Acute retinal pigment epitheliitis: a case presentation and literature review / Epitelite pigmentar retiniana aguda: apresentação de caso e revisão da literatura

ABSTRACT Acute retinal pigment epitheliitis (ARPE) is an idiopathic, self-limiting inflammatory retinal disorder that particularly affects healthy young individuals. The characteristic fundoscopic appearance of the acute retinal pigment epitheliitis includes a fine pigment stippling surrounded by a yellow-white hypopigmented halos in the macula. Although the exact pathogenesis of the disease remains unknown, some reports have suggested a relationship between a viral infection and acute retinal pigment epitheliitis. Acute retinal pigment epitheliitis is a rare disorder, and only single case reports or case series are found in the literature. The clinical and demographic characteristics of patients with this disease are not fully understood because of its rarity. In this study, we searched the literature to collect clinical and demographic features of the reported cases. We detail the characteristics of acute retinal pigment epitheliitis were pointed and discuss the pathogenesis of the disease.(AU)

RESUMO A epitelite pigmentar retiniana aguda (EPRA) é uma doença inflamatória idiopática e autolimitada da retina, que afeta especialmente indivíduos jovens e saudáveis. À fundoscopia, a aparência característica dessa entidade é de um pontilhado fino do pigmento, cercado de halos hiperpigmentados branco-amarelados na mácula. A patogênese exata da doença ainda é desconhecida, mas alguns relatos apontam uma relação entre epitelite pigmentar retiniana aguda e infecções virais. A epitelite pigmentar retiniana aguda é uma condição rara e na literatura há apenas relatos de casos individuais ou séries de casos. As características clínicas e demográficas da doença não são totalmente compreendidas, devido à sua raridade. Para este relato, foi feita uma busca na literatura para coletar os dados clínicos e demográficos dos casos relatados. Finalmente, são apontadas as características da epitelite pigmentar retiniana aguda e discute-se a patogênese da doença.(AU)

Vitritis and Outer Retinal Abnormalities in a Patient with COVID-19.

PURPOSE: To report a unique case of intraocular inflammation and outer retinal changes in a patient with coronavirus disease (COVID-19). CASE REPORT: A 57-year-old woman was seen 12 days after COVID-19 symptoms onset confirmed by positive IgM and IgG serological tests. No anterior chamber cells were seen. Color fundus photograph showed a yellowish lesion within the macular area, and fluorescein angiography revealed hyperfluorescence on the topography of the macular lesion in both eyes. Spectral-domain optical coherence tomography demonstrated hyperreflective pinpoints at the level of posterior vitreous hyaloid, corresponding to vitritis, hyperreflective lesions at the level of inner plexiform and ganglion cell layers, and disruption of the ellipsoid zone. CONCLUSION AND IMPORTANCE: COVID-19 is known to affect the inner retinal layers. The current case not only supports but also adds a vitreal and an outer retinal layer involvement that might also be caused by this infectious disease.

Outer retinal hyperreflective deposits (ORYD): a new OCT feature in naïve diabetic macular oedema after PPV with ILM peeling.

AIMS: We aimed to investigate a novel optical coherence tomography (OCT) finding of outer retinal hyperreflective deposits (ORYDs) in patients with naïve diabetic macular oedema (DMO) seen after small gauge pars plana vitrectomy (PPV) with internal limiting membrane (ILM) peeling. Furthermore, we evaluated the predictive value of ORYD for visual outcome over 24 months follow-up. METHODS: Retrospective cohort study including 111 eyes from 111 patients with naïve DMO treated by PPV and ILM peeling with a follow-up of 24 months. OCT scans were analysed for the presence of ORYD 1 week and 1, 6, 12, 18 and 24 months after surgery. Change in baseline best-corrected visual acuity (BCVA) and central subfoveal thickness (CST) after surgery were measured over the follow-up period. Presence of ORYD was correlated with baseline characteristics and BCVA after 24 months. RESULTS: Hundred and eleven eyes from 111 patients were included (mean age 67.5±14.8 years). ORYD was identified in the outer plexiform layer as hyperreflective deposits in 92 patients (82.8%) 7 days after surgery but it was not present before surgery. There was a significant reduction in the presence of ORYD. After 24 months ORYD disappeared in all cases (p<0.001). CONCLUSIONS: We describe a novel OCT feature of ORYD present in the early postoperative phase in the majority of patients after PPV with ILM peeling for naïve DMO, disappearing over the postoperative course. These deposits might be a result of sudden desinflammation and could shed new light on the process of DMO resolution after operative intervention.

OPTICAL COHERENCE TOMOGRAPHY ANALYSIS OF OUTER RETINAL TUBULATIONS: Sequential Evolution and Pathophysiological Insights.

PURPOSE: To describe the sequential evolution of outer retinal tubulations (ORTs) in patients diagnosed with choroidal neovascularization and/or retinal pigment epithelium atrophy. METHODS: Retrospective evaluation of spectral domain optical coherence tomography of a consecutive cohort of patients with various retinal conditions. RESULTS: We reviewed the clinical findings of 238 eyes of 119 consecutive patients (54 men and 65 women) with a mean age of 76.2 ± 14.2 years (range: 57-90) and a mean follow-up of 3 ± 1.6 years (range 1-7). Over the follow-up period, ORTs were diagnosed in 67 of 238 eyes (28.1%), 9 of which were imaged with sequential, eye-tracked spectral domain optical coherence tomography dating from the beginning of ORT formation. The presence of geographic atrophy and subretinal hyperreflective material at baseline were found to be risk factors for ORT development (P < 0.001 and P < 0.001, respectively). Outer retinal tubulations were divided into forming versus formed morphologies. The latter was comprised open and closed ORTs of which the open subtype was the most common. The formation of ORTs was significantly associated with microcystic macular lesions in the inner nuclear layer and the downward displacement of the outer plexiform layer, referred to as the outer plexiform layer subsidence sign (P < 0.001). CONCLUSION: Outer retinal tubulation is a frequent optical coherence tomography finding in eyes with choroidal neovascularization and geographic atrophy. Open ORTs with progressive scrolled edges and shortened diameter were significantly associated with microcystic macular lesions in the inner nuclear layer and the outer plexiform layer subsidence sign.

Photoreceptor assessment using adaptive optics in resolved central serous chorioretinopathy.

We present a case of a patient with resolved central serous chorioretinopathy (CSC) in the left eye with persistent mild visual alterations 4 years after the resolution of the disease. Left eye exam revealed a best corrected visual acuity of 20/25 and a slight change of macular pigmentation. Optical coherence tomography revealed only minor irregularities in the topography of retinal pigment epithelium and Bruch's membrane. Adaptive optics (AO) optics demonstrated lower density, spacing, and changes in the photoreceptor mosaic pattern in the left eye than in the right eye, suggesting that CSC may cause damage to cones after clinical recovery. We conclude that AO can provide additional information to assist in the treatment and follow-up of patients with CSC or other macular pathologies.

Photoreceptor assessment using adaptive optics in resolved central serous chorioretinopathy / Avaliação dos fotorreceptores com adaptive optics na coriorretinopatia serosa central cicatrizada

ABSTRACT We present a case of a patient with resolved central serous chorioretinopathy (CSC) in the left eye with persistent mild visual alterations 4 years after the resolution of the disease. Left eye exam revealed a best corrected visual acuity of 20/25 and a slight change of macular pigmentation. Optical coherence tomography revealed only minor irregularities in the topography of retinal pigment epithelium and Bruch's membrane. Adaptive optics (AO) optics demonstrated lower density, spacing, and changes in the photoreceptor mosaic pattern in the left eye than in the right eye, suggesting that CSC may cause damage to cones after clinical recovery. We conclude that AO can provide additional information to assist in the treatment and follow-up of patients with CSC or other macular pathologies.

RESUMO Apresentamos o relato de caso de paciente com coriorretinopatia serosa central (CSC) cicatrizada em olho esquerdo e queixa de discreta alteração visual, mesmo após quatro anos da resolução do quadro. O exame do olho esquerdo apresenta melhor acuidade visual corrigida de 20/25 e discreta alteração de pigmentação macular. Tomografia de coerência óptica (OCT) apresentou apenas pequenas irregularidades em topografia de EPR e Bruch. Foi realizado exame com Adaptive Optics (AO), evidenciando valores inferiores de densidade, espaçamento e alterações no padrão de mosaico dos fotorreceptores em olho esquerdo quando comparado com olho direito, sugerindo que a CSC pode causar danos em cones, mesmo após uma recuperação considerada satisfatória. Concluímos que o AO é uma tecnologia que traz novas informações para auxiliar o tratamento e seguimento dos pacientes com CSC ou outras patologias maculares.

Quantitative analysis of external limiting membrane, ellipsoid zone and interdigitation zone defects in patients with macular holes.

PURPOSE: To investigate the correlation between the length of external limiting membrane (ELM), ellipsoid zone (EZ) and interdigitation zone (IZ) defects and visual prognosis in patients undergoing macular hole (MH) surgery, using spectral-domain optical coherence tomography (SD-OCT). METHODS: This is a retrospective, consecutive, observational case series study. Fifty-two eyes of 52 patients with primary MH were evaluated. A quantitative analysis of ELM, EZ and IZ defects was performed preoperatively and at 3 and 6 months postoperatively using SD-OCT. The correlation between pre- and postoperative ELM, EZ and IZ defects and the best-corrected visual acuity (BCVA) was investigated. RESULTS: The lengths of ELM, EZ and IZ defects correlated significantly with BCVA in each study period (P < 0.001). Preoperative measures of these band defects were also associated with visual outcomes 3 and 6 months after surgery (P < 0.05). Considering all preoperative parameters, the length of the ELM defect was the factor most strongly correlated with BCVA at 6 months (ß = 0.643, P < 0.012). The integrity of the ELM was the only factor significantly associated with BCVA at 6 months (ß = 0.427; P = 0.004). CONCLUSIONS: The preoperative length of the ELM defect is the strongest predictor of visual acuity after MH surgery. Postoperative integrity of the ELM is significantly associated with visual restoration after surgical treatment of MH.

Evaluation of inner segment/outer segment junctions in different types of epiretinal membranes.

PURPOSE:: This study was conducted to evaluate the relationships of inner/outer segment (IS/OS) junction disruption, macular thickness, and epiretinal membrane (ERM) grade with best-corrected visual acuity (BCVA), as well as the relationship between IS/OS junction disruption and ERM grade. METHODS:: Fifty-four eyes of 54 patients with different grades of ERM were retrospectively reviewed. Patients were classified into three groups by ERM grade according to retinal striae and vessel distortion: grade/group 1, visible membranes without retinal striae or vessel distortion; grade/group 2, mild to moderate macular striae or vessel straightening; and grade/group 3, moderate to severe striae and vascular straightening. Correlations of BCVA with age, central retinal thickness, ERM grade, and IS/OS disruption as well as of IS/OS disruption, central macular thickness, and BCVA with ERM grade were evaluated. RESULTS:: Twenty-nine (53.7%) eyes exhibited IS/OS junction disruption. Groups 1 and 2 differed significantly with respect to BCVA (p=0.038), but groups 2 and 3 did not (p=0.070). Central macular thickness was significantly greater in group 2 than in group 1 (p=0.031) and in group 3 than in group 2 (p=0.033). Groups 1 and 2 differed significantly in terms of IS/OS disruption (p=0.000), but groups 2 and 3 did not (p=0.310). CONCLUSIONS:: The IS/OS junction appears to be disrupted during the early stages of ERM. Grade 3 ERM is associated with a significantly higher incidence of IS/OS disruption.

Evaluation of inner segment/outer segment junctions in different types of epiretinal membranes / Avaliação da junção segmento interno/segmento externo em diferentes graus de membranas epirretinianas

ABSTRACT Purpose: This study was conducted to evaluate the relationships of inner/outer segment (IS/OS) junction disruption, macular thickness, and epiretinal membrane (ERM) grade with best-corrected visual acuity (BCVA), as well as the relationship between IS/OS junction disruption and ERM grade. Methods: Fifty-four eyes of 54 patients with different grades of ERM were retrospectively reviewed. Patients were classified into three groups by ERM grade according to retinal striae and vessel distortion: grade/group 1, visible membranes without retinal striae or vessel distortion; grade/group 2, mild to moderate macular striae or vessel straightening; and grade/group 3, moderate to severe striae and vascular straightening. Correlations of BCVA with age, central retinal thickness, ERM grade, and IS/OS disruption as well as of IS/OS disruption, central macular thickness, and BCVA with ERM grade were evaluated. Results: Twenty-nine (53.7%) eyes exhibited IS/OS junction disruption. Groups 1 and 2 differed significantly with respect to BCVA (p=0.038), but groups 2 and 3 did not (p=0.070). Central macular thickness was significantly greater in group 2 than in group 1 (p=0.031) and in group 3 than in group 2 (p=0.033). Groups 1 and 2 differed significantly in terms of IS/OS disruption (p=0.000), but groups 2 and 3 did not (p=0.310). Conclusions: The IS/OS junction appears to be disrupted during the early stages of ERM. Grade 3 ERM is associated with a significantly higher incidence of IS/OS disruption.

RESUMO Objetivo: Este estudo foi realizado para avaliar a relação entre a interrupção da junção segmento interno/segmento externo (IS/OS), espessura macular e grau de membrana epirretiniana (ERM), com a melhor acuidade visual corrigida (BCVA), e a relação entre a interrupção da junção IS/OS com a severidade da ERM. Métodos: Cinquenta e quatro olhos de 54 pacientes com diferentes graus de ERM foram avaliados retrospectivamente. ERMs foram classificadas, de acordo com as estrias de retina e a distorção dos vasos, em 3 grupos: grupo 1 foram membranas visíveis sem estrias retinianas ou distorção dos vasos, grupo 2 membranas com estrias maculares discretas a moderadas ou retificação dos vasos, e grupo 3 membranas com estrias moderadas a graves e retificação vascular. A correlação da BCVA com a idade, espessura central da retina, severidade da ERM e interrupção da junção IS/OS foram avaliadas. A relação de interrupção da junção IS/OS, a espessura macular central e acuidade visual com a severidade da ERM também foram avaliadas. Resultados: Vinte e nove olhos (53,7%) apresentavam interrupção da junção IS/OS. A BCVA foi diferente entre ERMs grupo 1 e grupo 2 (p=0,038), a diferença entre o grupos 2 e 3 não foi estatisticamente significativa (p=0,070). A espessura macular central foi estatisticamente maior no grupo 2, quando comparado ao grupo 1 (p=0,031) e maior no grupo 3 quando comparado ao grupo 2 (p=0,033). A diferença entre o grupo 1 e grupo 2 em relação à interrupção da junção IS/OS foi estatisticamente significativa (p=0,000), ao passo que a diferença entre o grupo 2 e do grupo 3 não foi estatisticamente significativa (p=0,310). Conclusões: As junções IS/OS parecem estar interrompidas nos estágios iniciais da ERM. O grau 3 de ERM têm uma maior incidência significativa de interrupção da junção IS/OS.

Inner and outer segment junction (IS/OS line) integrity in ocular Behçet's disease.

PURPOSE: In this study, we examined the spectral domain optical coherence tomography (OCT) findings of ocular Behçet's disease (OB) in patients with inactive uveitis. Specifically, we analyzed the inner and outer segment junction (IS/OS line) integrity and the effect of disturbed IS/OS line integrity on visual acuity. METHODS: Patient files and OCT images of OB patients who had been followed-up between January and June of the year 2013 at the Dicle University Eye Clinic were evaluated retrospectively. Sixty-six eyes of 39 patients were included the study. RESULTS: OCT examination of the patients with inactive OB revealed that approximately 25% of the patients had disturbed IS/OS and external limiting membrane (EML) line integrity, lower visual acuity (VA), and lower macular thickness than others. Linear regression analysis revealed that macular thickness was not an independent variable for VA. In contrast, the IS/OS line integrity was an independent variable for VA in inactive OB patients. CONCLUSION: In this study, we showed that the IS/OS line integrity was an independent variable for VA in inactive OB patients. Further prospective studies are needed to evaluate the integrity of the IS/OS line in OB patients.

Inner and outer segment junction (IS/OS line) integrity in ocular Behçet's disease / Integridade da junção do segmento ınterno e externo (linha IS/OS) na doença de Behçet ocular

Purpose: In this study, we examined the spectral domain optical coherence tomography (OCT) findings of ocular Behçet's disease (OB) in patients with inactive uveitis. Specifically, we analyzed the inner and outer segment junction (IS/OS line) integrity and the effect of disturbed IS/OS line integrity on visual acuity. Methods: Patient files and OCT images of OB patients who had been followed-up between January and June of the year 2013 at the Dicle University Eye Clinic were evaluated retrospectively. Sixty-six eyes of 39 patients were included the study. Results: OCT examination of the patients with inactive OB revealed that approximately 25% of the patients had disturbed IS/OS and external limiting membrane (EML) line integrity, lower visual acuity (VA), and lower macular thickness than others. Linear regression analysis revealed that macular thickness was not an independent variable for VA. In contrast, the IS/OS line integrity was an independent variable for VA in inactive OB patients. Conclusion: In this study, we showed that the IS/OS line integrity was an independent variable for VA in inactive OB patients. Further prospective studies are needed to evaluate the integrity of the IS/OS line in OB patients. .

Objetivo: Neste estudo, examinamos os achados da tomografia de coerência óptica (OCT) de domínio espectral na doença de Behçet ocular (OB) em pacientes com uveíte inativa. Especificamente, analisamos a integridade da junção dos segmentos interno e externo (linha IS/OS) e o efeito da alteração da integridade da linha IS/OS na acuidade visual. Métodos: Avaliamos retrospectivamente os prontuários e as imagens de OCT dos pacientes com OB examinados entre janeiro e junho de 2013 na Dicle University Eye Clinic. Sessenta e seis olhos de 39 pacientes foram incluídos no estudo. Resultados: Análise das imagens de OCT dos pacientes com OB inativa revelou que aproximadamente 25% dos pacientes tinham alterações da integridade da linha IS/OS e da membrana limitante externa (ELM), e apresentavam acuidade visual (VA) e espessura macular menor do que os outros. A análise de regressão linear revelou que a espessura macular não é uma variável independente para VA. Por outro lado, a integridade da linha IS/OS foi uma variável independente para VA em pacientes com OB inativa. Conclusão: Neste estudo, nós mostramos que a integridade da linha IS/OS foi uma variável independente para a VA em pacientes com OB inativa. Precisamos de mais estudos prospectivos para avaliar a integridade da linha IS/OS em pacientes com OB. .

Outer retina analysis by optical coherence tomography in cone-rod dystrophy patients.

PURPOSE: To analyze the outer retinal layers using spectral domain optical coherence tomography (SD-OCT) in patients with cone-rod dystrophy. METHODS: The diagnosis of cone-rod dystrophy was determined by primary cone involvement or concomitant loss of both cones and rods. Electroretinography showed implicit time shift at 30-Hz flicker response and prevalent decrease of photopic over scotopic responses. Using SD-OCT, the outer retina was retrospectively evaluated in 24 eyes of 12 patients with cone-rod dystrophy. From the innermost to the outermost, the four studied hyperreflective outer retinal bands were labeled Band 1, the external limiting membrane; Band 2, the ellipsoid zone; Band 3, the interdigitation zone between the cone outer segments and the apical processes of the retinal pigment epithelium; and Band 4, the retinal pigment epithelium complex. RESULTS: The mean age of study patients was 30 years, and the median visual acuity was 20/30. A ring maculopathy appearance involving the fovea area was observed in all study eyes. There was an absence of interdigitation zone in the entire length of SD-OCT scan, including the foveal area, in all 24 study eyes. Outside the foveal area, the external limiting membrane and ellipsoid zone were intact in all study eyes. The intensity of the ellipsoid zone was decreased in the entire length of SD-OCT scan in all study eyes. Within the foveal area, there was loss of the external limiting membrane and ellipsoid zone in 20 (83%) and 22 eyes (92%), respectively. The retinal pigment epithelium complex was identified in all study eyes. None of the study eyes revealed cystoid macular edema. CONCLUSION: SD-OCT scans demonstrated complete absence of the interdigitation zone in patients with cone-rod dystrophy. Consistent with the known histology of animal models of cone dystrophy, this finding may represent abnormal outer retinal morphology, including an absence of the outer segments themselves or a defective or absent interdigitation between the apical processes of the retinal pigment epithelium with the cone outer segments.