Prevalência de miopia em crianças de escolas públicas do Sul do Brasil / Prevalence of myopia among children from public schools in Southern Brazil

RESUMO Objetivo: Avaliar a prevalência de miopia em crianças de escolas públicas da Região Metropolitana de Porto Alegre (RS, Brasil). Métodos: Estudo transversal prospectivo, com amostra de 330 estudantes de escolas públicas da Região Metropolitana de Porto Alegre entre 5 e 20 anos de idade. Os escolares foram submetidos à avaliação oftalmológica completa, incluindo acuidade visual com e sem correção, autorrefração dinâmica e estática, refração subjetiva dinâmica e estática sob cicloplegia e medida do diâmetro axial. Um questionário sobre tempo de uso de telas diário foi aplicado. Os desfechos foram prevalência de miopia, alta miopia e baixa miopia. O teste do qui-quadrado de Pearson foi utilizado para avaliar a relação da prevalência com a variável contemplada no questionário. Resultados: A prevalência de miopia foi de 17,4% (IC95% 13,8-21,7%). Baixa e alta miopia corresponderam a 15,2% (IC95% 11,9-19,3%) e 2,1% (IC95% 1,1-4,1%), respectivamente. Conclusão: Essa é a maior prevalência de miopia sob cicloplegia encontrada no Brasil até a presente data. Outros estudos para entender a prevalência e a evolução da ametropia no país são necessários.

ABSTRACT Objective: To assess myopia prevalence in children from public schools of the metropolitan region of Porto Alegre, in Rio Grande do Sul. Methods: It is a prospective cross-sectional study with a sample of 330 children from public schools of the metropolitan region of Porto Alegre, from 5 to 20 years old. The students were submitted to an ophthalmological evaluation including auto-refractor measurements, best corrected and uncorrected visual acuity, subjective refraction under cycloplegia and axial length. The outcomes were prevalence of myopia, high myopia, and low myopia. Pearson's chi-squared test was used to assess the relationship between prevalence and the variable contemplated in the questionnaire. Results: The prevalence of myopia was 17.4% (CI 13.8 - 21.7%). Low and high myopia corresponded to 15.2% (CI 11.9 - 19.3%) and 2.1% (CI 1.1 - 4.1%), respectively. Conclusion: This is the highest prevalence of myopia under cycloplegia found in Brazil to date. Other studies are necessary to understand the prevalence and evolution of the condition in the country.

Autologous serum eye drops diluted with 0.5% methylcellulose and 0.9% saline solution: a clinical comparative study / Colírio de soro autólogo diluído com metilcelulose 0,5% e solução salina 0,9%: um estudo clínico comparativo

ABSTRACT Purpose: This clinical study compared autologous serum eye drops diluted with 0.5% methylcellulose and 0.9% saline solution. The subjective criteria for symptom improvement and the objective clinical criteria for response to therapy were evaluated. Methods: This longitudinal prospective study enrolled 23 patients (42 eyes) with persistent epithelial defects or severe dry eye disease refractory to conventional therapy who had been using autologous serum 20% prepared with methylcellulose for > 6 months and started on autologous serum diluted in 0.9% saline solution. The control and intervention groups consisted of the same patients under alternate treatments. The subjective criteria for symptom relief were evaluated using the Salisbury Eye Evaluation Questionnaire. The objective clinical criteria were evaluated through a slit-lamp examination of the ocular surface, tear breakup time, corneal fluorescein staining, Schirmer's test, rose Bengal test, and tear meniscus height. These criteria were evaluated before the diluent was changed and after 30, 90, and 180 days. Results: In total, 42 eyes were analyzed before and after 6 months using autologous serum diluted with 0.9% saline. No significant differences were found in the subjective criteria, tear breakup time, tear meniscus, corneal fluorescein staining, or rose Bengal test. Schirmer's test scores significantly worsened at 30 and 90 days (p=0.008). No complications or adverse effects were observed. Conclusions: This study reinforces the use of autologous serum 20% as a successful treatment for severe dry eye disease resistant to conventional therapy. Autologous serum in 0.9% saline was not inferior to the methylcellulose formulation and is much more cost-effective.

RESUMO Objetivo: Este estudo comparou o colírio de soro au tólogo manipulado com metilcelulose a 0,5% com solução salina 0,9%. Critérios subjetivos de melhora dos sintomas e critérios clínicos objetivos para resposta à terapia foram avaliados. Métodos: Este estudo prospectivo longitudinal envolveu 23 pacientes (42 olhos) com defeitos epiteliais persistentes ou doença de olho seco grave refratária à terapia convencional que usavam colírio de soro autólogo 20% preparado com metilcelulose por mais de 6 meses e iniciaram soro autólogo diluído em solução salina 0,9%. Os grupos controle e intervenção consistiam dos mesmos pacientes sob tratamentos alternados. Os critérios subjetivos para o alívio dos sintomas foram avaliados usando o Salisbury Eye Evaluation Questionnaire. Os critérios objetivos foram avaliados por meio de exame em lâmpada de fenda incluindo: tempo de ruptura da lágrima, coloração da córnea com fluoresceína, teste de Schirmer, coloração com rosa bengala e altura do menisco lacrimal. Esses critérios foram avaliados antes da troca do diluente e após 30, 90 e 180 dias. Resultados: Um total de 42 olhos foram analisados antes e após 6 meses usando soro autólogo diluído com solução salina 0,9%. Nenhuma diferença significativa foi encontrada nos critérios subjetivos, tempo de ruptura da lágrima, menisco lacrimal, coloração com fluoresceína ou rosa bengala. Os resultados dos testes de Schirmer pioraram significativamente em 30 e 90 dias (p=0,008). Não foram observadas complicações ou efeitos adversos. Conclusões: Este estudo reforça o uso do colírio de soro autólogo 20% como um tratamento de sucesso para a doença do olho seco grave resistente à terapia convencional. O soro autólogo diluído em solução salina a 0,9% não foi inferior à formulação de metilcelulose.

Eye fungal infections: a mini review.

Ocular fungal infections annually affect more than one million individuals worldwide. The management of these infections is problematic, mainly due to the limited availability of effective antifungal agents. Thus, ocular infections are increasingly recognized as important causes of morbidity and blindness, especially keratitis and endophthalmitis. Thus, this review aims to demonstrate the importance of fungal eye infections through the description of the main related aspects, with emphasis on the treatment of these infections. For this purpose, a search for scientific articles was conducted in databases, such as Medline, published from 2000 onwards, addressing important aspects involving fungal eye infections. In addition, this work highlighted the limited therapeutic arsenal available and the severity associated with these infections. Thus, highlighting the importance of constantly updating knowledge about these pathologies, as it contributes to agility in choosing the available and most appropriate therapeutic alternatives, aiming at positive and minimally harmful results for that particular patient.

Cornea opacity, uveitis with iris atrophy and lens damage following cosmetic high-intensity ultrasound of the eyelid: a case report.

BACKGROUND: High-intensity focused ultrasound (HIFU) is a cosmetic procedure that aims to tone the skin through thermal collagen coagulation. The energy is delivered in the deep layers of the skin, and because of these characteristics, the risks of severe damage to adjacent tissue and the ocular surface may be underestimated. Previous reports have demonstrated superficial corneal opacities, cataracts, increased intraocular pressure, or ocular refractive changes in different patients following HIFU. In this case, we report deep stromal opacities associated with anterior uveitis, iris atrophy and lens opacity formation following a single HIFU superior eyelid application. CASE PRESENTATION: A 47-year-old female presented to the ophthalmic emergency department complaining of pain, hyperemia and photophobia in the right eye following a HIFU application to the superior right eyelid. A slit lamp examination showed three temporal-inferior corneal infiltrates with edema and severe anterior uveitis. The patient was treated with topical corticosteroids, and six months later, there was residual corneal opacity, iris atrophy and peripherical cataract formation. No surgical procedure was needed, and the final vision was Snellen 20/20 (1.0). CONCLUSION: The risk of severe impairment to the ocular surface and ocular tissues may be underestimated. Cosmetic surgeons and ophthalmologists must be aware of the complications, and the long-term follow-up of these changes needs further investigation and discussion. Safety protocols of the HIFU intensity threshold for thermal lesions in the eye and the use of protective eye devices should be better evaluated.

Conjunctival melanoma following cornea transplant from a cancer donor: A case report.

Purpose: Conjunctival melanoma is a rare ocular tumor. We report a case of ocular conjunctival melanoma during topical immunosuppression, after a corneal transplant from a donor with metastatic melanoma. Observation: A 59-year-old white male presented with a progressive nonpigmented conjunctival lesion in his right eye. He had previously undergone two penetrating keratoplasties, and he was being treated with topical immunosuppression with 0.03% tacrolimus (Ophthalmos Pharma; Sao Paulo, SP/Brazil). The histopathology evaluation revealed the nodule to be a conjunctival epithelioid melanoma. The donor's death cause was disseminated melanoma. Conclusion and importance: The correlation between cancer and systemic immunosuppression after a solid organ transplant is widely known. The local influence, however, has not been reported. In this case, a causal relationship was not established. The correlation between conjunctival melanoma, exposure to topical tacrolimus immunosuppressive therapy, and the malignance characteristic of donor cornea should be better evaluated.

Autologous fibrin glue versus sutures for conjunctival autograft in primary pterygium: a randomized clinical trial.

PURPOSE: To describe and compare the autologous fibrin glue and traditional sutures for conjunctival graft attachment in patients undergoing primary pterygium excision surgery. METHOD: A randomized clinical trial included patients who underwent pterygium surgery with conjunctival autologous graft (CAG). Using randomization, a single-trained surgeon performed graft fixation with autologous glue or sutures. The glue was prepared immediately before the surgery, using the patient's blood components. After centrifugation, the plasma was collected and enhanced with calcium gluconate. Postoperative assessments were performed on Days 1, 7, 21, 30 and 180. The study evaluated postoperative edema and pain and complications. The mean surgical time was compared. Recurrence of the pterygium was assessed 6 months postoperatively. RESULTS: The study evaluated 61 eyes. Thirty-three eyes underwent pterygium surgery using the glue technique, and 28 underwent the traditional suture technique. Fifty-one patients (83.60%) had successful graft adhered to at the end of follow-up. Ten patients (10/33) lost their graft in the glue group, and only 69.70% maintained graft presence in the fourth week versus 100% of the patients in the suture group (p = 0.001). Pain scores were lower in the glue group, and clinical edema was significantly higher. There was no significant difference in graft retraction or the presence of granuloma, necrosis or postoperative infection. CONCLUSION: Autologous fibrin glue is an affordable option for CAG fixation in particular contexts where commercial fibrin glue is unavailable, and it could offer advantages compared to sutures. A higher graft loss rate was observed and might be related to topical anesthesia, and improvements to the technique are needed. CLINICALTRIALS: gov Identifier: NCT04151017. https://clinicaltrials.gov/ct2/show/NCT04151017?term=20170467&draw=1&rank=1 .

Clinical and molecular diagnosis of Acanthamoeba keratitis in contact lens wearers in southern Brazil reveals the presence of an endosymbiont.

Acanthamoeba keratitis (AK) is an infection that is mostly observed in contact lens wearers. It is often misdiagnosed causing delays in the administration of the correct treatment. The aim of this study was to report the outcome of clinical and molecular diagnosis of AK cases during the summer of 2019 in the southern region of Brazil. Three suspected cases of AK were discovered after an ophthalmic examination at a public hospital in the city of Porto Alegre. These cases were then confirmed through laboratory diagnosis (cell culture and molecular analysis by PCR and sequencing). In each of the three clinical sample cell cultures of corneal scraping and molecular analysis confirmed the presence of Acanthamoeba spp., all belonging to the morphological group II and to the genotype T4, which is the most common genotype associated with AK. In addition, Acanthamoeba spp. isolated from one of the clinical samples was found to harbor the Candidatus Paracaedibacter acanthamoeba, a bacterial endosymbiont. The presence of Ca. Paracaedibacter acanthamoeba in clinical isolates requires further research to reveal its possible role in the pathogenicity of Acanthamoeba infections.

The influence of luminous intensity on the eyelid aperture and measurement of the margin reflex distance.

PURPOSE: To evaluate whether the flashlight feature of smartphones can be used to replace the traditional flashlight for measuring MRD and investigate the relation between increasing light intensities and MRD measurements. METHODS: A prospective clinical study was conducted with 40 individuals from Hospital de Clínicas de Porto Alegre. Outcome measures: MRD1, MRD2, and palpebral fissure height (PFH) were analyzed by photographic record using different intensity light sources. RESULTS: Flashlight (10 lux): MRD1 mean: 3.97 mm ±1.16; PFH mean: 9.87 mm ±1.53; Smartphone (100 lux) MRD1 mean: 4.02 mm ±1.17; and PFH mean: 9.62 mm ± 1.45 (p > .05). Using a dimmable source of light resulted in a mean reduction of the PFH of 0.75 mm with the highest light intensity (1200 lux). There was no statistically significant association between MRD changes and the iris color, age, and gender of the subjects. CONCLUSIONS: There was no statistically significant difference between the MRD measurements using traditional flashlights compared to higher intensity smartphone flashlight. Using a dimmable source of light, there is a statistically significant reduction in palpebral fissure with higher light intensity, which occurs mostly by upper eyelid lowering, probably due to orbicularis oculi muscle contraction. A smartphone with a built-in flashlight can be used to replace the traditional flashlight in clinical practice without prejudice to the evaluation of the MRD.

Antifungal Efficacy and Safety of Cycloheximide as a Supplement in Optisol-GS.

PURPOSE: The incidence of fungal infection after corneal transplant has increased significantly in recent years, especially Candida spp. This study aimed to evaluate the efficacy and safety of the addition of cycloheximide in Optisol-GS media in decreasing the growth of Candida spp. strains. METHODS: This in vitro laboratory efficacy study measured fungal colony growth in 24 vials of Optisol-GS that were divided into 6 groups of 4 vials each, as follows: (1) MIC/2 cycloheximide, (2) MIC cycloheximide, (3) MICx5 cycloheximide, (4) MICx10 cycloheximide, from MIC values obtained for each strain, (5) unsupplemented optisol-GS as a positive control (added inoculum), and (6) unsupplemented optisol-GS as a negative control (no inoculum). In each group was added Candida albicans, C. glabrata and C. parapsilosis, except in the negative control. The evaluated variables were fungal colony growth from the Optisol-GS vials, corneal endothelial cell density and endothelial cell viability at different concentrations of cycloheximide. RESULTS: In the efficacy study, all strains showed a reduction in fungal cell growth from the second day at all evaluated concentrations of optisol-GS supplemented with cycloheximide, even at subinhibitory concentrations (MIC/2). For C. glabrata, the colony count was reduced to 99%. No evidence of corneal endothelial toxicity was found at any concentration, in the safety study, compared with the paired control. CONCLUSION: The addition of cycloheximide to optisol-GS decreased the fungal growth, demonstrating fungicide action against C. glabrata and fungistatic action against C. albicans and C. parapsilosis. This drug did not demonstrate toxicity to the corneal endothelium at different concentrations.

Ocular manifestations in classic homocystinuria.

BACKGROUND: Classic homocystinuria (HCU), or cystathionine beta-synthase (CBS) deficiency, is a rare inborn error of methionine metabolism. Main clinical features may include skeletal and vascular manifestations, developmental delay, intellectual disability and eye disorders. MATERIAL AND METHODS: This is an observational and retrospective study aiming at describing eye abnormalities presented by a cohort of late-diagnosed HCU patients. Data regarding ophthalmological evaluation included visual acuity, refraction, biomicroscopy, Perkins tonometry, fundus examination, retinography, biometry, ocular ultrasound, optical coherence tomography, anterior segment photography and topography. RESULTS: Ten patients with HCU (20 eyes) were included. The most frequent findings were ectopia lentis(n = 20) and myopia (n = 9). Biometry, ultrasound, OCT and topography findings were available for four patients. One patient had keratoconus; one had abnormal retinal pigmentation; and two had lens surgery scars with irregular astigmatism. CONCLUSIONS: Eye abnormalities are very frequent in late-diagnosed HCU patients. The presence of ectopia lentis should always raise the diagnostic hypothesis of HCU.

Keratoconus progression after intrastromal corneal ring segment implantation according to age: 5-year follow-up cohort study.

BACKGROUND/AIMS: Intrastromal corneal ring segments (ICRS) have been found to be useful in correcting keratoconus by decreasing irregular astigmatism, thereby potentially improving visual acuity. However, its long-term effects in keratoconus progression are not completely understood, mainly concerning the effects of age on ICRS implantation results. This study aimed to evaluate long-term effects of ICRS implantation according to age at implantation. METHODS: We conducted a longitudinal retrospective study, where we evaluated patients with keratoconus who underwent ICRS implantation between 2004 and 2012. RESULTS: We evaluated 34 eyes for 5 years post-operatively. The mean age of the 21 men and 7 women was 20.59 ± 4.65 years. Best spectacle-corrected visual acuity (BSCVA) improved from 0.32 ± 0.19 in the preoperative period to 0.46 ± 0.27 6 months post-operatively. After up to 5 years of follow-up, there was no significant difference in mean visual acuity of the group. As expected, there was a significant decrease in keratometric values after corneal ring implantation, which remained stable over the 5-year follow-up. Among the 34 cases analysed, nine (26%) showed signs of disease progression. In eight of the nine documented progression cases, patients were 21 years old or younger, revealing that these patients were sevenfold more likely to progress than those aged over 21 years. CONCLUSION: In our series of cases, ICRS implantation was shown to be an excellent treatment to reduce corneal curvature and improve visual acuity at all ages, but it did not stabilize the disease, especially in young patients with more aggressive forms of the keratoconus.

Long-term results of accelerated and conventional corneal cross-linking.

PURPOSE: The aim of this study is to compare the long-term follow-up outcomes of traditional and accelerated corneal cross-linking (CXL) for keratoconus. METHODS: This retrospective comparative cohort study included patients with keratoconus who underwent corneal cross-linking (CXL) between August 2008 and December 2016. Patients treated before August 2013 underwent the traditional Dresden protocol, and those treated subsequently received the accelerated protocol, i.e., 0.1% riboflavin soaking for 10 min, followed by pulsed UV-A irradiation at 30 mW/cm2 for 8 min, and a total irradiation power of 7.2 J/cm2. The primary outcome was the success rate in halting the disease progression. The postoperative changes in visual acuity, keratometry readings, endothelial cell count and complications following accelerated CXL and conventional CXL were compared. RESULTS: This study evaluated 113 eyes over a mean follow-up period of 37.61 ± 16.34 months. It was found that 89.6% and 95.7% of the eyes were successfully treated with the traditional and accelerated protocols, respectively (p = 0.239). The corrected-distance visual acuity improved in both groups, but it was significantly better following the accelerated protocol (p = 0.037). Traditional protocol group had more postoperative complications than the accelerated group (8.9% versus 2%), although not reaching statistically significant difference (p = 0.142). There were no significant differences in the topographic (p = 0.122) and refractive (p = 0.570) outcomes. CONCLUSION: Accelerated CXL and conventional CXL are both safe and effective, and the long-term follow-up showed that the accelerated protocol was similar to the standard one in terms of halting keratoconus and achieving visual outcomes. Because of its potential benefits, the accelerated protocol may be considered for the treatment of progressive keratoconus.

Progressive eye pathology in mucopolysaccharidosis type I mice and effects of enzyme replacement therapy.

BACKGROUND: Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disorder caused by α-L-iduronidase deficiency, resulting in accumulation of glycosaminoglycans (GAG). Ophthalmological manifestations are common in MPS I patients and often lead to visual impairment. Accumulation of GAG in corneal or retinal tissues reduces vision causing corneal opacity and neurosensory complications. One available treatment for MPS I patients is enzyme replacement therapy (ERT), but the results of such treatment on eye disease are still debatable. Therefore, we aimed to determine the progression of ocular manifestations as well as the effectiveness of intravenous ERT in MPS I. METHODS: Corneal and retinal analyses were perform in eyes from 2- to 8-month normal and MPS I mice. Some MPS I mice received ERT (1.2 mg/kg of laronidase) every 2 weeks from 6 to 8 months and histological findings were compared with controls. Additionally, cornea from two MPS I patients under ERT were evaluated. RESULTS: Mouse corneal tissues had GAG accumulation early in life. In the retina, we found a progressive loss of photoreceptor cells, starting at 6 months. ERT did not improve or stabilize the histological abnormalities. MPS I patients, despite being on ERT for over a decade, presented GAG accumulation in the cornea, corneal thickening, visual loss and needed corneal transplantation. CONCLUSION: We provide data on the time course of ocular alteration in MPS I mice. Our results also suggest that ERT is not effective in treating the progressive ocular manifestations in MPS I mice and fails to prevent corneal abnormalities in patients.

Recommendations for the management of MPS IVA: systematic evidence- and consensus-based guidance.

INTRODUCTION: Mucopolysaccharidosis (MPS) IVA or Morquio A syndrome is an autosomal recessive lysosomal storage disorder (LSD) caused by deficiency of the N-acetylgalactosamine-6-sulfatase (GALNS) enzyme, which impairs lysosomal degradation of keratan sulphate and chondroitin-6-sulphate. The multiple clinical manifestations of MPS IVA present numerous challenges for management and necessitate the need for individualised treatment. Although treatment guidelines are available, the methodology used to develop this guidance has come under increased scrutiny. This programme was conducted to provide evidence-based, expert-agreed recommendations to optimise management of MPS IVA. METHODS: Twenty six international healthcare professionals across multiple disciplines, with expertise in managing MPS IVA, and three patient advocates formed the Steering Committee (SC) and contributed to the development of this guidance. Representatives from six Patient Advocacy Groups (PAGs) were interviewed to gain insights on patient perspectives. A modified-Delphi methodology was used to demonstrate consensus among a wider group of healthcare professionals with experience managing patients with MPS IVA and the manuscript was evaluated against the validated Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument by three independent reviewers. RESULTS: A total of 87 guidance statements were developed covering five domains: (1) general management principles; (2) recommended routine monitoring and assessments; (3) disease-modifying interventions (enzyme replacement therapy [ERT] and haematopoietic stem cell transplantation [HSCT]); (4) interventions to support respiratory and sleep disorders; (5) anaesthetics and surgical interventions (including spinal, limb, ophthalmic, cardio-thoracic and ear-nose-throat [ENT] surgeries). Consensus was reached on all statements after two rounds of voting. The overall guideline AGREE II assessment score obtained for the development of the guidance was 5.3/7 (where 1 represents the lowest quality and 7 represents the highest quality of guidance). CONCLUSION: This manuscript provides evidence- and consensus-based recommendations for the management of patients with MPS IVA and is for use by healthcare professionals that manage the holistic care of patients with the intention to improve clinical- and patient-reported outcomes and enhance patient quality of life. It is recognised that the guidance provided represents a point in time and further research is required to address current knowledge and evidence gaps.

Fusarium solani keratitis: role of antifungal susceptibility testing and identification to the species level for proper management

ABSTRACT We report a patient with fungal keratitis caused by a multiresistant Fusarium solani in a tertiary care hospital located in southern Brazil. A 55-year-old man with a history of ocular trauma presented with keratitis in left eye. The patient has a complicated clinical course and failed to respond to local and systemic antifungal treatment, and required eye enucleation. Despite multiple topical, intraocular and systemic antifungal treatments, hyphal infiltration persisted in the corneal transplant causing continuous recurrences. The cultures of corneal biopsy scrapings were positive for Fusarium spp. The organism was identified to species level by multi-locus sequencing for translation elongation factor 1 alpha (EF-1α), and RNA polymerase II subunit (RPB2). In vitro antifungal susceptibility testing of the isolate by the broth microdilution method, according to CLSI M38-A2, disclosed susceptibility to natamycin and resistance to amphotericin B, voriconazole, itraconazole and fluconazole. Considering previous unsuccessful antifungal treatments due to multiple drug resistance, the eye was enucleated. Our case report illustrates that management of fungal keratitis remains a therapeutic challenge. Optimal treatment for F. solani infection has not yet been established and should include susceptibility testing for different antifungal agents.

Recommendations for the management of MPS VI: systematic evidence- and consensus-based guidance.

INTRODUCTION: Mucopolysaccharidosis (MPS) VI or Maroteaux-Lamy syndrome (253200) is an autosomal recessive lysosomal storage disorder caused by deficiency in N-acetylgalactosamine-4-sulfatase (arylsulfatase B). The heterogeneity and progressive nature of MPS VI necessitates a multidisciplinary team approach and there is a need for robust guidance to achieve optimal management. This programme was convened to develop evidence-based, expert-agreed recommendations for the general principles of management, routine monitoring requirements and the use of medical and surgical interventions in patients with MPS VI. METHODS: 26 international healthcare professionals from various disciplines, all with expertise in managing MPS VI, and three patient advocates formed the Steering Committee group (SC) and contributed to the development of this guidance. Members from six Patient Advocacy Groups (PAGs) acted as advisors and attended interviews to ensure representation of the patient perspective. A modified-Delphi methodology was used to demonstrate consensus among a wider group of healthcare professionals with expertise and experience managing patients with MPS VI and the manuscript has been evaluated against the validated Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument by three independent reviewers. RESULTS: A total of 93 guidance statements were developed covering five domains: (1) general management principles; (2) recommended routine monitoring and assessments; (3) enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT); (4) interventions to support respiratory and sleep disorders; (5) anaesthetics and surgical interventions. Consensus was reached on all statements after two rounds of voting. The greatest challenges faced by patients as relayed by consultation with PAGs were deficits in endurance, dexterity, hearing, vision and respiratory function. The overall guideline AGREE II assessment score obtained for the development of the guidance was 5.3/7 (where 1 represents the lowest quality and 7 represents the highest quality of guidance). CONCLUSION: This manuscript provides evidence- and consensus-based recommendations for the management of patients with MPS VI and is for use by healthcare professionals that manage the holistic care of patients with the intention to improve clinical- and patient-reported outcomes and enhance patient quality of life. It is recognised that the guidance provided represents a point in time and further research is required to address current knowledge and evidence gaps.

Fusarium solani keratitis: role of antifungal susceptibility testing and identification to the species level for proper management.

We report a patient with fungal keratitis caused by a multiresistant Fusarium solani in a tertiary care hospital located in southern Brazil. A 55-year-old man with a history of ocular trauma presented with keratitis in left eye. The patient has a complicated clinical course and failed to respond to local and systemic antifungal treatment, and required eye enucleation. Despite multiple topical, intraocular and systemic antifungal treatments, hyphal infiltration persisted in the corneal transplant causing continuous recurrences. The cultures of corneal biopsy scrapings were positive for Fusarium spp. The organism was identified to species level by multi-locus sequencing for translation elongation factor 1 alpha (EF-1α), and RNA polymerase II subunit (RPB2). In vitro antifungal susceptibility testing of the isolate by the broth microdilution method, according to CLSI M38-A2, disclosed susceptibility to natamycin and resistance to amphotericin B, voriconazole, itraconazole and fluconazole. Considering previous unsuccessful antifungal treatments due to multiple drug resistance, the eye was enucleated. Our case report illustrates that management of fungal keratitis remains a therapeutic challenge. Optimal treatment for F. solani infection has not yet been established and should include susceptibility testing for different antifungal agents.

Posterior keratoconus and iris atrophy: a fortuitous association? / Ceratocone posterior e atrofia de iris: uma associação incidental?

ABSTRACT The authors describe an unusual association between posterior keratoconus and iris atrophy, confirmed by a complete ocular evaluation, scheimpflug imaging and pachymetric curve. A hypothesis for concomitant findings is discussed.

RESUMO Os autores descrevem a rara associação entre ceratocone posterior e atrofia de íris, confirmada por avaliação oftalmológica completa, imagens de scheimpflug e curva paquimétrica. Sugere-se uma hipótese que explique a concomitância de ambas as alterações.

Posterior keratoconus and iris atrophy: a fortuitous association?

The authors describe an unusual association between posterior keratoconus and iris atrophy, confirmed by a complete ocular evaluation, scheimpflug imaging and pachymetric curve. A hypothesis for concomitant findings is discussed.

Effects of sub-Tenon's triamcinolone injections in patients with uveitis / Efeitos da injeção subtenoniana de triancionolona em pacientes com uveítes

ABSTRACT Purpose: To evaluate the response to sub-Tenon's triamcinolone injection in patients with uveitis. Methods: We studied 28 eyes with macular edema associated with controlled uveitis. We administered sub-Tenon's injection of triamcinolone and followed the patients for 180 days to analyze the positive effects (improvement of macular edema and visual acuity) and monitor the possible adverse effects. This prospective study was conducted at the Department of Ophthalmology, Hospital de Clínicas de Porto Alegre, Brazil. Results: We observed improvement in macular edema in 86% of patients. The mean central macular thickness at each time point of assessment was 432.22, 298.80, 286.37, 267.49, 253.87, and 253.49 mm at baseline (before sub-Tenon's injection of triamcinolone), 15 days after the procedure, at 30 days, at 60 days, at 90 days, and at 180 days, respectively. The mean reduction in retinal thickness was 30.8%, 33.7%, 38.11%, 41.2%, and 41.35% at 15, 30, 60, 90, and 180 days of follow-up, respectively. Visual acuity also improved in 85.7% of patients, with a mean improvement of 1.36, 1.93, 2.23, 2.26, and 2.30 lines gained on the Early Treatment Diabetic Retinopathy Study chart at 15, 30, 60, 90, and 180 days of follow-up, respectively. No statistically significant increases in intraocular pressure and conjunctival abnormalities were caused by the procedure, and no other adverse effects were observed. Overall, the results of this study were similar to those described in the literature. Conclusions: Sub-Tenon's injection of triamcinolone provides reduced macular thickness and improvement in visual acuity with no significant adverse effects and is therefore an effective and safe procedure for the treatment of sequelae of uveitis.

RESUMO Objetivos: Avaliar os efeitos da injeção subtenoniana de triancinolona em pacientes com uveítes. Métodos: Foram incluídos na avaliação 28 olhos com edema macular associado à uveíte. Esses pacientes foram submetidos à injeção subtenoniana de triancinolona e acompanhados ao longo de 180 dias, para analisar os efeitos em relação à melhora do edema macular, da acuidade visual e acompanhamento de possíveis efeitos adversos. Trata-se de um estudo prospectivo, realizado no Serviço de Oftalmologia do Hospital de Clínicas de Porto Alegre. Resultados: Foi verificada melhora do edema macular em 86% dos pacientes, sendo uma redução média da espessura retiniana de 30,8% aos 15 dias, 33,7% aos 30 dias, 38,11% aos 60 dias, 41,2% aos 90 dias e 41,35% aos 180 dias de seguimento. Também foi observado melhora da acuidade visual em 85,7% dos pacientes e ganho de linhas na tabela de acuidade visual, sendo 1,36 linhas aos 15 dias de seguimento, 1,93 linhas aos 30 dias, 2,23 linhas aos 60 dias, 2,26 linhas aos 90 dias e 2,30 linhas aos 180 dias. Não houve significância estatística em relação ao aumento da pressão intraocular e às alterações conjuntivais causadas pelo procedimento, sem detecção de qualquer outro efeito colateral. Foi concluído que os resultados encontrados nesse estudo são similares aos descritos na literatura. Conclusões: A injeção subtenoniana de triancinolona é um procedimento eficaz e seguro para o tratamento das sequelas por quadros de uveítes, proporcionando redução da espessura macular e melhora da acuidade visual, sem relação com efeitos adversos significativos.