augmented with intracamerular bevacizumab in patients with open-angle glaucoma.MethodsRetrospective, cohort, two-centre, comparative study. Patients’ data were screened between October 2015 and March 2019, with inclusion requiring a minimum follow-up of 24 months. Primary outcome was intraocular pressure (IOP) lowering at 24 months, with surgical success defined with different maximum IOP targets (≤18, ≤16 and ≤14 mm Hg) and at least 30% reduction and higher than 5 mm Hg. Absolute success was achieved if no IOP-lowering medication was needed and a qualified success if otherwise. Safety outcomes were analysed.ResultsA total of 110 eyes underwent trabeculectomy with MMC, 51 of these combined with intracamerular bevacizumab. Both strategies were effective in terms of IOP lowering (baseline vs 2 years postoperatively: 24.4 (8.0) mm Hg vs 12.1 (5.3) mm Hg in the MMC group; 25.1 (8.7) vs 10.8 (3.8) mm Hg in the MMC+bevacizumab group; pConclusionsIntracamerular bevacizumab in MMC-augmented primary trabeculectomy increases the chances of obtaining low IOP outcomes. This strategy may be useful when planning for surgeries aiming at target pressures in the low teens.Trial registration numberISRCTN93098069.

To examine the influence of the inverted flap (IF) internal limiting membrane (ILM) technique in macular hole (MH) closure on outer retinal layers after MH surgery.Retrospective study. Postoperative position of ILM, recovery rate of external limiting membrane and ellipsoid zone, and best-corrected visual acuity were evaluated. The Inserted group, where the IF is placed inside the hole, was compared with the Cover group, where the IF completely covers the hole.Sixty-two eyes of 58 patients who underwent vitrectomy and ILM peeling with the IF technique for large MHs (400 µm) with successful MH closure and a follow-up of 12 months were evaluated. In the 24 eyes of the Inserted group, there was no regeneration of external limiting membrane or ellipsoid zone after 12 months. In the 38 eyes of Cover group, external limiting membrane recovered in 55.3% of patients 1 month after surgery, and in 86.1% after 12 months. The elipsoid zone layer was present in 58% of the patients.Poorer anatomical and visual results were associated with the IF technique where ILM insertion occurs compared with ILM placed over the hole. These findings suggest that insertion of the ILM in the hole might prevent outer retinal layers realignment and visual recovery in MH surgery.

BACKGROUND: Ciclo plasty using high-intensity focused ultrasound (HIFU) technology acts through the selective coagulation of the ciliary body. Our aim was to evaluate the safety and efficacy profiles of 8-s probe HIFU cyclocoagulation using the EyeOP1 device. METHODS: Prospective pragmatic trial. Inclusion criteria: adult glaucoma patients with uncontrolled IOP despite optimised medical therapy, and/or intolerant to medical therapy required to achieve target IOP. Primary outcome: surgical success defined as IOP reduction from baseline >20% with final IOP ≤21 mmHg, without adding any IOP-lowering drugs, and without loss of light perception; or decreased use of IOP-lowering drugs with stable/decreased IOP, without loss of light perception. Secondary outcomes: mean IOP, intra and postoperative complications, best-corrected visual acuity (BCVA) and number of IOP-lowering drugs at each visit. Outcome data were collected preoperatively and at postoperative day 1, and months 1, 3, 6 and 12. RESULTS: Forty-nine eyes of forty-nine patients (28 male) with a mean age of 70 ± 14 years were enroled. Pre-operative IOP was 26.9 ± 7.4 mmHg under 2.8 ± 0.9 topical medications, decreasing to 17.8 ± 6.4 mmHg under 2.3 ± 1 drugs at 12 months (p

To develop the first Ophthalmology joint guidelines with Paediatric Rheumatology with recommendations on the screening, monitoring and medical treatment of juvenile idiopathic arthritis-associated uveitis (JIA-U), endorsed by the Portuguese Society of Ophthalmology (SPO).A systematic literature review was conducted to include publications up to July 14th 2020, with no language restrictions, in order to include all the international position papers/guidelines concerning the medical management of JIA-U and randomised clinical trials assessing the efficacy and safety of medical treatment in this field. We searched through MEDLINE (PubMed), Scopus, Web of Science and Cochrane Library. The Delphi modified technique to generate consensus was used. Preliminary evidence statements were subject to an anonymous agreement assessment and discussion process using an online survey, followed by further discussion and update at a national meeting. A draft of the manuscript with all recommendations was then circulated among all participants and suggestions were incorporated. The final version was again circulated before publication.Twenty-six recommendations were developed focusing on the following topics: general management (3), screening and follow-up of uveitis (4), treatment (17) and health education in JIA-U among patients and families (2).These guidelines were designed to support the shared medical management of patients with JIA-U and emphasize the need for a multidisciplinary approach between Ophthalmology and Paediatric Rheumatology regarding the comprehensive care of JIA-U. We acknowledge that updating these recommendations will be warranted in the future, as more evidence becomes available.juvenile idiopathic arthritis, uveitis, biological treatment, conventional immunosuppressive treatment, multidisciplinary management, guidelines, consensus, review, Delphi Technique.

BACKGROUND AND OBJECTIVE: Current literature lacks good-quality evidence regarding the outcomes of early pars plana vitrectomy (PPV) for acute exogenous endophthalmitis. The aim for this study was to analyze and discuss the outcomes of PPV for endophthalmitis in a UK tertiary hospital. PATIENTS AND METHODS: This study was a retrospective case series. The clinical records of consecutive patients who had PPV for acute exogenous endophthalmitis were reviewed. Demographic data, etiology, timing of onset, timing of PPV, intraand postoperative complications, baseline and final best-corrected visual acuity (BCVA), therapeutic regimens, and microbiology details were collected. Primary efficacy and safety outcome measures were BCVA improvement of two or more logMAR lines and intra- or postoperative complications, respectively. RESULTS: Forty-one patients with a median age of 76 (interquartile range, 64 to 82) years were studied. The most common etiology was intravitreal injection (41%), followed by phacoemulsification (34%). Median interval to PPV was 1.0 (interquartile range, 1.0 to 3.0) days. In a multivariate model controlling for age, baseline BCVA, microbiology positivity, and etiology (post-intravitreal injection), PPV after 24 hours was seven times more likely to achieve significant BCVA improvement (odds ratio, 7.47; 95% confidence interval, 1.12 to 49.66; P = .038). PPV within 24 hours of presentation was associated with more intraoperative complications. CONCLUSIONS: Favorable outcomes may be achieved with current antibiotic regimens and PPV for endophthalmitis. The series suggests that an early surgical intervention may be associated with poorer functional outcomes. Tap and inject at presentation, followed by a semi-urgent PPV as required, seems to be a sensible approach. [ Ophthalmic Surg Lasers Imaging Retina. 2022;53:96–102.]

To present real-world adalimumab (ADA) efficacy and safety outcomes in non-infectious uveitis (NIU) from a single United Kingdom (UK) tertiary centre.Retrospective review of adult patients with NIU treated with ADA. Data were collected at baseline, 6 and 12 months. The primary goal was to evaluate the rate of treatment failure, using the VISUAL-I/II studies criteria, during 12 months of follow-up. Secondary aims were to quantify corticosteroid-sparing effect; to identify safety issues; to assess change in visual acuity; to identify reasons for treatment failure and to compare treatment outcomes at 6 and 12 months for patients classified as active or inactive at baseline.Fifty-one patients (102 eyes) with mean age of 48.3 years were included. One third had a known underlying systemic disease and 47.6% had panuveitis. The most common indication for ADA was failure to respond to oral immunosuppression. Treatment failure occurred in 9/51 patients (10 eyes) after 6 months and 13/51 (20 eyes) patients after 12 months. The need for rescue treatment in the 12 months prior to ADA was significantly associated with treatment failure at 12 months. The mean prednisolone dosage was10 mg/day at 6 and 12 months. There were no serious adverse events.Patients with NIU who received ADA therapy for 12 months were likely to achieve disease control, to stabilize or improve visual acuity, to experience a reduction in immunosuppression and to reduce corticosteroid dosage. No new safety events were observed.

To describe two surgical techniques for rescuing a retained heavy silicone oil (HSO) bubble in the posterior pole: i) light pipe method and ii) perfluorocarbon liquid method.The surgical techniques are described in detail, along with an illustrative video for each.A short 25-gauge metallic cannula and active suction pump are used for standard HSO removal. Both surgical approaches are practical and effective options to complete the removal of HSO when a bubble sinks to the posterior pole.Both the light pipe and perfluorocarbon methods are suitable to remove retained HSO and thus avoid the need for further incisions and the risks associated with the use of larger gauge instrumentation.

Aerosolization during vitrectomy surgery was assessed by measuring fluorescein droplet spread in the surgical field and airborne particle counts. This study demonstrates that valved-port vitrectomy surgery under regional anesthesia is not likely to be an aerosol generating procedure.

© The Author(s), under exclusive licence to The Royal College of Ophthalmologists 2019, Aim Previous data suggest the existence of retinal vascular changes and impaired autoregulation in the very early stages of diabetic retinopathy (DR). We compared the retinal plexuses between patients with type 1 diabetes (T1D) without DR and a demographically similar healthy cohort, using optical coherence tomography angiography (OCT-A). Methods Patients with T1D and no signs of DR were prospectively recruited from an outpatient clinic. Using OCT-A (AngioVue®), the parafoveal superficial (SCP) and deep (DPC) capillary plexus as well as the foveal avascular zone (FAZ) and perimeter were gathered. Mean comparison tests and linear regression analysis were used as statistical tests (STATA v14). Results Studied population included 48 subjects (24 T1D). The analysis of SCP revealed an attenuation of the capillary network compared with the control group in both parafoveal (51.8 ± 4.5 vs. 55.8 ± 3.2, p < 0.001) and perifoveal (51.9 ± 3.3 vs. 53.9 ± 1.9, p = 0.01) regions. A similar finding was observed in the DCP for both parafoveal (56.4 ± 4.3 vs. 60.4 ± 2.2, p < 0.001) and perifoveal (54.7 ± 3.9 vs. 60.8 ± 3.4, p = 0.001) sectors. Also, a longer time since T1D diagnosis was associated with a larger FAZ area (p = 0.055) and perimeter (p = 0.03). Conclusions Significant differences in the retinal microvasculature were observed between healthy subjects and T1D patients using OCT-A, even before clinically detectable disease on fundus biomicroscopy., The authors would like to thank AstraZeneca Foundation/Faculty of Medicine of the University of Lisboa.

Purpose. Internal limiting membrane (ILM) peeling is important for macular hole (MH) surgery but may have secondary effects visible on spectral domain optical coherence tomography (OCT) and multifocal electroretinography (mfERG). We relate integrity of inner and outer macular layers with functional results with mfERG. Methods. Nonrandomized prospective study of 33 consecutive eyes of 33 patients with macular hole who underwent vitrectomy with ILM peeling. Best-corrected visual acuity was assessed, and integrity of external layers was measured using OCT. Each component of mfERG, N1 and P1 amplitude and latency, was also measured. Results. All eyes showed macular hole closure. Visual acuity improved from 20/400 to 20/40 in the Snellen visual acuity chart (P<0.001), and OCT external lines were intact in 19 eyes and disrupted in 14 eyes. Postoperatively, N1 and P1 amplitudes in ring 1 increased compared to preoperative values (P<0.001 for both). Latency remained delayed for both N1 and P1 wave. In the group of 19 eyes with integrity of outer retinal layers, N1 amplitude in ring 1 was superior to the group of 14 patients with disrupted outer retinal layers (P=0.042). Conclusions. In macular hole surgery, structure analysis in OCT is one of the important outcomes for the retinal surgeon. Functional results are parallel with anatomic results, but visual gain is limited. The limited recovery in mfERG suggests an alteration of retinal physiology that could explain limited vision recover.

Copyright © 2020 The Authors. This work is licensed under a Creative Commons Attribution-Non-Commercial-No-Derivatives 4.0 International License., Purpose: We hypothesize that patients with type 1 diabetes (T1D) may have abnormal retinal vascular responses before diabetic retinopathy (DR) is clinically evident. Optical coherence tomography angiography (OCTA) was used to dynamically assess the retinal microvasculature of diabetic patients with no clinically visible retinopathy. Methods: Controlled nonrandomized interventional study. The studied population included 48 eyes of 24 T1D patients and 24 demographically similar healthy volunteers. A commercial OCTA device (AngioVue) was used, and two tests were applied: (1) the hypoxia challenge test (HCT) and (2) the handgrip test to induce a vasodilatory or vasoconstrictive response, respectively. The HCT is a standardized test that creates a mild hypoxic environment equivalent to a flight cabin. The handgrip test (i.e., isometric exercise) induces a sympathetic autonomic response. Changes in the parafoveal superficial and deep capillary plexuses in both tests were compared in each group. Systemic cardiovascular responses were also comparatively evaluated. Results: In the control cohort, the vessel density of the median parafoveal superficial and deep plexuses increased during hypoxia (F1,23 = 15.69, P < 0.001 and F1,23 = 16.26, P < 0.001, respectively). In the T1D group, this physiological response was not observed in either the superficial or the deep retinal plexuses. Isometric exercise elicited a significant decrease in vessel density in both superficial and deep plexuses in the control group (F1,23 = 27.37, P < 0.0001 and F1,23 = 27.90, P < 0.0001, respectively). In the T1D group, this response was noted only in the deep plexus (F1,23 = 11.04, P < 0.01). Conclusions: Our work suggests there is an early impairment of the physiological retinal vascular response in patients with T1D without clinical diabetic retinopathy.

Aerosolization during vitrectomy surgery was assessed by measuring fluorescein droplet spread in the surgical field and airborne particle counts. This study demonstrates that valved-port vitrectomy surgery under regional anesthesia is not likely to be an aerosol generating procedure.

Purpose To characterize the progression in retinopathy severity of different phenotypes of mild nonproliferative diabetic retinopathy (NPDR) in patients with type 2 diabetes. Design and methods Patients with type 2 diabetes and mild NPDR (ETDRS 20 or 35) were followed in a 5-year longitudinal study. Examinations, including color fundus photography (CFP) and optical coherence tomography (OCT and OCTA), were performed at baseline, 6 months and then annually. Phenotype classification was performed based on microaneurysm turnover (MAT, on CFP) and central retinal thickness (CRT, on OCT). Phenotype A is characterized by low MAT ( Results Analysis of ETDRS grade step changes showed significant differences in diabetic retinopathy (DR) progression between the different phenotypes (p p p 1c (p = 0.019), LDL levels (p = 0.043), and ocular factors as MAT (p = 0.010), MA formation rate (p = 0.014) and MA disappearance rate (p = 0.005). Capillary closure at 5-year follow-up, identified by lower vessel density (VD) on OCTA, was also associated with diabetic DR severity progression (p = 0.035). Conclusions Different DR phenotypes in type 2 diabetes show different risks of retinopathy progression. Phenotype C is associated with increased HbA1c values and presents a higher risk of a 2-or-more-step worsening of the ETDRS severity score.

Diabetic retinopathy (DR) is a common complication of diabetes and may lead to blindness through vision-threatening complications, such as diabetic macular edema and proliferative DR (PDR). Several studies have established that certain systemic factors have associations with incidence and progression of DR, namely, glycemic control, arterial hypertension, high cholesterol and hyperlipidemia obesity, inflammatory markers, sleep-disordered breathing, and exercise (1,2). In addition to systemic factors, there are ocular factors that should be considered, since they may identify the eyes at risk (2). We here report a 5-year prospective longitudinal observational cohort study that investigates the risk of both systemic and ocular factors that may play a role in the development of diabetic macular edema and PDR, the vision-threatening complications of DR. This observational cohort study included eyes/patients with mild nonproliferative PDR, Early Treatment Diabetic Retinopathy Study (ETDRS) classification grades 20 and 35 (3), who were followed for a period of 5 years or until the time of development of center-involved macular edema (CIME), clinically significant macular edema (CSME), or PDR. A total of 212 patients were included: men and women with diagnosed adult-onset type 2 diabetes, aged 42–82 years, with a maximum baseline HbA1c value of 10% (86 mmol/mol). Exclusion criteria included any laser treatment or intravitreal injections or any other comorbidity that could affect the retina. …

With the current COVID-19 pandemic, our societies have witnessed an unprecedented generation and dissemination of information, including of scientific nature. With thousands of new studies published every day, it is a challenge for physicians to keep up with the evidence relevant for their practice. Such times call for physicians’ ability to critically appraise the medical literature for its soundness and relevance. In addition, it is of the uttermost importance that research published in extremely short periods of time maintains high-quality standards and is useful for physicians all around the globe. The solution for this pandemic crisis depends on research and development and our doctors have been trained to diagnose and treat. They lack research training—essential to build, interpret and effectively apply scientific evidence. Therefore, it is time to acknowledge the critical importance of research training as an early component of medical education, which, as many studies have shown, carries many advantages. Research training skills allow critical assessment of medical literature, promote relevant clinical questions and the framing of its findings in a clinically meaningful way, ultimately resulting in improved knowledge translation into clinical practice.1 Doctors with appropriate research training can play a pivotal role in designing clinical trials and promoting patient-centred research.2 They are also more focused on teaching medical students, further contributing to the expansion of medical knowledge. The whole …

The XEN gel stent is a minimally invasive surgical device aimed at creating a subconjunctival drainage of aqueous humor, thereby reducing intraocular pressure (IOP). Being a novel device, doubts remain with regard to the efficacy and safety of its implantation in the early stage of new users. This paper illustrates the XEN implantation learning curve, assessed through several surgeons of different expertise.This was a retrospective study on the first 6 XEN implants performed by each of the 10 certified ophthalmic surgeons. Simultaneous cataract surgery was allowed (phaco-XEN). Primary outcomes were as follows: surgical time; intraoperative and postoperative surgical complications. Secondary outcomes were as follows: IOP; the number of topical drugs in use; the need for needling procedures. Outcome data were collected preoperatively and at postoperative days 1, 7, 15, 30, 60, and 90. Statistical analysis was performed with STATA 14.1 and SPSS.Sixty patients were included (56.7% female patients). Mean age was 73 years (45 to 89). Mean preoperative IOP was 23.8±8.95 mm Hg. From the included patients, 29 (48.3%) were submitted to simple XEN implant and 31 (51.7%) to phaco-XEN. In both groups, mean surgical time decreased by 9 minutes throughout the 6-implant learning curve. Final IOP was 15±7.27 mm Hg in the stent group (-43% than baseline), and 14.92±3.32 mm Hg in the phaco-XEN group (-16% than baseline). On average, patients decreased 2 topical IOP-lowering drugs. Needling procedures were performed in 17 patients (28.3%).XEN gel stent was associated with a fast learning curve, by both experienced surgeons and novice residents. By the sixth implant, both groups had considerably decreased mean surgical time and complication rates.

Purpose: The aim of this study is to assess the agreement between manual and automated gonioscopy for iridocorneal angle opening. Methods: The research is a cross-sectional observational study. Manual and automated gonioscopy were performed to consecutive patients in a glaucoma clinic. Iridocorneal angle opening grading was performed according to Shaffer’s classification. Automated gonioscopy was performed with NGS-1 automated gonioscope (NIDEK Co., Gamagori, Japan). The automated gonio-photos were graded by two independent observers. Agreement between automated and manual gonioscopy and also among raters was ascertained by Fleiss’ kappa statistic and comparison of area under curve. Results: In total, 88 eyes of 47 subjects were analysed. Mean age was 63 ± 10 years. Twenty eyes (22.7%) were excluded from grading due to poor quality images. Angle closure was detected in 23.4% with dynamic gonioscopy in comparison with 4.3% using automated image grading. The agreement for angle closure diagnosis between dynamic and automated gonioscopy was low (κ = 0.09 ± 0.10; p = 0.18). The area under curve for detecting eyes with angle closure showed poor accuracy between automated and manual methods (area under curve: 0.53 ± 0.05, 95% confidence interval: 0.44–0.62). There was modest inter-rater agreement for angle opening assessment of automated images with Fleiss’ kappa of 0.17 (95% confidence interval: 0.035–0.238). Conclusion: Manual and automated gonioscopy showed only slight agreement for the assessment of iridocorneal angle opening status. Further improvements of the NGS-1 automated gonioscopy and technique are desired for widespread use in a real-life setting.

Non-infectious uveitis is a heterogeneous collection of inflammatory eye diseases and is one of the most important causes of blindness among active adults in developed countries. Inflammation control is crucial in the prevention of ocular structural and functional damage. Regarding acute inflammatory control, corticosteroids are the mainstay of treatment. Several types of intra-ocular corticosteroids have been used with the aim of enhanced efficacy compared to their topical or peri-ocular administration, while minimizing its adverse effects associated with the systemic administration. The purpose of this Cochrane review was to synthetize the available evidence regarding the efficacy and safety of corticosteroid implants in comparison with standard treatment. Due to the heterogeneity in the design of the studies and outcome measures assessed, authors could not conclude that implants are superior to traditional systemic therapy in Non-infectious uveitis. The safety analysis suggested increased risks of post-implant surgery for cataract and high intraocular pressure compared with standard-of-care therapy.A uveíte não infecciosa inclui várias patologias com elevada morbilidade, sendo uma das causas mais importantes de cegueira na população activa de países desenvolvidos. O controlo da inflamação é essencial na prevenção do dano estrutural e funcional às estruturas oculares. Sendo os corticóides fármacos de primeira linha no controlo agudo da inflamação, têm sido usadas formulações intra-oculares destes fármacos, com o objectivo de aumentar a eficácia face ao uso tópico ou periocular e de induzir menos efeitos adversos secundários comparativamente ao uso sistémico. O objectivo desta revisão Cochrane foi sintetizar a evidência existente sobre a eficácia e segurança de implantes de corticóides intravítreos, em comparação com a terapêutica convencional. Devido à heterogeneidade no desenho e medidas de eficácia utilizadas, os autores não conseguiram demonstrar superioridade dos implantes intravítreos de corticóides em comparação com a terapêutica convencional, na uveíte não infecciosa. A análise de segurança sugere que estes possam aumentar a probabilidade de necessidade de cirurgia de catarata e de elevação da pressão intraocular, em comparação com a terapêutica standard.

© 2018 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd., Anti-tumour necrosis factor (TNF) drugs have been extensively used in non-infectious uveitis (NIU), when corticosteroids or conventional immunosuppressive drugs cannot adequately control inflammation or intolerable side-effects occur. However, systemic anti-TNF therapies are also associated with a myriad of side-effects. Therefore, intravitreal administration of anti-TNF biologics has been employed to minimize patient morbidity and systemic adverse effects, while maintaining therapeutic effectivity. We undertook a systematic review to determine evidence of efficacy and safety of intravitreal administration of anti-TNF drugs in adults with NIU. We conducted this systematic review according to the PRISMA guidelines. The protocol was registered with PROSPERO (CRD42016041946). We searched CENTRAL, MEDLINE and EMBASE, from inception to April 2017, as well as clinical trial registries and grey literature. The qualitative analysis included all studies of adult patients with a diagnosis of NIU and who received intravitreal anti-TNF drugs with a 4-week minimum follow-up. A total of 4840 references were considered for title and abstract screening. Seven full texts were screened, and five studies were considered for analysis. All studies were open-label, single-centre, prospective, non-randomized, interventional case series with a follow-up between 4 and 26 weeks, employing either adalimumab in two studies and infliximab in three. Three studies showed a treatment effect of anti-TNF intravitreal injections, while one study revealed short-term improvement and one study revealed no efficacy of anti-TNF intravitreal therapy. None of the studies reported ocular adverse effects but only two studies included electrophysiological assessment in the safety analysis and no study assessed systemic human anti-drug antibodies. The available evidence is not sufficiently robust to conclude about the clinical effectivity of intravitreal anti-TNF in NIU and so no recommendation can be made. In conclusion, intravitreal injection of anti-TNF antibodies remains a possible treatment option to be explored through robust clinical investigation.

Background: Cystinosis is a multisystemic autosomal recessive deficiency of the lysosomal membrane transporter protein (cystinosin) caused by mutations in CTNS gene. Objective: This study summarizes the Portuguese experience in the diagnosis and management of patients with this rare disease over the past few years and reports recurrent mutations in the CTNS gene. Methods: Unrelated patients from different pediatric and adult hospitals all over Portugal with non-nephrotic proteinuria, hypercalciuria, hypokalemia impaired proximal reabsorption of amino acids, glycosuria and hypophosphatemia, suggestive of a Fanconi syndrome and ocular problems, were studied. Intra-leukocyte cystine levels were determined and molecular analysis was performed, to determine the presence or absence of the 57-kb deletion in CTNS, followed by direct sequencing of the coding exons of CTNS. Results: From 1998 to 2017, twenty-one cystinotic patients were biochemically diagnosed. From the remaining seventeen (four deceased), eleven were studied for CTNS gene. Five out of eleven patients were homozygous for the 57-kb deletion (10/22; 45.5%), and other five were compound heterozygous for this variant (15/22; 68.2%). The other mutations found were p.Q128X (c.721 C>T; 2/22), p.S139F (c.755 C>T; 4/22) and c.18-21delGACT (p.T7FfsX7; 1/22). All of these seventeen cystinotic patients are in treatment. Approximately 84% are adults, 16% are young children, and 54.5% are kidney transplant recipient. Conclusions: The authors would like to emphasize the importance of first screening for the 57-kb deletion since it is very common in our population. This genetic study is the first in our country and it could be the basis for future genetic counseling in Portuguese population.

Background: The purpose of this prospective study was to evaluate the efficacy and safety of the Ultrasound Cyclo Plasty (UCP) procedure using high-intensity focused ultrasound in surgery-naïve open-angle glaucoma patients. Methods: prospective, non-randomized, single-arm, multicenter clinical trial. Sixty-six eyes with primary open-angle glaucoma, intraocular pressure (IOP) ≥21 mmHg and with no history of filtering surgery were enrolled. Patients were treated by UCP with a therapy probe comprising six piezoelectric transducers, consecutively activated for 8 s each. Complete ophthalmic examination was performed before the procedure, 1 day after the procedure, and 1, 3, 6, 12, 18 and 24 months after the procedure. Primary outcomes were complete success (defined as IOP lowering from baseline ≥20% without additional glaucoma medications) and vision-threatening complications. Secondary outcomes were the presence of complications and the reduction of the number of medications used. Results: IOP was significantly reduced after one procedure (p &lt, 0.05), from a mean pre-operative value of 24.3 ± 2.9 mmHg (n = 2.3 hypotensive medications) to a mean value of 15.9 ± 3.6 mmHg (n = 2.2 hypotensive medications) at 2 years (mean IOP lowering of 33%). Surgical success was achieved in 74% of eyes. Notwithstanding side effects such as transient anterior chamber inflammation, refractive error changes, transient hypotony and macular edema, no major intra or post-operative complications such as phthisis, induced cataract, neovascularization or significant vision loss were observed. Conclusions: Ultrasound Cyclo Plasty is a valuable, effective and well-tolerated procedure to lower IOP in patients with open-angle glaucoma without previous filtering surgery.

Purpose: To provide a spectral-domain optical coherence tomography (SD-OCT)-based analysis of retinal layers thickness and nasal displacement of closed macular hole after internal limiting membrane peeling in macular hole surgery. Methods: In this nonrandomized prospective interventional study, 36 eyes of 32 patients were subjected to pars plana vitrectomy and 3.5 mm diameter internal limiting membrane (ILM) peeling for idiopathic macular hole (IMH). Nasal and temporal internal retinal layer thickness were assessed with SD-OCT. Each scan included optic disc border so that distance between optic disc border and fovea were measured. Results: Thirty-six eyes had a successful surgery with macular hole closure. Total nasal retinal thickening (pConclusions: Internal limiting membrane peel is associated with important alteration in inner retinal layer architecture, with thickening of mIRL and shortening of papillofoveal distance. These factors may contribute to recovery of disrupted foveal photoreceptor and vision improvement after IMH closure.

Purpose: To highlight tomographic structural changes of retinal layers after internal limiting membrane (ILM) peeling in macular hole surgery. Methods: Nonrandomized prospective, interventional study in 38 eyes (34 patients) subjected to pars plana vitrectomy and ILM peeling for idiopathic macular hole. Retinal layers were assessed in nasal and temporal regions before and 6 months after surgery using spectral domain optical coherence tomography. Results: Total retinal thickness increased in the nasal region and decreased in the temporal region. The retinal nerve fiber layer (RNFL), ganglion cell layer (GCL), and inner plexiform layer (IPL) showed thinning on both nasal and temporal sides of the fovea. The thickness of the outer plexiform layer (OPL) increased. The outer nuclear layer (ONL) and outer retinal layers (ORL) increased in thickness after surgery in both nasal and temporal regions. Conclusion: ILM peeling is associated with important alterations in the inner retinal layer architecture, with thinning of the RNFL-GCL-IPL complex and thickening of OPL, ONL, and ORL. These structural alterations can help explain functional outcome and could give indications regarding the extent of ILM peeling, even though peeling seems important for higher rate of hole closure.

Copyright © 2019 Leal, Rodrigues, Sousa, Duarte, Romão, Marques-Neves, Costa and Fonseca. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms., Background: We aimed to assess efficacy and safety of anti-tumor necrosis factor (TNF) drugs for adult chronic non-infectious uveitis (NIU). Methods: CENTRAL, MEDLINE, and EMBASE, were searched from inception to January 2019. Double-masked randomized placebo-controlled trials, assessing any anti-TNF vs. best medical intervention/standard of care in adults with chronic NIU were considered. The PRISMA and SAMPL guidelines were followed. The risk of bias was assessed using the Cochrane risk of bias tool. Overall quality of the evidence was assessed according to GRADE. PROSPERO registration: #CRD42016039068. The primary efficacy and safety outcomes were preservation of visual acuity (VA) and withdrawals due to adverse events, respectively. Meta-analysis of efficacy analysis was not performed due to significant clinical heterogeneity between studies' population and interventions. Results: A total of 1,157 references were considered and 3 studies were included. The overall risk of bias was moderate. In active NIU, adalimumab group showed an increased likelihood of VA preservation (risk ratio (RR) 1.75, 95%CI 1.32 to 2.32, n = 217), whereas the etanercept group did not (RR 0.81, 95%CI 0.57 to 1.14, n = 20). In inactive NIU, adalimumab was associated with increased likelihood of VA preservation (RR 1.31, 95%CI 1.12 to 1.53, n = 226). The rate of adverse events did not differ between anti-TNF and control arms (RR 1.03, 95%CI 0.94 to 1.13, n = 410). Conclusions: There is high quality evidence that adalimumab decreases the risk of worsening VA in active and inactive NIU and very low quality evidence that the risk of etanercept worsening VA in inactive NIU is not different from placebo. Moderate quality evidence suggests that anti-TNF agents are not different from placebo on the risk of study withdrawal., This project received a research grant from a non-profit and scientific organization, Portuguese Society of Ophthalmology (Sociedade Portuguesa de Oftalmologia). UID/BIM/50005/2019, project funded by Fundação para a Ciência e a Tecnologia (FCT)/Ministério da Ciência, Tecnologia e Ensino Superior (MCTES) through Fundos do Orçamento de Estado (Portuguese State Funding).

Background: Retinoblastoma (RB) is the most common primary intraocular malignancy. Current therapies are associated with high morbidity in the short- and long-term. Human epidermal growth factor receptor 2 (HER2) is a transmembrane protein detected in 15-30% of breast cancers, but it has also been described in other malignancies. Recently, it has been claimed that a truncated version of this protein is expressed in RB, responsive to directed therapies in vitro. We scored HER2 overexpression in RB tissue samples and discussed its potential clinical utility. Methods: HER2 overexpression was investigated using immunohistochemistry; the overexpression was evaluated with a score ranging from 0 to 3+ according to the membranous staining pattern in archival formalin-fixed, paraffin-embedded RBs. Results: A total of 60 RB cases and a RB cell line (Y79) were considered. The mean age at enucleation was 31.6 ± 31.5 months. The mean time from diagnosis to enucleation was 11.8 ± 11.2 months (range 1-44). Five (8%) cases were multifocal. HER2 overexpression was negative in all RB cases (49 cases scored 0 and 11 scored 1+) and in the Y79 cell line. Conclusions: Overall, we were not able to demonstrate the overexpression of HER2. Further studies should clarify and better elucidate the potential role of HER2-targeted therapies in RB.

Purpose: To determine intra- and inter-rater agreement of anterior lamina cribrosa depth (ALCD) manual measurements using enhanced-depth imaging optical coherence tomography (EDI-OCT). Methods: Observational study. EDI-OCT of the optic nerve head was performed in healthy subjects. ALCD was defined as the perpendicular distance between the line connecting both edges of Bruch's membrane opening and the anterior border of the lamina cribrosa (LC), at the maximum depth point. Two experienced operators performed manual ALCD measurements of the LC independently, and one of the observers performed the same measurements twice. Intra- and inter-rater agreement was evaluated using intraclass correlation coefficients (ICC) and concordance correlation coefficients (CCC) for the right eye vertical (REV) and horizontal (REH) scans and left eye vertical (LEV) and horizontal (LEH) scans. Results: 120 eyes of 61 subjects, with a mean age of 62.1 ± 15.0 years. The mean REV, REH, LEV and LEH ALCD were 456.2 ± 84.3, 444.5 ± 92.2, 436.7 ± 81.6, 427.6 ± 82.7 μm, respectively. ALCD intra- and inter-rater ICC and intra- and inter-rater CCC varied between 0.85-0.95, 0.84-0.93, 0.85-0.95 and 0.84-0.93, respectively. Conclusions: ALCD manual intra- and inter-rater measurements with EDI-OCT showed high agreement. EDI-OCT is a reliable tool for ALCD measurement, which can provide potentially useful information for integrated glaucoma management.

There are currently limited data addressing the surgical outcomes of pars plana vitrectomy (PPV) in toxoplasmosis-related macular hole (tMH). We aim to report and discuss safety and efficacy of PPV for tMH.Surgical case series (n = 11), with minimum postoperative follow-up time of 6 months. Consecutive patients who underwent PPV for tMH from 2013 to 2016 were included. Indications for surgery were: visual acuity ≥ 0.6 logarithm of the minimum angle of resolution (Snellen 20/80 or less), no intraocular inflammation for more than 6 months, extrafoveal toxoplasmosis scar, elevated tMH borders on optical coherence tomography, and patient agreement with surgery. Surgery was performed-PPV with epiretinal (if present) and internal limiting membrane peeling. Safety and efficacy of PPV for tMH were addressed by evaluating: 1) surgery-related complications and 2) visual acuity improvement.A total of 11 patients (6 male), with a mean age of 33.2 ± 11.0 years were studied. Mean preoperative best-corrected visual acuity significantly improved from 1.10 ± 0.24 (Snellen 20/252) to 0.43 ± 0.18 logarithm of the minimum angle of resolution (Snellen 20/54) at last follow-up visit (P0.01). The rate of visual acuity improvement (i.e., a gain of at least three lines) and tMH closure was 100% for both. The only reported surgery-related complication was cataract in one case.Our results suggest that PPV is a safe and effective option in tMH cases. A controlled, longitudinal study would contribute to confirm these findings.

© The Author(s) 2018. Reprints and permissions: sagepub.co.uk/journalsPermissions.nav, Purpose: To compare the safety and efficacy profiles of Descemet membrane endothelial keratoplasty (DMEK) and Descemet stripping automated endothelial keratoplasty (DSAEK) in adult patients with Fuchs’ endothelial dystrophy. Methods: Electronic database search on MEDLINE and CENTRAL from inception to August 2017. We included all comparative studies of DMEK versus DSAEK in patients with diagnosed Fuchs’ endothelial dystrophy. Studies assessing rescue procedures were excluded to minimize bias. Primary outcome: mean difference in best-corrected visual acuity (BCVA) at 3, 6, and 12months postoperatively. Secondary outcomes: rates of graft primary failure, rejection, and rebubbling; other graft-related issues; mean difference in endothelial cell density; subjective visual outcomes; and patient satisfaction. Results: A total of 10 retrospective studies of moderate methodological quality were included (n=947 eyes, 646 DMEK). BCVA was better with DMEK at all evaluated time points (0.16 logMAR at 12months) comparing to DSAEK (0.30 logMAR; p

Copyright © Ordem dos Médicos 2019, Em outubro de 2018 foi apresentado o conceito e modo de implementação do programa Choosing Wisely Portugal — Escolhas Criteriosas em Saúde. Este programa tem como objetivo promover escolhas em Saúde baseadas na melhor evidência cientifica, reduzindo o número de intervenções desnecessárias, sem eficácia comprovada e/ou com uma relação risco-benefício desfavorável.1,2 A colaboração dos médicos é essencial para combater a sobreprescrição de exames e procedimentos, contribuindo para a segurança do doente e a sustentabilidade do Sistema Nacional de Saúde.

Aims: To report a case of a patient who developed uveitis-glaucoma-hyphema (UGH) syndrome after an uneventful cataract surgery and to discuss risk factors, diagnostic challenges, management options, and clinical implications. Background: Uveitis-glaucoma-hyphema syndrome is a rare but potentially serious cataract surgery complication. Clinical manifestations include increased intraocular pressure (IOP), anterior chamber inflammation, and recurrent hyphema or microhyphema. Uveitis-glaucoma-hyphema Plus syndrome also includes accompanying vitreous hemorrhage. Although classically associated with rigid anterior chamber intraocular lenses (lOLs), cases of malpositioning and subluxated posterior chamber lOLs have also been described as possible triggers. Case description: We report a case of a 70-year-old Caucasian man who developed UGH Plus syndrome after an uneventful cataract surgery with an lOL implanted in the capsular bag. During postoperative follow-up, persistent intraocular inflammation, increased IOP, hyphema, and vitreous hemorrhage were consistent with this diagnosis. Slit-lamp examination demonstrated progressive localized iris atrophy, compatible with chafing of the posterior iris by the IOL haptic as the trigger for UGH syndrome. A pars plana vitrectomy was performed and a retropupillary intraocular lens was implanted. No further complications occurred during follow-up. Conclusion and clinical significance: Given the increasing prevalence of single-piece lOLs implanted in the capsular bag, it is important to recognize UGH syndrome as a rare but potentially serious complication. How to cite this article: Sousa DC, Leal I, Faria MY, Pinto LA. A Rare Manifestation of Uveitis-glaucoma-hyphema Syndrome. J Curr Glaucoma Pract 2016;10(2):76-78.

To compare intracameral 20% sulfur hexafluoride (SF6) versus 100% air as tamponade for graft attachment in Descemet membrane endothelial keratoplasty (DMEK).Using an electronic database search on MEDLINE and CENTRAL from inception to December 2017, we performed a literature review and meta-analysis including all comparative studies of SF6 at a 20% concentration (20% SF6) versus pure air (100% air) for anterior chamber tamponade in DMEK. The primary outcome was the rebubbling rate at the final observation. The secondary outcomes were 1) the graft detachment rate, 2) mean difference (MD) in best-corrected visual acuity (BCVA), 3) manifest refraction spherical equivalent, 4) central corneal thickness (CCT), 5) percentage of endothelial cell loss (ECL), and 6) rate of pupillary block by the final observation. Statistical analysis was performed using RevMan5.3 software.Five retrospective studies were included, assessing 1195 eyes (SF6 277; air 918). The main indication for surgery was Fuchs endothelial dystrophy (SF6 85.2%; air 86.2%) and bullous keratopathy (SF6 10.8%; air 10.0%). Overall, studies were of moderate to good methodological quality. Patients in the SF6 group required 58% less rebubbling procedures (risk ratio 0.42, 95% confidence interval (CI), 0.31-0.56, P0.0001). No differences were found regarding BCVA improvement (MD 0.03, 95% CI, -0.05 to 0.11, P = 0.49). SF6 was associated with a minor hyperopic shift (MD 0.37 D, 95% CI, -0.95 to -0.21, P = 0.21). No differences were found regarding CCT, ECL, and rate of pupillary block (P0.05).In DMEK, 20% SF6 tamponade and longer postoperative time supine were associated with 58% fewer rebubbling procedures, and an ECL not statistically different from using 100% air.

Fibromyalgia can be clinically defined by widespread pain lasting for longer than 3 months with tenderness on palpation in 11 or more of 18 specified tender points. Many people with fibromyalgia are significantly disabled, and experience moderate to severe pain for many years, for which conventional analgesics are usually not effective. For these patients treatment options generally include antidepressants like tricyclic agents, serotonin and noradrenaline reuptake inhibitors, or anticonvulsants like pregabalin or gabapentin. Pregabalin is a drug licensed for the treatment of fibromyalgia in the United States of America, with a mechanism of action similar to gabapentin. This mode of action confers antiepileptic, analgesic, and anxiolytic effects. This Cochrane systematic review included 8 randomized, placebo-controlled trials with low risk of bias, which studied the effect of a daily dose of pregabalin for the treatment of moderate to severe pain in adult patients suffering from fibromyalgia. Of the main results of this systematic review we highlight the major effect that a daily dose of 300 to 600 mg of pregabalin had in the reduction of pain intensity over a follow-up period of 12 to 26 weeks, with tolerable adverse effects, for a minority of people with moderate to severe pain due to fibromyalgia. This paper aims to summarize and discuss the main results and conclusions of this systematic review, as well as its implications for the daily clinical practice.A fibromialgia é uma doença caracterizada por dor generalizada, com mais de três meses de duração, com dor à palpação em pelo menos 11 de 18 pontos anatomicamente definidos. Um elevado número de doentes com fibromialgia sofre de dor moderada a grave durante vários anos, para a qual os analgésicos convencionais se mostraram ineficazes, de tal forma que foram substituídos por antidepressivos tricíclicos, inibidores seletivos da recaptação da serotonina e noradrenalina e anticonvulsivantes, como a pregabalina e a gabapentina. A pregabalina é um fármaco anti-epilético aprovado para o tratamento da fibromialgia nos Estados Unidos da América, cujo mecanismo de ação se assemelha ao da gabapentina, com efeitos anticonvulsivante, analgésico e ansiolítico. Nesta revisão sistemática Cochrane os autores incluíram um total de oito ensaios clínicos aleatorizados, controlados por placebo, com baixo risco global de viés, que estudaram o efeito da administração de uma dose diária de pregabalina no tratamento da dor crónica moderada a grave, em doentes adultos com fibromialgia. Dos principais resultados desta revisão sistemática destacam-se o efeito substancial que uma dose diária de 300 a 600 mg de pregabalina teve na diminuição da intensidade da dor moderada a grave, numa minoria dos doentes adultos com fibromialgia, durante um período de follow-up de 12 a 26 semanas, bem como a elevada incidência de eventos adversos associados ao tratamento, a maioria dos quais bem tolerados. Neste artigo sumarizam-se e discutem-se os principais resultados e conclusões desta revisão sistemática, bem como as suas implicações para a prática clínica diária.

Purpose High-intensity focused ultrasound (HIFU) technology aims to achieve selective coagulation of the cilliary body, thus reducing aqueous humor production and lowering intraocular pressure (IOP). This study evaluated safety and efficacy of HIFU cyclo-coagulation using EyeOP-1® device in 44 glaucoma patients. Methods Glaucoma patients with uncontrolled IOP despite optimal medication were scheduled for HIFU treatment and followed for 12 months. The primary efficacy and safety outcomes were IOP reduction and major adverse events, respectively. Statistical analyses were performed using STATA v14.1. Results Fourty-four patients (23 male) with a mean age of 70 ± 15 years were studied. Mean preoperative IOP reduced from 26.6 ± 3.8 mmHg to 17.7 ± 4.3 mm Hg at month 6 and 17.4 ± 3.3 mm Hg after 12 months (P

Purpose Glaucoma is the main cause of irreversible blindness worldwide. Early detection and personalized management is the key to prevent or slow disease progression. Our group previously evaluated the quality of within-hospital referral to a glaucoma subspecialty department (GSD), raising awareness to the importance of doing applanation tonometry (AT), gonioscopy and visual fields (VF) before referral. The aim of this work was to perform an audit cycle, one year after the awareness training session in December 2015. Methods Retrospective analysis of a random sample of within-hospital referrals to the GSD, in Lisbon Academic Medical Center (January 2016 to May 2017). Electronic records were screened for clinical data, exams, medical and surgical treatments prior to referral. Descriptive statistics were performed with SPSS. Results A total of 150 patients (69 males) were included in this analysis, with a mean age of 65 years [range 10–93]. Mean intraocular pressure (IOP) was 19.8 mmHg. Almost 20% of patients were not on any IOP-lowering drugs, while nearly half were under ≥2 classes of treatment. AT and VF were performed respectively in 43% and 59% of the referred patients, both versus 49% in 2015 (p > 0.05). Gonioscopy was described for 23% of patients, more than the 12% mentioned in 2015 (p = 0.014). Optical coherence tomography (OCT) was performed in the large majority, 85%, as in the first analysis. Only 6% of patients had all AT, gonioscopy and VF; and 19% were referred with none of these exams, comparing to the 22% in the original 2015 screening (p > 0.05). Conclusions Overall, training session did not significantly improve the quality of GSD referral, though more physicians reported gonioscopy results. It seems many clinicians still prefer more expensive and sophisticated exams as OCT, often forgetting valuable and simpler tools.

PURPOSE Previous studies report that the response of retinal vessels to a decrease in oxygen (hypoxia) is vasodilation, thus increasing blood flow. We aimed to characterize the changes in retinal microvasculature induced by a mild hypoxia stress test in a healthy population, using ocular coherence tomography angiography (OCT-A) technology. METHODS Interventional repeated-measures study. The standardized hypoxia challenge test (HCT) was performed to all volunteers, according to the British Thoracic Society protocol. Ocular coherence tomography angiography (OCT-A) was performed at three time-points (baseline, during HCT and 30' posthypoxia). Macular and peripapillary vessel densities were assessed using the built-in software. To minimize bias, analysis was performed separately in right (OD) and left (OS) eyes. Repeated-measures anova and mean comparison analysis were used as statistical tests (stata v13). RESULTS Studied population included 30 healthy subjects (14 women), with a mean age of 28.8 ± 4.2 [range 22-37] years. Baseline vessel density increased in hypoxic conditions and subsequently decreased to near-baseline values in posthypoxia conditions. This pattern was observed for both eyes in both parafovea (OD: 55.3 ± 2.3 to 56.7 ± 1.9 to 55.8 ± 1.9, p