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María Moreno-López, Julio José González-López, Elena Jarrín, Josefina BertrandOphthalmology Department, Hospital Universitario Ramón y Cajal, Madrid, SpainAbstract: We describe a case of maculopathy consisting of macular retinoschisis and serous macular detachment occurring in a patient with an acquired enlarged optic disc cup, similar to the maculopathy observed in congenital optic nerve abnormalities, mainly optic nerve pits and colobomas, without vitreomacular traction nor angiographic leak. Pars plana vitrectomy with argon laser endophotocoagulation and gas tamponade was found to be useful. Traction from membranes covering deep optic disc cups may create small retinal dehiscences, as described in congenital optic nerve abnormalities, which will enable the liquefied vitreous to pass, leading to retinoschisis with or without associated neurosensory detachment. Vitrectomy, photocoagulation, and gas tamponade may be a useful therapy for this entity.Keywords: glaucoma, macular detachment, macular surgery, optic disc, retinoschisis

Gyrate atrophy of the choroid and retina (GACR) is a rare autosomal recessive disease characterised by elevated plasma ornithine levels due to deficiency of the enzyme ornithine aminotransferase (OAT). The accumulation of this amino acid in plasma leads to the development of patches of chorioretinal atrophy in the peripheral retina extending into the macular area. Patients usually present with night blindness followed by constriction of the visual field and, finally, decreased central vision and blindness. The disease is diagnosed by the presence of the characteristic clinical picture, the presence of hyperornithinaemia in plasma and the detection of mutations in the OAT enzyme gene. There is currently no effective gene therapy and the most common therapeutic intervention mainly involves dietary modifications with arginine restriction. This article aims to summarise the pathogenesis, clinical and diagnostic findings and treatment options in patients with GACR., (Copyright © 2024 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.)

Purpose: To assess factors that impact the risk of relapse in patients with noninfectious uveitis (NIU) who undergo adalimumab tapering after achieving remission., Design: Retrospective study., Methods: In this multicenter study, patients with NIU were treated with adalimumab and subsequently tapered. Patient demographics, type of NIU, onset and duration of disease, the period of inactivity before tapering adalimumab, and the tapering schedule were collected. The primary outcome measures were independent predictors of the rate of uveitis recurrence after adalimumab tapering., Results: Three hundred twenty-eight patients were included (54.6% female) with a mean age of 34.3 years. The mean time between disease onset and initiation of adalimumab therapy was 35.2 ± 70.1 weeks. Adalimumab tapering was commenced after a mean of 100.8 ± 69.7 weeks of inactivity. Recurrence was observed in 39.6% of patients at a mean of 44.7 ± 61.7 weeks. Patients who experienced recurrence were significantly younger than those without recurrence (mean 29.4 years vs 37.5 years, P = .0005), and the rate of recurrence was significantly higher in younger subjects (hazard ratio [HR] = 0.88 per decade of increasing age, P = .01). The lowest rate of recurrence was among Asian subjects. A faster adalimumab taper was associated with an increased recurrence rate (HR = 1.23 per unit increase in speed, P < .0005). Conversely, a more extended period of remission before tapering was associated with a lower rate of recurrence (HR = 0.97 per 10-weeks longer period of inactivity, P = .04)., Conclusions: When tapering adalimumab, factors that should be considered include patient age, race, and duration of disease remission on adalimumab. A slow tapering schedule is advisable., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose: To describe the epidemiology, indications and surgical results of pars plana vitrectomy (PPV) in patients over 85 years of age., Methods: A retrospective cohort study was performed including all consecutive patients aged 85 years or older who underwent PPV between September 2018 and March 2022 in a single hospital in Madrid, Spain. Data on diagnosis, comorbidities, surgical indication, surgical details, surgical complications and surgical outcomes were collected from medical records., Results: A total of 124 eyes of 119 patients (56 males, 47.1%) underwent PPV. Median age was 87 years (range 85-96). The most common surgical indications were complications of cataract surgery in 34 patients (28.6%), macular epiretinal membrane in 32 (26.9%), and rhegmatogenous retinal detachment (RRD) in 12 (10.1%). Mean preoperative best corrected visual acuity (BCVA) was 13.33 ± 42.34 ETDRS letters and improved to 40.05 ± 41.04 letters at 3 months (p < 0.001). BCVA had improved in 68.82% of patients at 3 months. Patients with chronic kidney disease (CKD; p < 0.001), RRD (p = 0.003), ocular trauma (p = 0.001) and age-related macular degeneration (AMD; p = 0.002) showed worse BCVA at 3 months from surgery. Patients with better preoperative BCVA (p < 0.001), and those who underwent 25G PPV (p = 0.041) showed better visual outcomes., Conclusions: PPV is an effective technique for improving visual acuity in patients aged 85 years and older with vitreoretinal diseases. Visual outcomes were better when patients had a better preoperative visual acuity and underwent 25G PPV. Patients with a previous diagnosis of AMD or CKD, and those undergoing surgery for ocular trauma or RRD had worse visual outcomes., (© 2023. The Author(s).)

Purpose: To derive a Delphi method-based consensus for the surgical management of Full Thickness Macular Hole (FTMH) and Lamellar Macular Hole (LMH)., Methods: 37 expert VR surgeons from 21 mainly European countries participated in Delphi method-based questionnaire for diagnosis and treatment of FTMHs and LMHs., Results: A total of 36 items were rated in round 1 by 37 participants, of which 10 items achieved consensus: intraoperative verification of PVD; clinical superiority of OCT-based FTMH classification; practical ineffectiveness of ocriplasmin; circular 360° ILM peeling for small macular holes; use of regular surgical technique for the size of the hole in concomitant retinal detachment; performing complete vitrectomy; SF6 gas as preferred tamponade; cataract surgery if crystalline lens is mildly/moderately opaque; removal of both ILM and LHEP in LMH surgery. In round 2, 18 items with moderate consensus (45-70% agreement) in round 1 were rated by 35 participants. Final consensus was reached in 35% of questions related to both diagnosis and surgical procedures., Conclusions: This Delphi study provides valuable information about the consensus/disagreement on different scenarios encountered during FTMH and LMH management as a guide tosurgical decision-making. High rate of disagreement and/or variable approaches still exist for treating such relatively common conditions., (© 2023 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To describe the incidence, prevalence and etiologies of uveitis in Madrid, Spain., Methods: A retrospective cross-sectional study was performed in a single hospital. All consecutive cases of uveitis that attended the Hospital during year 2019 were included. Mean incidence and prevalence were calculated., Results: Three hundred and one cases were included. Of these, 127 were incident. This represents an incidence of 21.24 new cases per 100,000 persons/year, and a prevalence was 50.43 cases per 100,000 persons. Mean age was 56.89 ± 18.78 years, and 159 were women (52.8%). Sixty-two cases were infectious (20.6%). Age (p = .005), initial visual acuity (p = .001) and cystoid macular oedema (CMO; p = .010) were found to be independent predictors of the final best corrected visual acuity (BCVA) in patients with uveitis., Conclusions: Uveitis affects approximately 1 in 1800 persons in northern Madrid. Younger age, better initial visual acuities and the presence of CMO predicted better final BCVA.

Objectives: To describe multimodal imaging findings of vitamin A deficiency retinopathy., Methods: A retrospective study of patients with serum retinol < 0.3 mg/L. Fundus color photos, spectral domain-optical coherence tomography (SD-OCT), and fundus autofluorescence (FAF) were reviewed and, when available, electrophysiological tests were analyzed., Results: Forty-five eyes (63.9 ± 15.7 years) were included. Ultra-widefield fundus photography showed drusen-like deposits (53.3%) and macular retinal pigment epithelium (RPE) mottling (40%). The deposits were hypoautofluorescent, and a perifoveal hyperautofluorescent ring was present in 8.9%. By SD-OCT, the ellipsoid zone had an irregular appearance (100%) and conical deposits anterior to the RPE (33.3%). Electroretinogram (ERG) (66.7%) showed a decrease in b-wave in the scotopic registers, and microperimetry (4.4%) showed decreased foveal sensitivity. After vitamin A supplementation, SD-OCT and FAF showed resolution of all findings. Forty percent of eyes had restoration of the scotopic registers in ERG and improved macular sensitivity by microperimetry (4.4%)., Conclusions: Vitamin A deficiency causes a mild cone dysfunction in addition to the more severe absent rod response. [ Ophthalmic Surg Lasers Imaging Retina 2023;54:330-336.] .

Purpose: To describe the epidemiology and clinical characteristics of psoriatic arthritis (PsA) related uveitis in Madrid, Spain., Methods: A case series nested in a retrospective single-center cohort of 494 patients with PsA was performed. Patients older than 18 years old whit a clinical diagnosis PsA-related uveitis who attended the Ramon y Cajal University Hospital in Madrid, Spain, between 1st January 2017 and 31st December 2019 were included in the study. Epidemiological and clinical data were retrieved from the electronic medical records., Results: Thirteen cases of psoriatic arthritis-related uveitis (6 men and 7 women) were included. PsA-related uveitis showed an incidence of 0.05 cases per 100,000 persons/year (CI95 0.00-0.35), and a prevalence of 2.19 cases per 100,000 persons (CI95 1.24-3.79). The prevalence of active uveitis in the cohort of PsA patients was 2.6%. The first episode of uveitis (mean age of 48.15 ± 15.41 years) was anterior and unilateral in 92.31% of the cases. Most of the patients had a recurrent course (69.2%) with 0.92 flare-ups per patient/year (CI95 0.85-0.96). The uveitis preceded the diagnosis of psoriatic arthritis in 62.5% of the patients. In patients with PsA-related uveitis, HLA-B27 was present in 23.1%, HLA-Cw6 in 7.7%., Conclusions: Uveitis is a PsA manifestation that affects roughly 1 in 37 PsA patients, and that may precede the articular symptoms. It generally presents as a unilateral acute anterior uveitis and has a recurrent course. The most frequent observed complications are elevated intraocular pressure and cataracts., (© 2022. The Author(s), under exclusive licence to Springer Nature B.V.)

Sepsis is a heterogeneous disease with variable clinical course and several clinical phenotypes. As it is associated with an increased risk of death, patients with this condition are candidates for receipt of a very well-structured and protocolized treatment. All patients should receive the fundamental pillars of sepsis management, which are infection control, initial resuscitation, and multiorgan support. However, specific subgroups of patients may benefit from a personalized approach with interventions targeted towards specific pathophysiological mechanisms. Herein, we will review the framework for identifying subpopulations of patients with sepsis, septic shock, and multiorgan dysfunction who may benefit from specific therapies. Some of these approaches are still in the early stages of research, while others are already in routine use in clinical practice, but together will help in the effective generation and safe implementation of precision medicine in sepsis., Competing Interests: Conflict-of-interest statement: The authors declare having no conflicts of interest., (©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.)

Purpose: Coronavirus Disease 2019 (COVID-19) can cause conjunctivitis in up to 31.6% of patients. Additionally, retinal findings compatible with retinal microvascular ischemia have also been associated with coronavirus disease in asymptomatic patients. We describe a case of bilateral retinal changes compatible with microangiopathy occurring during the late phase of COVID-19. Case report: A 50-year-old man with bilateral pneumonia and positive polymerase chain reaction for SARS-CoV-2 developed an arcuate visual field defect in his left eye. Funduscopy revealed multiple, bilateral cotton-wool spots without haemorraghes. OCT-angiography revealed multifocal areas of retinal microvascular ischemia in the superficial plexus, the largest of which corresponded to the arcuate scotoma observed in the automated perimetry. Conclusion: Visual field defects due to retinal microangiopathy can occur during the late phase of COVID-19. Vascular changes observed in the retina may mimic what may be happening in other, less-accessible organs.

Background: To evaluate the contribution made to the diagnostic work-up for patients with suspected ocular tuberculosis (TB) by QuantiFERON-TB Gold In-Tube (QFT) tests using latent class analysis model., Methods: A single centre retrospective cohort study. A Bayesian latent class model was constructed on the basis of demographics, phenotypes and test results from patients attending a tertiary referral center in the UK. This estimated the probability of ocular TB for each patient in two versions, first with and then without QFT. The estimated probability of ocular TB was compared with treatment failure., Results: From a database of 365 patients with clinical signs suggestive of ocular TB, 267 patients who had QFT and complete data were evaluated. Mean age was 45.0 ± 15.4 years with 141 (52.9%) male and 148 (50.5%) of Asian ethnicity. QFT was positive in 208 (70.1%) patients and ATT was instituted in 145 (49.5%) patients with 100 (34.1%) patients also having concurrent systemic corticosteroid therapy. The best estimate of a QFT level separating TB-positive and TB-negative patients was extremely low. This weak discrimination between TB and non-TB groups was reflected in poor positive and negative predictive values for treatment failure., Conclusions: The latent class model did not successfully predict treatment failure, despite taking all variables into account. The threshold between TB and non-TB in QFT values was implausibly low and removing QFT from the model made prediction slightly worse. A larger prospective study is required to establish the role of all tests, demographics and phenotypes in diagnosis.

Purpose: Describe the clinical features and outcomes of patients with peripheral retinal vasculitis (RV) and describe clinical characteristics of presumed tubercular RV in a nonendemic setting., Methods: Retrospective cohort study of 110 consecutive patients with peripheral RV at a tertiary referral eye care center in the United Kingdom. Retinal vasculitis was defined as RV with vitritis associated with peripheral retinal ischemia. Patients who also had positive Quantiferon Gold in Tube test, positive tuberculin skin test, and/or other evidence of systemic tuberculosis such as biopsy were labeled with presumed tubercular RV. Treatment success was defined as resolution of inflammation, and successful tapering of oral corticosteroids to less than 10 mg/day or topical steroids to less than twice a day., Results: Mean age of the study population was 42.69 ± 14.95 years. Patients were predominantly Asian (49.1%) and Male (67.0%). A total of 73.2% had bilateral involvement. Sixty-nine (62.72%) patients had presumed tubercular RV. A total of 52.8% patients received antitubercular therapy, 65.5% received oral corticosteroids, and 17.3% required steroid-sparing oral immunosuppressive agents. A total of 85.19% of patients with presumed tubercular RV achieved treatment success with concurrent antitubercular therapy as against 75.61% of patients with nontubercular RV., Conclusion: This is the largest study of the epidemiology, clinical features, and outcomes of both peripheral RV and presumed tubercular RV to date. Presumed tubercular RV commonly seems to affect young males of Asian descent and had vitreous hemorrhage as common clinical findings and also demonstrated a good treatment outcome with antitubercular therapy.

Intraocular tuberculosis remains a diagnostic and management conundrum for both ophthalmologists and pulmonologists. We analyze the efficacy and safety of anti-tubercular therapy (ATT) in patients with intraocular tuberculosis and factors associated with favorable outcome. Twenty-eight studies are included in this review, with a total of 1,917 patients. Nonrecurrence of inflammation was observed in pooled estimate of 84% of ATT-treated patients (95% CI 79-89). There was minimal difference in the outcome between patients treated with ATT alone (85% successful outcome; 95% CI 25-100) and those with concomitant systemic corticosteroid (82%; 95% CI 73-90). The use of ATT may be of benefit to patients with suspected intraocular tuberculosis; however, this conclusion is limited by the lack of control group analysis and standardized recruitment and treatment protocols. We propose further prospective studies to better establish the efficacy of ATT and ascertain the factors associated with favorable treatment outcomes., Competing Interests: There are no financial support and conflict of interest for any author., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Aim: To assess the impact of antitubercular therapy (ATT), oral steroids and steroid sparing immunosuppressive treatment on treatment success in cases with presumed ocular tuberculosis in an area of low endemic prevalence., Methods: A retrospective cross-sectional study was performed for 213 patients with presumed ocular tuberculosis from a database from a tertiary referral eye hospital in the UK. A logistic regression model was constructed incorporating demographics, baseline characteristics and different cut-offs of QuantiFERON-TB Gold In-Tube test (QFT-G) to identify significant factors accounting for the variability of the response variable ('failure') across the whole group. Treatment failure was defined as the recurrence of inflammation or inability to taper steroids within 6 months of completion of ATT or after at least 6 months of treatment in the non-ATT group., Results: There were 126 patients who had at least 6 months of ATT. Patients with QFT-G values >1.50 (OR=0.20, 95% CI 0.09 to 0.48, p<0.001) had less risk of treatment failure as against those with QFT-G values between 0.35 and 1.50. Steroid sparing immunosuppressive agents reduced the chances of treatment success (OR=24.10, 95% CI 6.75 to 86.11, p<0.001). This effect persisted even after adjusting for potential confounding factors., Conclusions: Patients with higher values of QFT-G (>1.5) are more likely to have treatment success with ATT. In our model, steroid sparing immunosuppressive agents reduced the chances of success in both ATT and non-ATT-treated patients. It is unclear whether this effect reflects the intrinsic underlying severity of disease (ie, study bias), or whether steroid sparing immunosuppressive agents mitigate against successful ATT., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/)

Purpose: To describe the clinical and epidemiological characteristics of patients with posterior scleritis, and to analyze the response to treatment and time to relapse., Methods: Retrospective study of 114 cases of posterior scleritis from two tertiary care, university-affiliated, referral centers in the United Kingdom and India between 2004 and 2013. Data included sociodemographic factors, medical history, clinical, laboratory and ultrasound findings, therapies, and outcomes. LogMAR visual acuity at presentation and final visit and time to relapse were the main outcome measures., Results: The mean age was 45.9 ± 16.8 years, 71.1% were women, and 18 (15.8%) patients had bilateral disease; 71 (62.3%) cases were idiopathic. Rheumatoid polyarthritis (12.28%), systemic lupus erythematous (4.38%) and pANCA(+) systemic vasculitis (5.26%) were the most frequent systemic associations. VA improved by 0.24 ± 0.36 LogMAR between presentation and last follow up (p < 0.001). The median time to remission was 210 days (95% CI: 184-256 days). Recurrences after remission were observed in 36.63%. The observed incidence rate of posterior scleritis relapse after remission was 15.81% per person-year (95% CI: 11.78-20.77%). Systemic disease was present significantly in patients more than 50 years of age (OR = 2.29; 95% CI: 1.01-5.17; p = 0.044)., Conclusion: Posterior scleritis is an uncommon disease causing pain and visual loss. In around 40% of the cases, it can be associated with other systemic diseases. Median time to relapse was 210 days. Relapses may occur in around 1 in 3 patients, with an incidence rate of 15.81% per person/year.

Objective: To analyze the safety and efficacy of a nonselective cyclo-oxygenase (COX) inhibitor in the management of noninfectious, non-necrotizing anterior scleritis., Methods: Retrospective chart review of 126 patients with non-necrotizing anterior scleritis treated with oral flurbiprofen (Froben®(Abbott Healthcare)) with (Group B, n = 61) or without (Group A, n = 65) topical steroids was performed and time to remission was plotted., Results: The observed incidence rate was 1.07 (95% CI: 0.57-1.99) per 1000 person-years with failure rate of 0.68 (95% CI: 0.22-2.12) per 1000 person-years in Group A and 1.41 (95% CI: 0.67-2.96) per 1000 person-years in Group B. The failure rate was 3.97 (1.89-9.34) per 1000 person-years with hazard ratio of 10.01 (95% CI: 2.52-39.65; p < 0.001) for patients with associated systemic disease., Conclusion: To the of our best knowledge, this is the first and largest case series on the safety and efficacy of a nonselective COX inhibitor in the management of anterior scleritis.

Purpose: To describe the ocular and systemic features in biopsy proven (definite) and non-biopsy proven (clinical) ocular sarcoidosis and to compare the ocular features with those proposed by the International Workshop for Ocular Sarcoidosis (IWOS)., Methods: Retrospective chart review of 83 patients who attended a tertiary referral uveitis clinic and were diagnosed with sarcoidosis. Patients were divided into two groups based on the type of diagnosis: those who had tissue biopsy confirmed diagnosis 'definite sarcoidosis' (n= 42; 50.60 %) and those who had 'clinical sarcoidosis' (n= 41; 49.40%). Ocular and systemic manifestations, including lung function tests and bronchoalveolar lavage findings were compared in the two groups. The ocular features were also compared with the categories laid down by the International Workshop on Ocular Sarcoidosis (IWOS)., Results: The mean age at presentation was 38.75 years (SD=12.33), 55.42% patients were female and mean follow-up was 24.35 months (SD=18.35). Trabecular meshwork nodules and/or tent-shaped PAS (category II of IWOS) were observed more frequently in patients with biopsy proven sarcoidosis (26.19 % v/s 9.76%; p=0.08). After logistic regression analysis, the predictor coefficient curve showed area under curve of 0.7262. Lymphocytosis (38.61% and 28.02%, p=0.93) and monocytosis (55.11% and 53.83%, p=0.56) on bronchoalveolar lavage analysis was present in both the groups, highlighting presence of granulomatous disease., Conclusion: This study suggests high reliability for the clinical diagnosis of ocular sarcoidosis in patients with signs recommended by IWOS and that our diagnostic criteria are consistent with that of the IWOS.

Objective: To analyze the factors affecting the treatment outcome in patients with presumed ocular tuberculosis on anti-tubercular therapy (ATT)., Methods: Retrospective chart review of patients with presumed ocular tuberculosis seen at a tertiary referral eye care center in the United Kingdom. Failure was defined as recurrence of inflammation within 6 months of completion of ATT., Results: There were a total of 175 patients with presumed ocular tuberculosis who had ATT. Patients with intermediate uveitis or panuveitis and those on immunosuppressive therapy had higher odds of treatment failure (p < 0.05) while those with more than 9 months of ATT (77, 79.38%) had less likelihood of failure., Conclusion: We present the largest case series of patients with presumed ocular tuberculosis in a low endemic area treated with ATT. Longer duration of treatment resulted in reduced risk of recurrence of inflammation, whereas immunosuppression adversely affected the final treatment outcome.

Background: Due to the high rates of antimicrobial resistance to certain antibiotics, together with the emergence of Neisseria gonorrhoeae (NG) with reduced susceptibility and resistance to third-generation cephalosporins, gonococcal infection is becoming a public health problem. The objectives of the study were: To keep track of the antimicrobial susceptibility of NG strains obtained from January to August 2011. To study the population dynamics., Methods: The antimicrobial susceptibility was studied by disk-diffusion and E-test. The genotyping was performed by NG-MAST method., Results: Of a total of 100strains studied, 59% showed intermediate sensitivity to penicillin and 9% were resistant. According to EUCAST, we detected 3gonococci with reduced susceptibility to ceftriaxone, 10 to cefixime and one with high-level resistance to both antibiotics (MIC 1.5μg/ml). MIC50 and MIC90 to cefixime were 0.016 and 0.125μg/ml, respectively, whereas to ceftriaxone they were <0.016 and 0.064μg/ml, respectively. Almost all (99%) of the strains were resistant to doxycycline, 53% to ciprofloxacin, 3% to azithromycin, and 1% to spectinomycin. The most prevalent ST was ST1407, predominantly associated to resistance or reduced sensitivity to cephalosporins or macrolides., Conclusions: NG has developed significant rates of resistance to various antibiotics. One strain has been detected with high level resistance to third generation cephalosporins, and several strains with reduced susceptibility. An increase in MIC50 and MIC90 to these antibiotics has also been observed. NG population structure remains stable and common to the rest of Europe, although two new ST (ST7226 and ST7227) have been identified that could be selected and acquire high levels of resistance to cephalosporins., (Copyright © 2012 Elsevier España, S.L. All rights reserved.)