Repositório RCAAP

Purpose: To describe the imaging features of choroidal nevi using optical coherence tomography angiography (OCT-A) and optical coherence tomography with enhanced depth imaging (EDI-OCT). Material and Methods: Retrospective observational case series of patients with choroidal nevi. The tumor and overlying retina structural features were analyzed with EDI-OCT. The OCT-A images were evaluated for vascular changes at the level of the retinal plexus, outer retina, choriocapillaris and choroid. Statistical analysis were performed using IBM SPSS Statistics. Results and Discussion: 21 patients, 73% female, were included. The mean age was 55±10 years old. The mean tumor thickness was 359±42 µm and the mean largest basal diameter was 3089±261µm. 27% of the nevi had their epicenter at the macula. US showed a solid flat lesion with high reflectivity. With EDI-OCT all lesions appeared as a highly reflective band within the choriocapillaris with posterior shadowing. 67% of the nevi had drusen and 25% had drusenoid pigment epithelium detachment (dPED). Chronic changes in the retina were found in 52% and subretinal fluid in 16%. On OCT-A the superficial and deep capillary plexus were normal. A neovascular membrane was detected in 1 case and a polypoidal vasculopathy in 3 cases. At the level of the choriocapillaris, 16% were hyperreflective, 74% were isorreflective and 10% were hyporreflective. Conclusion: Our work demonstrated that OCT-A may be useful in the diagnosis of vascular complications associated to small stable nevi. The identification of vascularized PEDs is crucial in order to decide treatment, avoiding further visual impairment.

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Purpose: Evaluate the Anterior Segment Swept-source Optical Coherence Tomography (AS-OCT) imaging features of medial rectus with previous surgery and to study the association of this features with the surgical plan. Methods: Evaluation of 43 control eyes (Group A) and 25 eyes submitted to medial rectus surgery (Group B). AS-OCT  scans were obtained on lateral gaze at a fixation light. Muscle insertion(MI) was detected using a graphical user interface(GUI) that identifies the corneo-scleral junction automatically and obtain a automatic muscle insertion to corneo-scleral(ML) distance. All algorithms and GUI were built as a unified tool in MATLAB.A receiver operating curve analysis (ROC) was used to identify cutt-off values from eye with previous muscle recession. Results: In group B, 6 eyes were submitted to resection (23,1%), 5 eyes to faden operation (19,2%) and 6 eyes to recession (23,1%) and 8 eyes to a combination of recession and faden operation (34,6%). In group A, the mean ML distance was 5,12mm [+/-0,47mm]. On recession the mean ML distance was 7,50mm [+/- 1,01mm] and we found a strong correlation between the ML distance and the surgical plane (r=0.77; p<0.01). On faden operation the ML distance was not statistically different from Group A (p=0.88). In our resection cohort we verified that ML distance was statistically different from patients in control group (p < 0.01) and non-different from eyes submitted to recession surgery. Conclusion: AS-OCT evaluation of the ML distance have a strong correlation with the surgical plan and may identify with high sensitivity muscles submitted to surgery.  

Introduction: Phlyctenular keratoconjunctivitis is an uncommon and often misdiagnosed ocular immunological disorder, secondary to ocular or systemic illness, that usually responds well to topical steroids. In a few cases, it can be refractory or steroid dependent, hindering treatment. Case Report: A healthy 12-year-old dark skin female without prior ocular or systemic disease presented in our department with a corneal ulcer and centripetal corneal neovascularization in the left eye and phlyctenules in both eyes, without blepharitis. Testing for different possible inciting agents, such as chlamydia and tuberculosis, was negative. During the follow-up, the patient presented episodes of chalazia and facial pustules and the diagnosis of bilateral phlyctenular keratoconjunctivitis secondary to recurrent ocular rosacea was presumed. As soon as the corneal ulcer was completely healed, the patient started topical corticosteroid treatment with clinical improvement. After several failed attempts of steroids tapering, topical cyclosporine was added to therapy, enabling corticosteroid discontinuation. Discussion: Phlyctenular keratoconjunctivitis is frequently caused by a hypersensitivity reaction to microorganisms, such as staphylococcus species and mycobacterium tuberculosis, but it can be also associated with ocular rosacea without evidence of other inciting agents. The progression of phlyctenulosis to severe complications, as corneal scarring or even perforation, can compromise visual acuity at an early age and for that reason it is important to find and treat any inciting agent for the successful management of this disease. Even with adequate treatment for ocular rosacea with steroids, side effects of long-term therapy to control recurrent ocular inflammation grants topical cyclosporine an important role in disease management.

Abstract Background: Ocriplasmin is a serine protease approved for the treatment of symptomatic vitreomacular traction (VMT). Post-marketing experience has revealed safety concerns. Materials and Methods: Retrospective case report. Clinical and surgical data were analyzed.  Results: We report a case of intraindividual response to bilateral ocriplasmin injection in a 70 year-old female patient with symptomatic vitreomacular traction syndrome. A single surgeon injected the same patient twice, two years appart, following the same protocol. In the first eye we observed the commonest side effects and no VMT release while in the second eye we documented the rarer and severe ocriplasmin’s adverse events nevertheless with successful VMT release. Conclusions: Ocriplasmin’s severe adverse events are unpredictable and underreported. The noticeable difference in adverse events is not accounted by patient, surgeon or tecnique’s variability. This case raises the question of whether the degree of enzymatic cleavage necessary for drug efficacy and VMT release may coexist with more retinal toxicity.

Objetivo: O objectivo deste trabalho é descrever um caso atípico de neuropatia óptica associada ao uso de amiodarona com rápida evolução e com edema da papila unilateral que desenvolveu, posteriormente, edema da papila contralateral. Caso clínico: Doente de 80 anos, medicado com amiodarona há 2 meses, apresentava  diminuição da acuidade visual (AV) bilateral progressiva, mais marcada no olho esquerdo (OE). Apresentava AV do olho direito (OD) de 4/10 e no OE de conta dedos, defeito pupilar aferente relativo e edema da papila do OE. Subsequentemente, verificou-se edema da papila no OD. Associou-se o uso de amiodarona à neuropatia óptica, tendo esta sido suspensa. Actualmente, apresenta quadro estabilizado de perda da AV e dos campos visuais. Discussão: A neuropatia óptica associada a amiodarona é uma situação rara que pode ter implicações graves. Importa excluir a utilização deste fármaco em situações de edema da papila bilateral, mas também em situações unilaterais atípicas.

Objetivo: A Neuropatia Ótica Hereditária de Leber (NOHL) é uma neuropatia ótica resultante da mutação pontual do DNA mitocondrial de herança materna. O objetivo deste trabalho é avaliar as características clínicas e genéticas dos doentes diagnosticados com NOHL no Centro Hospitalar Universitário São João (CHUSJ). Material e Métodos: Análise retrospetiva dos doentes com diagnóstico genético de NOHL seguidos na consulta de Neuroftalmologia do CHUSJ. Resultados: Foram incluídos 11 doentes (22 olhos), a maioria do sexo masculino (63.6%). A idade média, à apresentação, era de 19.0 ± 10.1 anos. A mutação mais frequente foi a G11778A (n= 8, 72.7%). A heteroplasmia foi detetada em dois doentes. A média da melhor acuidade visual corrigida inicial foi de 0.95 ± 0.43 logMAR. O achado mais comum na fundoscopia foi a palidez papilar. O defeito de campo visual mais comum foi escotoma cecocentral. Os exames de neuroimagem não apresentaram alterações na maioria dos doentes. Contudo, foi identificado hipersinal no nervo ótico em dois doentes e alterações da substância branca numa doente. Conclusão: As características clínicas e genética deste estudo estão de acordo com outras populações caucasianas estudadas. Por outro lado, alguns dados do nosso estudo, sugerem que o fenótipo desta patologia pode ser influenciado por fenómenos epigenéticos ainda não completamente compreendidos.

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Purpose: Retinal folds (RF) are an underestimated complication of rhegmatogenous retinal detachment (RRD) surgery. Our aim was to report a case of spontaneous resolution of a full-thickness RF with foveal involvement following an uncomplicated pars plana vitrectomy (PPV) with intraocular gas tamponade for a superior on-macula RRD.   Materials and Methods: Case report.   Results: A 61-year old male underwent PPV with fluid-air exchange and intraocular gas tamponade (SF6) in the left eye for a superior on-macula RRD with vitreous hemorrhage. At the end of surgery, the retina was attached with very marginal subretinal fluid. He assumed face down positioning immediately after surgery, followed by right cheek-to-pillow positioning a few hours later. One week later, the retina remained attached but the patient had developed a mildly symptomatic full-thickness RF with foveal involvement confirmed by optic coherence tomography (OCT). A conservative approach was carried out, with spontaneous progressive anatomical and functional improvement. Two months after surgery, there was only a residual RF on fundus examination and OCT and autofluorescence were unremarkable. The patient was asymptomatic and best corrected visual acuity was 20/25.   Conclusion: Full-thickness macular folds following RRD surgery may be present even after on macula RRD repair. Our case report illustrates that they may have a spontaneous regression with complete functional recovery, in accordance with a few other publications on this topic. As such, conservative management might be indicated even in full-thickness RF if the patient has few complaints and a reasonable visual function.   Keywords: retinal detachment surgery, residual subretinal fluid, intraocular gas tamponade, retinal fold, full-thickness macular fold

INTRODUCTION Leber's Hereditary Optic Neuropathy (LHON) is a maternally inherited disease that manifests as painless bilateral visual loss, usually in early adulthood. Patients with LHON associated with Multiple Sclerosis were mostly female and the association of both pathologies is termed as "Harding's disease". CASE REPORT A 21-year-old woman presented with a typical episode of optic neuritis (ON) in the right eye, which led to Multiple Sclerosis (MS) diagnosis after imagiological and analytical investigation. The episodes of optic neuritis were repeated, with progressive severe visual impairment and the lack of recovery with the treatment for acute attacks, which warned the possibility of LHON. The diagnose was supported by genetic tests by detecting a mutation of the mtDNA 11778 base pair. The patient started oral Idebenone 900mg/day and remained stable for 12 months, with progressive RE visual recovery. Genetic tests for her mother were also applied and the same mutation was identified, which supports the diagnosis of LHON even in her mother, although she always remained without visual symptoms. DISCUSSION Due to the presentation and optic disc findings, LHON is frequently misdiagnosed as ON in the context of MS. However, continuous vision loss in patients with MS during the treatment should raise suspicion for an alternative diagnosis. CONCLUSIONS LHON associated with MS is suggested to be a distinct entity with challenging diagnose and more severe involvement of optic nerve. Our case highlights the importance of genetic tests in individuals who present recurrent ON, with reduced visual recovery during the treatment, even if there is a positive study for MS and no identified family history for LHON. Keywords: Leber's Hereditary Optic Neuropathy, Multiple Sclerosis, Harding's disease, Neuroinflammation, Neurodegeneration

Objetivo: Descrição do caso clínico de uma doente diagnosticada com pseudotumor cerebri que apresentou melhoria após ser submetida a cirurgia bariátrica para obesidade mórbida associada. Caso Clínico: Doente de 42 anos, com obesidade mórbida (IMC de 44.4 kg/m2) e antecedentes de transplante renal, recorre ao Serviço de Urgência por queixas de hipovisão e cefaleias refratárias à terapêutica. A acuidade visual inicial era de 4/10 e 10/10 no olho direito e esquerdo, respetivamente. Foi objetivado defeito pupilar aferente relativo no olho direito, edema da papila bilateral, com neuro-imagem normal e pressão inicial do líquido cefalorraquidiano de 450 mmH2O. Foi medicada inicialmente com acetazolamida 500 mg e topiramato 75 mg e não tolerando doses superiores, pelo que o tratamento médico foi insuficiente. Posteriormente, a paciente foi submetida a sleeve gástrico por laparoscopia.  Um ano e meio após a cirurgia, foi verificada uma perda de 44% do peso corporal e sem novos episódios de cefaleias. O exame oftalmológico demonstrou melhoria da acuidade visual, do papiledema e dos campos visuais. Discussão: A cirurgia bariátrica pode beneficiar de forma significativa os doentes com obesidade mórbida e pseudotumor cerebri.

Purpose: To analyze the refractive, biometric and topographic changes in last year Portuguese medical students exposed to high educational demands. Methods: One-year longitudinal cohort study was performed. Data collected consisted in ocular refraction, axial length (AL), keratometry, corneal thickness, macular thickness and volume. Ophthalmological evaluations were performed 6 months before the students’ final exam, one month after the exam and 6 months later.   Results: Twenty-five medical students were included. The mean spherical equivalent was -1,57 ± 1,29 D at the 1st visit, -1,73 ± 1,44 D in the 2nd visit and -1,40 ± 1,39 D in the 3rd visit. There were no significant differences in the mean spherical equivalent between the 1st and 2nd visits (p=0,055) and between the 1st and 3rd visits (p=0,331). In the 1st, 2nd and 3rd visits, the AL was 23,91 ± 0,99 mm; 24,01 ± 0,98 mm and 23,94 ±1,03 mm, respectively. The AL increased significantly between the 1st and 2nd visits (p<0,001), but the variation between the 1st and 3rd visits was not significant (p=0,414). Corneal thickness and posterior surface corneal keratometry in the steepest axis decreased significantly at the 2nd visit (p<0,05). Macular thickness and volume variation during the follow-up were not statistically significant (p>0,05). Conclusions: This study failed to show statistically significant changes in refraction towards myopia in Portuguese medical students after a long period of near-work; nonetheless the small sample size may have contributed to the lack of statistical significance. The refractive values returned to baseline after 6 months.

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Descrição de nova técnica para a reparação de feridas dos canalículos lacrimais. A técnica cirúrgica descrita, foi usada em  doentes com feridas dos canalículos lacrimais, que recorreram ao serviço de urgência do CHSJ. Pode ser usada em regime de urgência na reparação de feridas dos canalículos, ou em regime de cirurgia programada, na reconstrução de canalículos não reparados, estenosados ou parcialmente obstruídos.

OBJETIVO: Rever as estratégias de estimulação visual (EV) em pacientes com défice visual cerebral (DVC) e criar uma nova aplicação (app) de EV. MATERIAL E MÉTODOS: Foram identificados 394 artigos com os termos “estimulação visual” e “deficiência visual/cortical/cerebral”, através de uma pesquisa na base de dados PubMed®, desde o início até 9 de Janeiro de 2021. Foram selecionados 40 artigos. Em relação ao desenvolvimento da aplicação, foram aplicadas novas estratégias numa app móvel chamada Steve. A plataforma foi desenvolvida na ferra-menta Adobe Phonegap, usando Javascript, HyperText Markup Language e Cascading Style Sheets, sendo compatível com dispositivos iOS, Android e outros dispositivos através de site. A app permite a realização de treinos de estimulação visual, através de animações e formas geométricas com diferentes propósitos. O sistema permite alterar o tamanho e a velocidade do conteúdo, possibilitando uma aprendizagem personalizada. RESULTADOS: O treino de objetivos, programas e métodos individuais de EV deve ser adaptado para cada tipo de DVC. A estimulação com objetos do quotidiano, cores brilhantes e altos contrastes com movimentos suaves são as estratégias de estimulação mais eficazes, que foram inseridas na nossa sugestão de app. CONCLUSÃO: A nossa proposta de aplicação reúne estratégias baseadas em programas previamente existentes, permitindo a estimulação das crianças num ambiente familiar. Acreditamos que este projeto pode auxiliar o processo de reabilitação visual em crianças com DVC, que requer persistência e outras intervenções comportamentais simultâneas.