Spontaneous outer retinal layer recovery in a case of hypertensive choroidopathy secondary to pre-eclampsia: a multimodal evaluation / Recuperação espontânea das camadas externas da retina em um caso de coroidopatia hipertensiva secundária a pré-eclâmpsia: avaliação multimodal

ABSTRACT A 26-year-old woman presented at 28 weeks gestation with hypertensive choroidopathy associated with pre-eclampsia. Fundus photography, fundus autofluorescence, spectral-domain optical coherence tomography (SD-OCT), fluorescein angiography, and indocyanine green angiography were performed in both eyes in the immediate postoperative period. SD-OCT images were obtained before delivery and during a 3-month follow-up. Fundus autofluorescence exhibited patchy hyper- and hypoautofluorescent lesions; fluorescein and indocyanine green angiography revealed areas of choroidal ischemia; and SD-OCT showed disorganization of the outer retinal layers and disruption of the ellipsoid zone. After her blood pressure was stabilized, progressive recovery of the outer retinal layer was monitored on SD-OCT.

RESUMO Uma mulher de 26 anos de idade, com 28 semanas de gestação apresentando coroidopatia hipertensiva associada à pré-eclâmpsia. Retinografia, autofluorescência, tomografia de coerência óptica de domínio espectral, angiofluoresceínografia e angiografia com indocianina verde foram realizadas em ambos os olhos no período pós-operatório imediato do parto. Imagens da tomografia de coerência óptica de domínio espectral foram obtidas antes do parto e durante o seguimento de 3 meses. A autofluorescência apresentou lesões heterogêneas hiper e hipoautofluorescentes, a angiofluoresceínografia e angiografia com indocianina verde revelaram áreas de isquemia de coroide, e a tomografia de coerência óptica de domínio espectral apresentou desorganização das camadas externas da retina e interrupção da zona elipsóide. Após a estabilização da pressão sanguínea, a recuperação progressiva da camada externa da retina foi monitorada pela tomografia de coerência óptica de domínio espectral.

Autofluorescência em um caso de distrofia macular anular concêntrica benigna / Fundus autofluorescence in a case of benign concentric annular macular dystrophy

Resumo A distrofia macular anular concêntrica benigna (DMACB) é uma patologia retiniana rara e provavelmente subdiagnosticada em nosso meio, que se caracteriza por um defeito retiniano em bull's eye sem uso prévio de antimaláricos, associado à preservação relativa da acuidade visual. Devido à escassez de publicações sobre o tema, existem poucos dados referentes aos resultados dos exames complementares nesta patologia. No presente artigo, apresenta-se a descrição da autofluorescência em um caso clássico de DMACB, ainda inédita na literatura, podendo acrescentar achados importantes para auxiliar no diagnóstico e seguimento da doença.

Abstract The benign concentric annular macular dystrophy (BCAMD) is a very rare and probably underdiagnosed eye disease, characterized by a retinal fault in bull's eye pattern, without the association with antimalarial use, but related with good visual acuity. Since there aren't many publications about this condition, is hard to find data regarding the results of complementary examination. In this article, is presented the description of fundus autofluorescence in a classic BCAMD case, yet unpublished, and capable of helping the diagnosis and follow-up of this pathology.

Manifestação ocular incomum em paciente com incontinência pigmentar / Unusual ocular manifestation in a patient with incontinentia pigmenti

RESUMO Incontinência pigmentar, também conhecida como síndrome de Bloch-Sulzberger, é uma doença rara de herança dominante ligada ao X cujas manifestações clínicas incluem lesões dermatológicas típicas combinadas com acometimento neurológico, oftalmológico e dentário. Alterações oculares são comuns e variadas, sendo o acometimento da retina o mais frequente e associado a perda visual severa. Foi relatado um caso de uma criança com perda visual grave decorrente de glaucoma, sem alteração retiniana significativa.

ABSTRACT Incontinentia pigmenti, also known as Bloch-Sulzberger syndrome, is a rare dominant X-linked inheritance disease whose clinical manifestations include typical dermatological lesions combined with neurological, ophthalmic and dental involvement. Ocular involvment is common and diverse and retinal changes are the most frequent and associated with serious visual loss. We reported a case of a child with severe visual loss due to glaucoma, with no significant retinal changes.

Multimodal assessment of patients with chronic central serous chorioretinopathy / Avaliação multimodal em pacientes com coriorretinopatia serosa central crônica

ABSTRACT Purpose: To examine differences between fluorescein angiography (FA) and indocyanine green angiography (ICG) findings in patients with chronic central serous chorioretinopathy (CSC), comparing these with optical coherence tomography (OCT) findings. Methods: Ten consecutive patients with chronic CSC (19 eyes; 7 men; mean age, 50.7 ± 8.4 years) underwent multimodal evaluation that included FA, ICG, and OCT (Spectralis HRA-OCT, Heidelberg Engineering, Heidelberg, Germany). Changes such as hyperfluorescence (caused by increased transmission of the normal choroidal fluorescence, staining, or "pooling") and hypofluorescence (caused by a blockage or vascular filling defect) were evaluated in the early (4 min), middle (4-8 min) and late (>8 min) angiography phases and compared to OCT findings. Results: Bilateral disease was present in nine of the 10 patients. Areas of discontinuation or attenuation of the hyporeflective layer of the retinal pigment epithelium (RPE) on OCT were observed at the same locations as hyperfluorescent angiography window defects on FA and ICG within examination phases. In areas of serous or RPE detachment, the hyperfluorescence pattern was similar on FA and ICG. However, ICG demonstrated areas of hyperfluorescence secondary to choriocapillaris hyperpermeability, with no corresponding change on FA in 12 (70%) of the 19 eyes. This finding was more evident in the middle and late phases of the examinations and there was no evident change in retinal architecture on OCT in these hyperpermeable choroidal regions. Conclusion: In patients with chronic CSC, ICG may reveal choroidal abnormalities that are not evident on FA. This finding may help optimize the monitoring and treatment of CSC.

RESUMO Objetivo: Descrever as diferenças de achados entre a angiofluoresceinografia (FA) e a angiografia digital com indocianina verde (ICG) em pacientes com coriorretinopatia serosa central crônica (CSC), incluindo imagens de tomografia de coerência óptica (OCT). Métodos: Série de casos em que 10 pacientes consecutivos com CSC crônica submetidos à avaliação multimodal, que incluiu FA, ICG e OCT (Spectralis HRA-OCT, Heidelberg Engineering, Heidelberg; Germany). Os pacientes foram avaliados quanto às mudanças como hiperfluorescências (causadas por aumento da transmissão da fluorescência coroidal normal, impregnação ou "pooling") e hipofluorescências (causadas pelo bloqueio ou defeito de enchimento vascular) nas fases precoce (4 minutos), intermediárias (4-8 minutos) e tardias (acima de 8 minutos) da angiografia e comparadas aos achados de OCT. Resultados: Sete dos 10 pacientes (19 olhos) eram homens, média (± DP) de idade dos pacientes foi de 50,7 ± 8,4 anos, e doença bilateral estava presente em nove dos 10 pacientes. Áreas de descontinuação ou atenuação da camada do epitélio pigmentado da retina (EPR), hiporreflectivas no OCT e hiperfluorescência por defeito em janela na FA e ICG ocorreram em locais coincidentes durante as mesmas fases do exame. Em áreas de descolamento seroso ou do EPR, o padrão de hiperfluorescência também foi semelhante em relação à FA e à ICG. No entanto, a ICG demonstrou áreas de hiperfluorescência secundária a hiperpermeabilidade coriocapilar sem mudança correspondente na FA em 12(70%) dos 19 olhos. Este achado da ICG ficou mais evidente nas fases precoces e intermediárias dos exames e não houve mudança evidente na arquitetura da retina no OCT nessas regiões de alteração de hiperpermeabilidade da coroide. Conclusão: Em pacientes com CSC crônica, a ICG pode revelar anormalidades da coróide não evidentes na FA. Esta informação pode ajudar a aperfeiçoar o moni to ramento e tratamento da CSC.

Bietti Crystalline Retinopathy Confirmed by Mutation of CYP4V2 Gene in a Korean Patient

No abstract available.

Pachychoroid neovasculopathy in a male patient: a case report / Espessamento de coroide com neovascularização em paciente do sexo masculino: relato de um caso

ABSTRACT Pachychoroid neovasculopathy is a form of type 1 (subretinal pigment epithelium) neovascularization characterized by the involvement of dilated choroidal vessels in areas of increased choroidal thickness. This disease was originally described in three white female patients. Here we report the multimodal evaluation of a clinical case of PN in a white male patient.

RESUMO O espessamento de coroide com neovascularização é uma forma neovascularização de coroide tipo 1 (sub-epitélio pigmentar retiniano), que ocorre sobre áreas de aumento da espessura da coroide e vasos coroidianos dilatados. Esta doença foi relatada em três pacientes brancas do sexo feminino. Descrevemos um caso clínico da doença com avaliação multimodal em um paciente do sexo masculino.

Comparison of the Clinical Manifestations between Acute Vogt-Koyanagi-Harada Disease and Acute Bilateral Central Serous Chorioretinopathy

PURPOSE: To compare clinical, angiographic, and optical coherence tomographic characteristics between eyes with acute Vogt-Koyanagi-Harada (VKH) disease and eyes with acute bilateral central serous chorioretinopathy (CSC), and to demonstrate distinguishing features between the two diseases in confusing cases. METHODS: The medical records of 35 patients with VKH disease and 25 patients with bilateral CSC were retrospectively reviewed. Characteristics according to slit-lamp biomicroscopy, ophthalmoscopy, fundus photography, fluorescein angiography, indocyanine green angiography, and spectral-domain optical coherence tomography were compared between the two diseases. RESULTS: Five of 35 patients (10 of 70 eyes, 14.3%) with VKH disease were initially misdiagnosed as CSC patients, and six of 25 patients (12 of 50 eyes, 24%) with bilateral CSC were initially misdiagnosed as patients with VKH disease. Pigment epithelial detachment in CSC and optic disc hyperemia in VKH disease show the highest positive predictive values of 100% for each disease. CONCLUSIONS: Optic disc hyperemia in VKH disease and pigment epithelial detachment in bilateral CSC are the most specific clinical manifestations of each disease at initial patient presentation.

A Case of Congenital Simple Hamartoma of the Retinal Pigment Epithelium and Coats' Disease in the Same Eye

No abstract available.

Transient spectral domain optical coherence tomography findings in classic MEWDS: a case report / Alterações transitórias evidenciadas na tomografia de coerência óptica de domíno espectral em quadro clássico de MEWDS: relato de caso

The purpose of this study was to describe a patient with multiple evanescent white dot syndrome (MEWDS) who presented with classic retinal findings and transient changes in outer retinal anatomy. A 20-year-old man presented with mild blurred vision in the left eye, reporting flu-like symptoms 1 week before the visual symptoms started. Fundus examination of the left eye revealed foveal granularity and multiple scattered spots deep to the retina in the posterior pole. Fluorescein angiography and indocyanine green angiography showed typical MEWDS findings. Spectral Domain Optical Coherence Tomography has shown transient changes in outer retinal anatomy with disappearance of inner segment-outer segment junction and mild attenuation of external limiting membrane. Six months later, Spectral Domain Optical Coherence Tomography has shown complete resolution with recovery of normal outer retinal aspect.

O propósito deste estudo é descrever o caso de um paciente com síndrome dos múltiplos pontos brancos evanescentes (MEWDS), apresentando achados retinianos clássicos e alterações transitórias na anatomia retiniana externa. Paciente do sexo masculino, 20 anos de idade, apresentando embaçamento visual no olho esquerdo, relatando sintomas gripais uma semana antes do início dos sintomas visuais. Fundoscopia do olho esquerdo revelou granularidade foveal, múltiplos pontos brancos retinianos no polo posterior. A angiografia fluoresceínica e a indocianinografia verde evidenciaram achados típicos de MEWDS. A tomografia de coerência óptica de domínio espectral evidenciou alterações transitórias na anatomia retiniana externa como desaparecimento da junção dos segmentos interno-externo dos fotorreceptores e leve atenuação da membrana limitante externa. Após 6 meses, a tomografia de coerência óptica mostrou completa resolução com recuperação total da anatomia retiniana externa.

Spontaneously resolving macular cyst in an infant

The purpose of this study is to describe transient macular cysts in an infant and correlate their occurrence with normal development events. A newborn Caucasian girl presented with a protruding corneal mass in her left eye at birth. She underwent a complete ophthalmic examination. A keratinized staphylomatous malformation involving the entire cornea and precluding further visualization of the anterior and posterior segment was observed in the left eye. Spectral domain optical coherence tomography [SD-OCT] of the right eye performed when the child was approximately 6-week-old had revealed an unexpected finding of macular cysts involving the inner nuclear and outer retinal layers. Corneal transplant in the left eye was performed a month later. Ocular examination under anesthesia just prior to surgery revealed normal intraocular pressure, anterior segment and retina in the right eye. SD-OCT was normal in both eyes and showed complete resolution of the cysts in the right eye. The patient had not been on any medications at that time. Although clinical retinal examination might be unremarkable, SD-OCT may reveal cystic spaces in the macula. In the absence of conditions known to be associated with macular edema, transient macular cysts may arise due to a developmental incompetence of the blood-retinal barrier or may represent transient spaces created during normal migration of retinal cells. Further study is warranted to delineate the entity of transient macular cysts in infancy

Spectral-domain Optical Coherence Tomography of Combined Hamartoma of the Retina and Retinal Pigment Epithelium in Neurofibromatosis

A 5-year-old girl was diagnosed with neurofibromatosis type 2 (NF-2) due to multiple neurofibromas, cafe-au-lait spots, and schwannomas of the brain. During ophthalmologic evaluation, a posterior subcapsular cataract and a gray-green colored subretinal lesion were found in right eye. Fluorescein angiography (FA) revealed a combined hamartoma of the retina and retinal pigment epithelium (CHRRPE). At age 9, she underwent cataract surgery. At this time FA and spectral-domain optical coherence tomography (SD-OCT) were taken. The SD-OCT showed an elevated hyperreflective mass in the retina with prominent attenuation of the inner and outer retina, but minimal attenuation in the photoreceptor layers. The underlying retina appeared to be disorganized and thick (791 microm). This is the first case report of SD-OCT imaging of a CHRRPE associated with NF-2 in a pediatric patient. By using SD-OCT in this patient, we could obtain detailed tumor characteristics, and SD-OCT may be helpful in the diagnosis and management of CHRRPE.

Effects of hydroquinone on retinal and vascular cells in vitro.

Aim: To explore the molecular pathophysiology that might explain the epidemiologic association between cigarette smoke and age-related macular degeneration (AMD) by examining the effects of hydroquinone (HQ), a toxic compound present in high concentration in cigarette smoke-related tar, on human retinal pigment epithelial cells (ARPE-19), rat retinal neurosensory cells (R-28), and human microvascular endothelial cells (HMVEC). Materials and Methods: ARPE-19, R-28, and HMVEC were treated for 24 h with four different concentrations of HQ (500 μM, 200 μM, 100 μM, 50 μM). Cell viability, caspase-3/7 activation, DNA laddering patterns, and lactate dehydrogenase (LDH) levels were analyzed. Results: At 50 μM HQ, R-28 cells showed a significant decrease in cell viability compared with the dimethyl sulfoxide (DMSO)-treated controls. At the 100–500 μM concentrations, all three cell lines showed significant cell death (P < 0.001). In the ARPE-19, R-28, and HMVEC cultures, the caspase-3/7 activities were not increased at any of the HQ concentration. Conclusion: Our findings suggest that the mechanism of cell death in all three cell lines was through non-apoptotic pathway. In addition, neuroretinal R-28 cells were more sensitive to HQ than the ARPE-19 and HMVEC cultures.

Time-Lag between Subretinal Fluid and Pigment Epithelial Detachment Reduction after Polypoidal Choroidal Vasculopathy Treatment

PURPOSE: The goal of the present research was to study post-treatment changes in polypoidal choroidal vasculopathy (PCV) shown by optical coherence tomography (OCT). METHODS: The study included 12 patients with naive PCV. Photodynamic therapy and 3 consecutive intravitreal bevacizumab injections at 6-week intervals were given. Best corrected visual acuity, subretinal fluid (SRF), pigment epithelium detachment (PED), central macular thickness (CMT), and total macular volume (TMV) were measured before and after treatment as assessed by Stratus OCT3. RESULTS: After treatment, the SRF height decreased earlier than the PED height. The SRF diameter decreased with statistical significance. However, the PED diameter did not show a statistically significant improvement, persisting at pre-treatment levels. Both CMT and TMV decreased significantly after treatment. CONCLUSIONS: After PCV treatment, SRF and PED stabilized, as shown by OCT. However, the PED treatment response was both delayed and refractory compared to the SRF response. The small change in post-treatment PED diameter may suggest the possibility of PCV recurrence.

Short-Term Clinical Observation of Acute Retinal Pigment Epitheliitis Using Spectral-Domain Optical Coherence Tomography

We investigated the case of a young man with blurred vision in his left eye. His visual acuity was slightly decreased, and ophthalmoscopy disclosed a gray-white lesion in the macula. He had no systemic or ocular history. On the visual field test, the threshold sensitivity was decreased in the corresponding region. Spectral domain optical coherence tomography (OCT) demonstrated a disruption in the photoreceptor inner and outer segment (IS/OS) junction and undulation of the retinal pigment epithelium (RPE) with backscattering. We re-examined the patient after two weeks and after three months without any treatment. Visual acuity and visual field results were gradually normalized, and OCT demonstrated the recovery of continuity in the photoreceptor IS/OS junction, as well as decreased RPE irregularity with minimal backscattering. We used spectral domain OCT instead of time domain OCT (OCT3) so that we could provide better image resolution of the acute retinal pigment epitheliitis (ARPE). Finally, we observed recovery of the functional and anatomical changes in the ARPE patient with a resolution of the condition within three months following the initial examination, using OCT and visual field tests.

Multiple transfused thalassemia major: Ocular manifestations in a hospital-based population.

Purpose: To study the ocular manifestations in multiple transfused beta-thalassemia major patients and assess the ocular side-effects of iron chelating agents. Materials and Methods: In this prospective observational study, 45 multiple transfused beta-thalassemia major children between six months and 21 years of age were enrolled and assigned groups according to the treatment regimens suggested. Group A received only blood transfusions, Group B blood transfusions with subcutaneous desferrioxamine, Group C blood transfusions with desferrioxamine and oral deferriprone and Group D blood transfusions with deferriprone. Ocular status at the time of enrolment was documented. Subjects were observed quarterly for one year for changes in ocular status arising due to the disease process and due to iron chelation therapy. Children with hemoglobinopathies other than beta-thalassemia major, congenital ocular anomalies and anemia due to other causes were excluded. Results: Ocular involvement was observed in 58% of patients. Lenticular opacities were the most common ocular finding (44%), followed by decreased visual acuity (33%). An increased occurrence of ocular changes was observed with increase of serum ferritin and serum iron levels as well as with higher number of blood transfusions received. Desferrioxamine seemed to have a protective influence on retinal pigment epithelium (RPE) mottling. Occurrence of lenticular opacities and RPE degeneration correlated positively with use of desferrioxamine and deferriprone respectively. Follow-up of patients for one year did not reveal any change in ocular status. Conclusion: Regular ocular examinations can aid in preventing, delaying or ameliorating the ocular complications of thalassemia.

Characterization of Peripapillary Atrophy Using Spectral Domain Optical Coherence Tomography

PURPOSE: To characterize the features of peripapillary atrophy (PPA), as imaged by spectral-domain optical coherence tomography (SD-OCT). METHODS: SD-OCT imaging of the optic disc was performed on healthy eyes, eyes suspected of having glaucoma, and eyes diagnosed with glaucoma. From the peripheral beta-zone, the retinal nerve fiber layer (RNFL), the junction of the inner and outer segments (IS/OS) of the photoreceptor layer, and the Bruch's membrane/retinal pigment epithelium complex layer (BRL) were visualized. RESULTS: Nineteen consecutive eyes of 10 subjects were imaged. The RNFL was observed in the PPA beta-zone of all eyes, and no eye showed an IS/OS complex in the beta-zone. The BRL was absent in the beta-zone of two eyes. The BRL was incomplete or showed posterior bowing in the beta-zone of five eyes. CONCLUSIONS: The common findings in the PPA beta-zone were that the RNFL was present, but the photoreceptor layer was absent. Presence of the BRL was variable in the beta-zone areas.

Bilateral macular hole secondary to remote lightning strike.

We report a case of a 16-year-old girl, who was struck by lightning, and experienced blurred vision in the right eye (RE) immediately following the episode. She reported for ophthalmic evaluation two months later. Examination revealed relative afferent pupillary defect in the RE. Posterior subcapsular cataract was noted in both eyes. Fundus examination revealed macular holes and multiple areas of RPE hyperpigmentation in the periphery in both eyes. Fundus fluorescein angiography showed increased choroidal transmission with early fluorescence and late fading in the foveal region and retinal pigment epithelium (RPE) stippling in the periphery in both eyes. This is the first case report of such nature in India to the best of our knowledge.

Intravitreal bevacizumab in pigmented rabbit eyes: histological analysis 90 days after injection / Bevacizumabe intravítreo em olhos de coelhos não albinos: análise histológica 90 dias após a injeção

PURPOSE: To evaluate bevacizumab toxicity in neurosensorial retina and retinal pigment epithelium in pigmented rabbit eyes by means of histological studies. METHODS: Thirty eyes of fifteen rabbits were distributed into three groups: sham group (S), that received a 0.1 ml balanced saline solution (BSS) intravitreal injection (10 eyes); group 1, that received a 1.25 mg (0.1 ml) bevacizumab intravitreal injection (10 eyes); and group 2, that received a 2.5 mg (0.1 ml) bevacizumab intravitreal injection (10 eyes). Rabbits were sacrificed 90 days after the procedure and both eyes of each rabbit were enucleated. A histological examination of neurosensorial retina and retinal pigmented epithelium (RPE) was performed. Its morphological features and layer thickness were also analyzed. RESULTS: No histological differences in neurosensorial retina or in retinal pigmented epithelium were found and layer thickness did not differ significantly between balanced saline solution-injected eyes and bevacizumab-injected eyes. CONCLUSION: After a 90-day follow-up period, a single 1.25 or 2.5 mg bevacizumab intravitreal injection did not lead to toxic damage in the neurosensorial retina and retinal pigment epithelium of pigmented rabbit eyes, and it appears to be a safe procedure for retinal neovascular diseases.

OBJETIVOS: Avaliar a toxicidade do bevacizumabe na retina neurossensorial e epitélio pigmentado da retina (EPR) em olhos de coelhos não albinos pelos estudos histológicos. MÉTODOS: Trinta olhos de 15 coelhos foram distribuídos em três grupos: 10 olhos no grupo placebo (P), que recebeu uma injeção intravítrea de 0,1 ml de solução salina balanceada (SSB); 10 olhos no grupo 1, que recebeu uma injeção intravítrea de 1,25 mg (0,1 ml) de bevacizumabe; e 10 olhos no grupo 2, que recebeu uma injeção intravítrea de 2,5 mg (0,1 ml) de bevacizumabe. Os coelhos tiveram seus dois olhos enucleados sob anestesia geral e submetidos à eutanásia 90 dias após a injeção. Foi realizada avaliação histológica na retina neurossensorial e no epitélio pigmentado da retina e seus aspectos morfológicos e espessuras das camadas retinianas foram analisadas. RESULTADOS: Não foi observada diferença significante entre a morfologia histológica e espessura das camadas retinianas entre o grupo P (SSB) e os grupos 1 e 2 (bevacizumabe 1,25 mg e 2,5 mg, respectivamente). CONCLUSÃO: Após um seguimento de 90 dias, uma única injeção intravítrea de bevacizumabe com 1,25 e 2,5 mg não levou a danos histológicos na retina neurossensorial e epitélio pigmentado da retina em olhos de coelhos não albinos e parece ser um procedimento seguro para o tratamento das doenças neovasculares da retina.