Transepithelial Accelerated Crosslinking for Progressive Keratoconus: A Critical Analysis of Medium-Term Treatment Outcomes.

Purpose: To report the 4-year outcomes of transepithelial accelerated corneal collagen crosslinking (TE-ACXL) in the treatment of eyes with progressive keratoconus (KC). Methods: Eyes of patients who underwent TE-ACXL (6mW/cm2 for 15 minutes) for progressive KC and presented 48 months of follow-up were included. Corrected distance visual acuity (CDVA), keratometry measurements (Kmax, maximum keratometry, Kmean, mean keratometry and Astg, corneal astigmatism), thinnest corneal thickness (PachyMin), and topographic, and tomographic indices (specifically the posterior radius of curvature from the 3.0 mm centered on the thinnest point of the cornea (PRC), and the D-index) were analysed preoperatively and every 12 months after TE-ACXL, up to 48 months. Progression after TE-ACXL was considered when eyes presented ≥1 criteria: (1) increase of ≥1D in Kmax or increase of ≥0.75D in Kmean or increase of ≥1D in Astg; (2) reduction of ≥0.085 mm in PRC; (3) decrease ≥5% in PachyMin. Results: 41 eyes from 30 patients were included, with a mean age at crosslinking of 20.90±4.69 years. There was a significant increase in Kmean (+0.64±1.04 D, p<0.001; +0.98 ± 1.49 D, p<0.001; +1.27±2.01 D, p<0.001; +1.13±2.00 D, p=0.006) and a significant decrease in PRC throughout follow-up (-0.12±0.22, p=0.002; -0.15±0.24, p<0.001; -0.17±0.43, p=0.021; -0.16±0.43, p=0.027). PachyMin decreased significantly at 36 and 48 months (-8.50±15.93 µm, p=0.004; -7.82±18.37, p=0.033). According to our progression criteria, there was a major progression rate throughout follow-up (57.1%, 61.1%, 58.8%, and 67.9%, respectively). Surgery and follow-up were uneventful in all subjects. Eleven eyes (26.8%) required further procedures, ≥36 months after the initial TE-ACXL, due to persistent progressive disease. Conclusion: TE-ACXL proved to be a safe therapeutic option for progressive KC. However, its efficacy is deemed unsatisfactory, as a notable proportion of affected eyes may continue to advance within a 4-year timeframe, necessitating additional procedures to halt the disease's course.

Clinical outcomes and prognostic factors in Acanthamoeba keratitis.

PURPOSE: To report clinical findings and prognostic factors for visual and morphological outcomes in patients with Acanthamoeba keratitis (AK). METHODS: Single-center, retrospective, longitudinal study of 51 cases of AK diagnosed by real-time polymerase chain reaction (RT-PCR) between March 2010 and October 2022. The primary outcome was the final best corrected visual acuity (BCVA). Poor visual outcome was defined as a final BCVA ≥ 1 logMAR unit, while good visual outcome was defined as a final BCVA < 1 logMAR unit. Eyes from these two groups were compared, regarding demographic and initial clinical variables, anti-Acanthamoeba treatment used, and complications of the disease. Early diagnosis was defined as ≤ 14 days from symptom onset to diagnostic confirmation and initiation of Acanthamoeba medical treatment. Multivariable logistic regression was used to determine predictors of poor visual outcome. RESULTS: A total of 51 eyes from 46 patients diagnosed with AK, all contact lens (CL) wearers, were included in this study. Average follow-up was 39.0 ± 30.2 [total range 14-120] months. Thirty-one eyes (60.8 %) presented good visual outcome, with a lower baseline age (30.5 ± 9.0 vs. 42.3 ± 15.8; p = 0.020), better initial BCVA (0.8 ± 0.7 logMAR units vs. 1.3 ± 0.9 logMAR units; p = 0.047), higher rate of early diagnosis (45.2 % vs. 5.6 %; p = 0.004), and higher rate of therapeutic epithelial debridement (64.5 % vs. 10 %; p < 0.001). 20 eyes (39.2 %) presented poor visual outcome, with 12 eyes undergoing evisceration/enucleation (23.5 %). These 20 eyes presented a higher rate of complications (90 % vs. 61.3 %; p = 0.031). In multivariable analysis, early diagnosis of AK (OR 19.78; 95 % CI 2.07-189.11; p = 0.010) and therapeutic epithelial debridement (OR 19.02; 95 % CI 3.27-110.57; p = 0.001) were associated with a good visual outcome. CONCLUSIONS: In the present study, poor visual outcome was present in 39 % of affected eyes. Early AK diagnosis (≤14 days from symptom onset) and therapeutic epithelial debridement were associated with good final visual outcome.

O enfermeiro de reabilitação e a saúde escolar: postura corporal / The rehabilitation nurse and school health: body position

Introdução: O estágio de natureza profissional é o culminar do curso de mestrado em enfermagem de reabilitação e permite desenvolver atividades em contexto prático e real promovendo o desenvolvimento de capacidades específicas desta área de intervenção. Assim, este momento de aprendizagem desenvolveu-se numa Unidade de Cuidados na Comunidade, no âmbito da saúde escolar. O uso regular de mochilas com peso excessivo produz um grande impacto na postura corporal das crianças e adolescentes, devido às alterações no sistema músculo-esquelético, deformação e dor. Emerge daqui a necessidade de desenvolvimento de um conjunto de intervenções de cariz preventivo relativamente às mochilas. Objetivos: Explorar a mais-valia de uma ação de sensibilização em enfermagem de reabilitação na prevenção de sequelas músculo-esqueléticas decorrentes da utilização e transporte desadequados das mochilas. Metodologia: Metodologia de Projeto, desenvolveram-se três atividades major: Avaliação antropométrica e avaliação do peso das mochilas; Sessões de educação para a saúde; implementação do projeto O Enfermeiro de Reabitação e a Saúde Escolar: postura corporal; reavaliação antropométrica e reavaliação do peso das mochilas, de modo a avaliar a mais-valia de uma ação de sensibilização em enfermagem de reabilitação, ao capacitar alunos para a adoção de hábitos que previnam sequelas músculo-esqueléticas decorrentes da utilização e transporte desadequados das mochilas. Resultados: Os resultados do estudo permitem-nos concluir que, relativamente ao transporte, ajuste e organização da mochila, houve melhorias. Relativamente ao peso da mochila, diminuiu do primeiro para o segundo momento de avaliação. A análise estatística indica que o peso da mochila diminuiu de 12,27% para 8,60% do peso corporal, do primeiro para o segundo momento de avaliação. Conclusões: A ação de sensibilização implementada no âmbito da saúde escolar surtiu efeito positivo na correção da organização e método de transporte das mochilas, bem como na redução do peso das mesmas.

Introduction: The internship of a professional nature is the culmination of the master's course in rehabilitation nursing and allows the development of activities in a practical and real context, promoting the development of specific capacities in this area of intervention. Thus, this learning moment was developed in a Community Care Unit, within the scope of school health. The regular use of backpacks with excessive weight has a great impact on the body posture of children and adolescents, due to alterations in the musculoskeletal system, deformation and pain. From this emerges the need to develop a set of interventions of a preventive nature in relation to backpacks. Objectives: To explore the added value of an awareness-raising action in rehabilitation nursing in the prevention of musculoskeletal sequelae resulting from the inappropriate use and transport of backpacks. Methodology: Project Methodology, three major activities were developed: Anthropometric assessment and backpack weight assessment; Health education sessions; implementation of the project The Rehabilitation Nurse and School Health: body posture; anthropometric reassessment and reassessment of the weight of the backpacks, in order to assess the added value of an awareness-raising action in rehabilitation nursing, by training students to adopt habits that prevent musculoskeletal sequelae resulting from the inappropriate use and transport of backpacks. Results: The results of the study allow us to conclude that, regarding the transport, adjustment and organization of the backpack, there have been improvements. Regarding the weight of the backpack, it decreased from the first to the second moment of evaluation. Statistical analysis indicates that the backpack weight decreased from 12.27% to 8.60% of body weight, from the first to the second moment of evaluation. Conclusions: The awareness-raising action implemented in the context of school health had a positive effect on correcting the organization and method of transporting the backpacks, as well as reducing their weight.

Microbiological Profile of Infectious Keratitis During Covid-19 Pandemic.

Purpose: The Covid-19 pandemic introduced significant changes in our daily life, including the widespread use of face masks. The purpose of this study was to assess if significant changes occurred in the microbiological profile of infectious keratitis. Patients and Methods: A retrospective study was performed, based on a survey review of the electronic medical records of all patients with presumed infectious keratitis, between March 2020 and October 2021. The microbiological isolates in this sample (pandemic group) were compared with those obtained in our center between 2009 and 2018 (pre-pandemic group). Results: A total of 194 samples were included in the pandemic group. We obtained a culture-positivity rate of 43.3%, which was significantly higher when compared with the pre-pandemic data (35.15%, p = 0.033). Several further significant differences were found between the pandemic and the pre-pandemic groups: the proportion of bacteria, including gram-positive and gram-negative groups, was higher in our sample (pre-pandemic vs pandemic: 76.78% vs 83.33%, p = 0.010; 53.35% vs 60.71%, p = 0.016; 23.43% vs 34.52%, p = 0.005, respectively); two populations of Gram-positive bacteria found in this study were not isolated in the pre-pandemic sample - Dolosigranulum pigrum and Propionibacterium spp.; and two bacterial isolates were significantly increased in our sample - Corynebacterium spp. (18.41% vs 29.76%, p = 0.003) and Pseudomonas aeruginosa (9.00% vs 16.66%, p = 0.012). Conclusion: In conclusion, significant changes were found in the microbiological profile of infectious keratitis in our center during the Covid-19 pandemic. While these changes could be related to face mask use, more observational and experimental studies are needed to explore this possible association.

Rhino-Orbital Mucormycosis After COVID-19 Recovery: A Case Report.

The pandemic caused by SARS-CoV-2 remains a health care concern, despite vaccination programs. Mucormycosis, especially rhino-orbital-mucormycosis, has been described as a severe complication of COVID-19. Although it has been described mostly in India and other developing countries, few cases in the western world have also been described. We present a case of rhino-orbito-mucormycosis after recovery from severe COVID-19 in Portugal. A 75-year-old diabetic and obese man presented with right proptosis associated with right eye pain and low vision one month after recovery from severe COVID-19. Considering the most probable etiology for this clinical picture, anti-fungal therapy with liposomal amphotericin B was promptly initiated, followed by endoscopic sinus debridement. However, due to persistent and progressive infection, and after a multidisciplinary revision of the case, orbital exenteration was performed. One year after surgery, the patient is stable, without clinical or imagological signs of relapse of the disease. Although the evolution of the pandemic, along with vaccination programs, led to a lower incidence of severe COVID-19 disease, there are still patients presenting with severe COVID-19, requiring intensive care and at risk for serious complications. This case illustrates the importance of being aware of the development of post-COVID-19 mucormycosis and the need for close surveillance of patients recovering from severe COVID-19. COVID-19 prompt diagnosis and multidisciplinary approach are essential for a timely intervention achieving better survival while minimizing morbidity.

Hypotony Maculopathy Related to Anti-VEGF Intravitreal Injection.

Purpose: To describe a case of hypotony maculopathy following anti-VEGF intravitreal injection (IVI) in a patient with pseudoxanthoma elasticum (PE). Methods: Clinical case report. Results: A 52-year-old male complained of right eye (OD) vision loss 2 days after an uncomplicated anti-VEGF IVI for the treatment of choroidal neovascularization secondary to angioid streaks. Relevant medical history included PE, pathologic myopia, and a previous pars plana vitrectomy (PPV) due to a retinal detachment. OD best-corrected visual acuity (BCVA) dropped from 6/12 to 6/18 after the IVI. Intraocular pressure (IOP) was 3 mmHg and chorioretinal folds were evident in the posterior pole. Topical dexamethasone and atropine were prescribed, and full recovery was noticed after 3 days. Four months later, the patient developed a new episode of vision loss after another IVI. His BCVA was counting fingers, IOP was 2mmHg, and more noticeable chorioretinal folds were found. This time, an open scleral wound at the injection site was evident and a scleral suture was necessary. Once again, the patient recovered well. Conclusion: Hypotony maculopathy following intravitreal injection is a rare condition. However, the described patient presented several conditions which could be related with poor scleral wound closure: intrinsic scleral fragility due to myopia and pseudoxanthoma elasticum; repeated IVI procedures; and absence of vitreous in the posterior segment due to prior vitrectomy. Despite the good outcome, hypotony maculopathy may be a sight-threatening condition, and special attention is necessary for specific patients with risk factors.

Prevalence and Area of Retinal Pigment Epithelium and Outer Retinal Atrophy in Eyes with Non-Exudative Macular Neovascularization.

PURPOSE: The objective of this study wasto assess the prevalence of complete retinal pigment epithelium (RPE) and outer retinal atrophy (cRORA) in patients with unilateral exudative age-related macular degeneration (AMD) of the fellow eye and establish if the presence of non-exudative macular neovascularization (NE-MNV) influences the prevalence of RPE and outer retinal atrophy in eyes with AMD. METHODS: This is an observational cross-sectional study of 68 patients with unilateral exudative AMD. Demographic and clinical data were collected, and multimodal retinal imaging was performed in all patients. Two groups of patients were defined according to the presence (NE-MNV) or absence (no NE-MNV) of NE-MNV in the study eye. We compared the prevalence of tomographic cRORA and fundus autofluorescence (FAF) geographic atrophy (GA) and differences in cRORA greatest linear diameter (GLD) and GA area between groups. RESULTS: Globally, cRORA was present in 11 eyes (16.2%), FAF GA was present in 10 eyes (14.7%), and NE-MNV was present in 10 eyes (14.7%) of patients with unilateral exudative AMD of the fellow eye. The overall cRORA GLD was 1,950.64 ± 1,428.31 µm, and the mean area of GA was 9.25 ± 7.50 mm2. Regarding comparisons between groups, cRORA was present in 9 eyes (15.5%) without NE-MNV and in 2 eyes (20%) with NE-MNV (p = 0.66). Tomographic signs of atrophy were more frequent in eyes with NE-MNV (50% vs. 24.1% in eyes without NE-MNV; p = 0.008). No significant differences were found in cRORA GLD (p = 0.30) between groups. Eyes with NE-MNV and eyes without NE-MNV had a similar prevalence of FAF GA (2 eyes out of 10 and 8 eyes out of 58, respectively; p = 0.64). Eyes with NE-MNV had a smaller mean area of GA (2.07 ± 0.24 mm2 vs. 11.05 ± 7.34 mm2; p = 0.01). CONCLUSION: In our study, the presence of NE-MNV was not associated with the prevalence of cRORA and/or FAF GA. Nonetheless, eyes with NE-MNV presented smaller areas of GA, which suggests that this type of neovascularization may prevent the progression of RPE and outer retinal atrophy. Longitudinal studies are required to confirm these preliminary results.

Adaptation and validation of the Mood Rhythm Instrument for use in Brazilian adolescents

Objective: Adapt and validate the Mood Rhythm Instrument (MRhI), a self-reported questionnaire that assesses self-perceived rhythmicity of mood-related symptoms in adults, into a version that assesses and evaluates perceived mood-related symptoms in adolescents (MRhI-Y). Methods: Adaptation of the Brazilian Portuguese version of the MRhI for an adolescent population followed three steps: review by consultants, analysis by experts, and pilot testing through a visual analogue scale (VAS). The final questionnaire (MRhI-Y) was applied to 171 adolescents aged 12-17 years. Internal consistency was calculated using Cronbach's alpha and McDonald's omega. The psychometric properties of the MRhI-Y were evaluated using exploratory factor analysis (EFA). Results: The MRhI-Y was designed to use wording more appropriate for adolescents than that of the MRhI. Expert agreement about item quality ranged between 82 and 100%. Adolescents' VAS ratings indicated good comprehension of the items. Cronbach's alpha and McDonalds' omega coefficients were 0.71 and 0.74. The EFA resulted in a three-factor solution (affective, cognitive, and somatic). Younger adolescents (ages 12 to 13) reported lower rhythmicity scores than older groups (ages 14 to 15 and 16 to 17), even controlling for chronotype. Conclusions: The Brazilian Portuguese version of the MRhI-Y presented adequate comprehension by adolescents and good internal consistency. The MRhI-Y is a promising tool to improve our understanding of the underlying characteristics of mood fluctuation in adolescence.

Adaptation and validation of the Mood Rhythm Instrument for use in Brazilian adolescents.

OBJECTIVE: Adapt and validate the Mood Rhythm Instrument (MRhI), a self-reported questionnaire that assesses self-perceived rhythmicity of mood-related symptoms in adults, into a version that assesses and evaluates perceived mood-related symptoms in adolescents (MRhI-Y). METHODS: Adaptation of the Brazilian Portuguese version of the MRhI for an adolescent population followed three steps: review by consultants, analysis by experts, and pilot testing through a visual analogue scale (VAS). The final questionnaire (MRhI-Y) was applied to 171 adolescents aged 12-17 years. Internal consistency was calculated using Cronbach's alpha and McDonald's omega. The psychometric properties of the MRhI-Y were evaluated using exploratory factor analysis (EFA). RESULTS: The MRhI-Y was designed to use wording more appropriate for adolescents than that of the MRhI. Expert agreement about item quality ranged between 82 and 100%. Adolescents' VAS ratings indicated good comprehension of the items. Cronbach's alpha and McDonalds' omega coefficients were 0.71 and 0.74. The EFA resulted in a three-factor solution (affective, cognitive, and somatic). Younger adolescents (ages 12 to 13) reported lower rhythmicity scores than older groups (ages 14 to 15 and 16 to 17), even controlling for chronotype. CONCLUSIONS: The Brazilian Portuguese version of the MRhI-Y presented adequate comprehension by adolescents and good internal consistency. The MRhI-Y is a promising tool to improve our understanding of the underlying characteristics of mood fluctuation in adolescence.

Quantitative Optical Coherence Tomography Angiography Biomarkers for Alport Syndrome.

PURPOSE: The aim of this study was to evaluate microvascular abnormalities of patients with Alport syndrome (AS) using optical coherence tomography angiography (OCT-A) quantitative biomarkers. METHODS: This was cross sectional, prospective evaluation of consecutive patients with AS and healthy subjects. AS diagnosis was performed by the genetic test. All participants underwent a retinal vasculature evaluation by spectral-domain optical coherence tomography (SD-OCT) and OCT-A of the macula. Quantitative analysis included whole vascular density, foveal avascular zone area, fractal dimension (FD), and lacunarity (LAC). RESULTS: Ninety-four eyes were included in this study, 45 eyes from patients with AS and 49 eyes from healthy subjects. The pathogenic mutation in the COL4A5 gene on the chromosome X was found in 14 patients; the pathogenic autosomal recessive mutations in the COL4A3 gene were found in 9 patients. Quantitative evaluation demonstrated a significant difference between AS and healthy subjects on LAC of the superficial capillary plexus and deep capillary plexus (DCP) (p < 0.001 and p < 0.001, respectively) and on FD in the DCP (p < 0.001). CONCLUSION: The DCP Alport patients have a higher vessel nonuniformity than DCP of healthy subjects. We hypothesize that endothelial cell lesion in the setting of low resistance at the DCP circuit could lead to long-term structural disorganization.

Increased Choroidal Thickness in Morquio Syndrome.

The purpose of this clinical case report is to describe a case of mucopolysaccharidosis type IVA (MPS IVA), or Morquio syndrome, with increased choroidal thickness in enhanced-depth imaging optical coherence tomography (EDI-OCT) which can represent choroidal deposition of glycosaminoglycans (GAGs). A 21-year-old male with genetically confirmed diagnosis of MPS IVA was examined at our Pediatric Ophthalmology clinic as part of our follow-up protocol for MPS patients. His best-corrected visual acuity was 4/10 in his right eye (OD) and 6/10 in the left eye (OS). Mild diffuse corneal opacification was evident. Intraocular pressure was within normal range. Fundus examination and color fundus photography revealed no abnormalities. EDI-OCT revealed significantly increased choroidal thickness in his right eye and in his left eye, suggesting the presence of choroidal deposition of GAGs, despite absence of retinal or optic disc GAG deposition or other chorioretinal involvement. To our knowledge, this is the first case of MPS IVA described in the literature with suspected choroidal deposition of GAGs. With improved control of systemic features of MPS IVA, life expectancy of these patients has increased, allowing for more ocular manifestations to develop. The parallel development of technology in ophthalmology, such as the EDI-OCT, further contributes to the detection of these unprecedented ocular features in MPSs.

Keratoconus enlargement as a predictor of keratoconus progression.

Numerous approaches have been designated to document progression in keratoconus, nevertheless there is no consistent or clear definition of ectasia progression. In this present study, we aim to evaluate Keratoconus Enlargement (KCE) as a parameter to document ectasia progression. We define KCE as an increase of more than 1D in the anterior curvature of non-apical corneal areas. We have designed a longitudinal study in 113 keratoconic eyes to assess keratoconus progression. KCE was compared with variables commonly used for detection of keratoconus progression like Kmax, Km, K2, PachyMin, D-Index, Corneal Astigmatism and PRC of 3.0 mm centered on the thinnest point. The variations of keratometric readings, D-index and ELEBmax showed positive associations with KCE. Evaluating the performance of Kmax, D-index and KCE as isolated parameters to document keratoconus progression we found a sensitivity of 49%, 82% and 77% and a specificity of 100%, 95% and 66% to detect keratoconus progression (p < 0.001 for all). This difference in sensitivity can be explained by the changes in keratoconus outside the small area represented by Kmax. The inclusion of KCE should be considered in the evaluation of keratoconus progression in conjunction with other variables to increase the reliability of our clinical evaluation.

Endophthalmitis following Intravitreal Injection, Cataract Surgery, and Vitrectomy: Clinical Features and Visual Outcomes.

PURPOSE: To describe and compare the clinical features and visual outcomes of endophthalmitis following intravitreal injections (IVI), cataract surgery, and pars plana vitrectomy (PPV). METHODS: This is a single-centre, retrospective study. All included patients had acute postoperative endophthalmitis secondary to one of these three procedures. Visual acuity (VA), comorbidities, time to presentation, and treatment were assessed. The primary outcome was visual outcome. A poor outcome was considered if final VA was worse than or equal to counting fingers (CF) and a good outcome was classified as VA better than CF. RESULTS: Over 12 years, a total of 61 patients were included. Twenty-seven cases were post-cataract endophthalmitis; twenty-five were post-IVI and nine post-PPV. Endophthalmitis post-PPV had a worse visual outcome (88.9% of patients with VA worse than or equal to CF 95% CI 51.3 to 100.0%) than endophthalmitis following cataract surgery (25.9% of patients with VA worse than or equal to CF 95% CI 11.0 to 39.9%) and the IVI subgroup (44.0% of VA worse than or equal to CF 95% CI 24.0 to 67.0%) (p=0.001 and p=0.047). There were no significant differences in the proportion of patients with a poor visual outcome between endophthalmitis following cataract surgery and IVI (p=0.171). CONCLUSIONS: The number of patients with poor visual outcomes following acute endophthalmitis was similar in endophthalmitis following IVI and cataract surgery, but better than endophthalmitis following vitrectomy.

Macular Changes in a Mucopolysaccharidosis Type I Patient with Earlier Systemic Therapies.

PURPOSE: To describe retinal findings in a patient with mucopolysaccharidosis type I (MPS I) that underwent an early treatment with hematopoietic stem cell transplantation (HSCT) and enzyme replacement therapy (ERT). Case Report. We describe a case of a 12-year-old female with a biochemical and genetic diagnosis of MPS I. She underwent HSCT and ERT on the first year of life. The visual acuity was 5/10 in both eyes and she had bilateral grade 2 corneal haze. Spectral domain optical coherence tomography (SD-OCT) revealed thickening of the external limiting membrane (ELM) at the fovea. In the parafoveal and perifoveal regions, SD-OCT displayed a loss of the interdigitation, ellipsoid, and myoid zones and of the ELM accompanied by progressive thinning of the outer nuclear layer. Fundus infrared imaging revealed a hyperreflective ring centred on the fovea and hyporeflective areas in temporal parafoveal regions in both eyes. En face OCT imaging revealed two hyperreflective rings on the outer retinal level. CONCLUSION: This patient developed macular changes with foveal deposition of hyperreflective material and parafoveal thinning, despite early systemic treatment. Systemic therapies can provide an increase in life expectancy and stabilize visual acuity and corneal clouding, although their effect on retinal degeneration is unknown.

Childhood-Onset Leber Hereditary Optic Neuropathy: Particular Features.

BACKGROUND: Leber hereditary optic neuropathy (LHON) is an optic neuropathy of mitochondrial inheritance. Childhood-onset disease is relatively rare and there are limited data on this important patient subgroup. CASE PRESENTATION: We present 3 particular presentations of LHON. Patient 1 was an 8-year-old boy admitted to the emergency department reporting a progressive bilateral visual loss and intermittent headaches. Neuro-ophthalmological examination revealed a bilateral pseudopapilledema. Lumbar puncture identified intracranial hypertension and the brain and orbits magnetic resonance imaging showed T2 hyperintensity in the posterior region of the left optic nerve and the optic chiasm. Patient 2 was a 12-year-old boy admitted to the emergency department reporting painless, progressive central vision loss in the right eye. Fundus examination revealed a hyperemic disc and vascular network papillary and peripapillary vascular microdilations. Three months later, the left eye presented visual loss. Patient 3 was a 6-year-old female child referred to the neuro-ophthalmology specialist due to painless central visual loss in both eyes. Her BCVA was 1/10 and counting fingers in right and left eye, respectively, and fundus examination revealed a pallor optic disc in the temporal sector. DISCUSSION: The phenotype of childhood-onset disease may present itself distinct from classical adult-onset LHON. The absence of classical clinical features could lead to initial misdiagnosis. There should exist a high index of suspicion in children presenting unexplained subnormal vision in order to avoid potential diagnostic delays.

Dental Management of a Patient with Special Health Care Needs.

Patients with special health care needs (PSHCN) may have an increased risk of oral disease throughout the course of their life and require particular delivery of dental care due to their medical condition or limitations. The purpose is to report the dental management of a patient with Cornelia de Lange Syndrome (CdLS), which was classified as PSHCN due to physical, behavioural, cognitive, and emotional impairment. A 14-year-old female with a clinical diagnosis of CdLS and its common craniofacial features such as microcephaly, short neck, synophrys, arched eyebrows, downturned angle of the mouth, high arched palate, micrognathia, and microdontia was referred to the hospital where the dental treatment was performed under general anesthesia. Multiple tooth extraction, fillings, and coronary polishing were performed. During the follow-up, we observed that dental restorations were clinically satisfactory and there was an improvement in the patient's behaviour during dental treatment. Managing and shaping behaviour of such patients are crucial to delivering quality dental care, as they require specialized care due to their behavioural and clinical conditions.

Child with a mild phenotype of Incontinentia Pigmenti and inner retinal dysfunction.

PURPOSE: To describe a case of a child with mild phenotype of Incontinentia Pigmenti (IP), with changes in Spectral-Domain Optical Coherence Tomography (SD-OCT) and Optical Coherence Tomography Angiography (OCT-A) and an electronegative dark-adapted (DA) 3.0 electroretinogram (ERG), suggestive of inner retinal dysfunction. CASE REPORT: We described a 7-year-old female child with IP. Her best corrected acuity was 8/10 in the right eye and 6/10 in the left eye. Biomicroscopy, intraocular pressure and fundoscopy were normal. The electroretinography findings showed an electronegative DA 3.0 ERG with a normal a-wave but a b-wave that did not elevate above baseline. SD-OCT identified irregularities in the outer plexiform layer in both eyes, and OCT-A assessment revealed at the superficial capillary plexus, areas of decrease in the flow in parafoveal and perifoveal regions. CONCLUSION: Classically, IP affects the peripheral retina; however, vascular and structural changes in macula can occur as well. To our knowledge, we report the first electronegative electroretinogram in a patient with IP.

Nocardia Subretinal Abscess: A Rare and Challenging Case Report.

PURPOSE: To describe a positive clinical response of a patient with submacular Nocardia abscess due to a rapid and efficient treatment. Case report. We describe a case of a 50-year-old man with a painless visual decline of the left eye. Four years later, he had been diagnosed with systemic nocardiosis. Examination of the left eye revealed a submacular white mass with fluffy borders and another smaller white lesion, with well-defined borders, in the inferior temporal vascular arch. A systemic antibiotic treatment with SMX-TMP and intravenous imipenem and a single intravitreal injection of bevacizumab was performed. CONCLUSION: Prompt diagnosis and treatment ensured an expeditious resolution of the abscess and significant improvement of visual acuity. The diagnostic approach of a high index of suspicion coupled with directed treatment is required when dealing with subretinal inflammatory lesions.

Covid-19 Impact on Macular Neovascularization and Retinal Vein Occlusion Treatment: Single-Center Experience.

PURPOSE: The aim of this study was to evaluate whether the coronavirus disease 19 (COVID-19) pandemic resulted in undertreatment and subsequent loss of visual acuity (VA) in patients with macular neovascularization (MNV) or retinal vein occlusion (RVO) regularly treated with intravitreal antivascular endothelial growth factor injections. METHODS: Single-center, retrospective study of patients scheduled for treatment between March 19 and June 1, 2020, the national mandatory quarantine period. Patients' demographics, VA, and scheduled treatment during this period were reviewed via medical records. All patients were analyzed regarding treatment attendance rates. The visual impact of COVID-19 was assessed in patients who had been treated and presented a stable VA for >6 months before the beginning of the quarantine. RESULTS: This study included 927 eyes from 769 patients. The attendance rate increased throughout the study timeframe (p < 0.001) and correlated negatively with higher patient's age (r = -0.142; p = 0.005). Patients with age-related macular degeneration (67.6%) had lower attendance rates (p = 0.007) and were older (p < 0.001). The visual impact analysis included 400 eyes from 325 patients. The average VA variation throughout this period was -1.7 ± 8.4 ETDRS letters and was similar in different retinal pathologies (p = 0.334). VA variation did not correlate with the number of missed treatments per patient (r = 0.100; p = 0.150). The prevalence of subretinal fluid and intraretinal fluid, as well as central retinal thickness decreased significantly throughout the study period (p values of <0.001, <0.001, and 0.032, respectively). CONCLUSION: The COVID-19 pandemic had a significant impact on the attendance rate of patients with MNV or RVO to their scheduled treatments, which was higher in the first week of mandatory quarantine. Nevertheless, VA did not decrease significantly during this period, with a limited VA variation regardless of primary retinal disorder and morphological parameters even improved in the eyes included in the visual impact analysis.

A 10-Year Retrospective Clinical Analysis of Fungal Keratitis in a Portuguese Tertiary Centre.

PURPOSE: To determine the microbiological profile, risk factors, treatment and surgical intervention rates of fungal keratitis at a tertiary referral centre. METHODS: A retrospective review of microbiological and medical records from hospitalised patients treated for fungal keratitis at Centro Hospitalar Universitário de São João from 2009 to 2019 was conducted. RESULTS: Overall, 43 patients were included in our study. The mean age of patients was 63.7 years and 46.5% were men. In culture were isolated 22 (51.2%) filamentous fungi and 21 (48.8%) yeast. Candida species (n = 20, 46.5%), Fusarium species (n = 10, 23.4%) and Aspergillus species (n = 4, 9.3%) were the most common isolated species. Important risk factors were contact lens use (n = 24, 55.8%), long-term users of topical corticosteroids (n = 19, 44.2%) and previous keratitis (n = 19, 44.2%). Yeast isolates had a statistically significant higher prevalence in long-term users of topical corticosteroids compared to filamentous ones (p = 0.043). Twenty-four cases (55.8%) required surgical intervention, of which 23 cases underwent therapeutic penetrating keratoplasty. Ocular complications, such as evisceration was noted in 12 patients (27.9%) and endophthalmitis in 5 (11.6%). No statistically significant changes of best corrected visual acuity (BCVA) were found after treatment (p = 0.687). CONCLUSION: Most patients with fungal keratitis have associated risk factors. Filamentous and yeast species have equally prevalent etiologies. In general, our results mirror how difficult and challenging the approach and treatment of fungal keratitis could be.