Multimodal imaging in acute idiopathic blind spot enlargement syndrome.

Two cases are reported of acute idiopathic blind spot enlargement syndrome (AIBSE) that presented with peripapillary white spots in the fundus. AIBSE belongs to the acute zonal occult outer retinopathy (AZOOR) complex. Conditions of this AZOOR complex may overlap. Typically, in AIBSE, a peripapillary hyperautofluorescence is seen in the autofluorescence. En-face OCT angiography at the ellipsoid level showed hyper-reflective spots around the optic disk in both cases. One case showed a reversible enlargement of the blind spot in visual field. AZOOR complex is an inflammatory disorder that affects the outer retina, and can now be confirmed with structural optical coherence tomography. In the cases presented, there was a reversible severe loss of the outer retina.

Paracentral acute middle maculopathy in patient with atherosclerosis and angioplasty with stents in the coronary artery.

Sophisticated imaging systems have helped to redefine the clinical presentation of acute macular neuroretinopathy and have markedly enhanced diagnostic sensitivity. The proposed mechanism of paracentral acute middle maculopathy is related to ischemia at the level of the superficial and deep retinal capillary plexi. This is a case report of a patient who developed an acute macular neuroretinopathy after an uneventful angioplasty with stents in the coronary artery.

Ellipsoid layer restoration after Ozurdex® treatment in a patient with acute posterior multifocal placoid pigment epitheliopathy.

An atypical case of acute posterior multifocal placoid pigment epitheliopathy with a clear reappearance of the ellipsoid layer of the retina after Ozurdex® intravitreal implantation is presented. A 51-year-old woman reported a 3-week history of left eye photopsia. On slit-lamp examination, yellowish placoid lesions were found on her left eye fundus. Ancillary tests were performed. The patient was diagnosed as a left eye acute posterior multifocal placoid pigment epitheliopathy, and observational approach was decided. Later, the condition started to progress in an ampiginous manner and a decrease of visual acuity caused by an increase in number and size of the lesions was observed. As the disease was progressing with the conservative, observational approach, and the macula was menaced, an intravitreal dexamethasone implant was injected in the left eye with a consequent improvement of the visual acuity and lesion stabilization. The ellipsoid layer, unidentifiable inside the placoid lesions in previous optical coherence tomography tests, reappeared after the treatment. Intravitreal dexamethasone implants can be used to stabilize acute posterior multifocal placoid pigment epitheliopathy lesions and help resolve the condition. Spectral domain optical coherence tomography can also be useful for monitoring these lesions, as the ellipsoid layer may reappear upon resolution.

Longitudinal Retinal Layer Changes and Clinical Outcome in Patients with Multiple Evanescent White Dot Syndrome.

Purpose: To evaluate longitudinal changes in retinal layer thickness and clinical outcome in patients with MEWDS.Methods: In 20 patients with MEWDS, SD-OCT images and BCVA were assessed at baseline, and at months 1, 3, and 12. SD-OCTs were segmented and measurements were performed within the fovea and a MEWDS lesion. Baseline and follow-up values in the affected eye were compared to measurements performed at the corresponding location in the fellow eye.Results: ONL thickness was 4.7% thicker in MEWDS-eyes compared with the baseline, with a significant decrease of 9% at 3 months. Within the lesion, INL thickness was 7.9% increased at baseline and decreased significantly over the follow-up of 12 months. BCVA was decreased at baseline (0.2 ± 0.18logMAR) and at the 3 months but after 12 months had increased to 0.01 ± 0.04 logMAR.Conclusion: MEWDS shows the involvement of different retinal layers and characteristic changes over the disease course.

Multimodal Imaging Characteristics and Functional Test Findings in a Case of Acute Macular Neuroretinopathy Accompanied by Behçet Disease.

Purpose: To report a case of acute macular neuroretinopathy (AMN) in Behçet Disease.Case: A 23-year-old male presented with a complaint for central scotoma in his right eye. He had been diagnosed with Behçet Disease 3 years ago. Best-corrected visual acuity (BCVA) was 20/20. Anterior chamber and fundus examinations were unremarkable. Optical coherence tomography revealed a paracentral area of outer nuclear layer thinning. Infrared reflectance showed a well-defined, circular, hyporeflective area. Optical coherence tomography angiography revealed an area of capillary dropout in deep retinal capillary plexus corresponding to that hyporeflective lesion. Microperimetry test showed decreased macular sensitivity on the lesion area and the loss of the macular integrity. In multifocal electroretinogram, diminished amplitudes of the central cone responses were detected nasal to fixation.Conclusion: Behçet disease is a cause of occlusive retinal vasculitis. Accompanied retinal microvascular disease may be a possible risk factor of AMN suggesting ischemic etiopathogenesis for AMN.

Multimodal Imaging of Multiple Evanescent White Dot Syndrome: A New Interpretation.

PURPOSE: To investigate the pathogenesis of Multiple Evanescent White Dot Syndrome (MEWDS) using multimodal imaging (MMI). METHODS: Retrospective case series of 7 patients with acute MEWDS. Each patient underwent: near-infrared reflectance (IR), blue and near-infrared autofluorescence (FAF and NIRAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), enhanced depth imaging optical coherence tomography (EDI-OCT) on Spectralis, and optical coherence tomography angiography on OCTA Spectralis, XR Avanti or Plex Elite 9000. RESULTS: OCTA and FA findings of early hyperfluorescence depict an unaffected choriocapillaris. On ICGA early to late hypofluorescent lesions corresponded to the hyporeflectivity on IR, consistent with altered reflectivity of the RPE. The SDI-OCT showed ellipsoid zone disruption as confirmed by FAF hyperautofluorescence. Some lesions showed a hypertransmission sign underneath the RPE, possibly due to changes in RPE intracellular melanin as suggested by NIRAF hypoautofluorescence. CONCLUSIONS: The MMI findings of MEWDS are secondary to RPE reflectivity changes, suggesting its pivotal role.

A CARE-compliant article: a case report of possible association between recurrence of multiple evanescent white dot syndrome and the Herpesviridae family.

RATIONALE: Multiple evanescent white dot syndrome (MEWDS) is a self-limited multifocal chorioretinopathy that typically affects otherwise healthy young females in the second to fourth decades of life. Current understanding of the pathophysiology of MEWDS is still limited. One of the possible underlying causes is an infectious etiology. PATIENT CONCERNS: A 24-year-old female with recurrent episodes of typical MEWDS ocular manifestation was observed over 2 years. Viral-specific antibody serologic tests showed evidence of exposure to the Herpesviridae family during the acute stage of MEWDS in the first and recurrent episodes. DIAGNOSES: MEWDS was diagnosed by the clinical findings and ancillary testing results of fundus photography, optical coherence tomography, fluorescein angiography, indocyanine green angiography and electroretinogram. The laboratory serology data was positive for varicella-zoster virus (VZV) immunoglobulin M (IgM) in the first episode and exhibited high Epstein-Barr virus (EBV) elevated immunoglobulin G (IgG) titer in the recurrent episode. INTERVENTIONS: Due to the self-limited nature of MEWDS, we observed the clinical course without intervention. OUTCOMES: During acute onset of MEWDS, serologic data for VZV IgM antibody was positive in the first episode. Two years later, the patient had recurrent episodes of MEWDS in the contralateral eye. Serologic study showed highly elevated IgG titer (1:160) of Epstein-Barr virus capsid antigen (EB-VCA) in the acute stage. The follow-up paired serum virus serology test showed that the prior EB-VCA IgG titer decreased fourfold to 1:40 in the recovery stage. LESSONS: Recurrence of MEWDS may be associated with acute systemic infection of the Herpesviridae family or virus-induced autoimmune inflammatory reaction.

Acute macular neuroretinopathy (AMN) related to energy drink consumption.

This case identifies a newly found association between energy drinks and acute macular neuroretinopathy (AMN). Our patient, a 34-year-old woman with no significant ocular or previous medical history, presented with a 3-day history of decreased vision after consumption of multiple energy drinks. After near infrared and optical coherence tomography imaging, we were able to diagnose her with AMN. Our patient's vision improved over a 2-month course with no intervention. Our case aims to emphasise the effect of caffeine on the retina, as well as encourage clinicians to consider energy drinks as a causative agent of AMN.

Intravitreal and posterior subtenon triamcinolone acetonide for severe acute posterior multifocal placoid pigment epitheliopathy.

A 21-year-old man presented with visual acuity of 20/200 in both eyes. The fundus picture, fluorescein angiography, and optical coherence tomography revealed severe bilateral acute posterior multifocal placoid pigment epitheliopathy and serous macular detachments. We treated the patient with triamcinolone acetonide, an intravitreal injection (4 mg/0.1 mL) in one eye and a posterior subtenon injection (40 mg/1 mL) in the other eye. Within 2 weeks the visual acuity was 20/80 in both eyes. At the 8-week follow-up visit his vision was 20/63 bilaterally. One year later the vision remained 20/63 in both eyes. In this patient, the triamcinolone acetonide injections, whether administered intravitreally or via the posterior subtenon route, achieved similar anatomic and functional recovery results.

Textural Properties of Choriocapillaris on OCTA in Healed Inflammatory Choriocapillaropathies.

BACKGROUND AND OBJECTIVE: To evaluate the flow characteristics and textural properties of choriocapillaris (CC) on optical coherence tomography angiography in eyes with resolved inflammatory choriocapillaropathies and Vogt-Koyanagi-Harada (VKH) disease. PATIENTS AND METHODS: A cohort of eyes with healed acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous choroiditis (SC), and VKH disease were included. A 3 mm × 3 mm OCT angiogram of CC was acquired and graded for flow characteristics and textural properties. RESULTS: This study included 16 patients. Texture was heterogeneous in all eyes in the SC and VKH groups, and in four eyes (40%) in the APMPPE group. Most of the eyes with VKH disease had severe low flow, whereas most of the SC and APMPPE eyes demonstrated mild low flow. Heal duration had a strong negative correlation with severity of CC low flow and a weak, statistically nonsignificant correlation with texture heterogeneity. CONCLUSION: Despite the resolution of active inflammation, partial CC hypoperfusion and texture disruptions persist for longer durations and may resolve in a time dependent manner. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:566-572.].

Paracentral acute middle maculopathy in patient with atherosclerosis and angioplasty with stents in the coronary artery / Maculopatia média aguda paracentral em paciente com arterioesclerose e angioplastia com stent na artéria coronária

ABSTRACT Sophisticated imaging systems have helped to redefine the clinical presentation of acute macular neuroretinopathy and have markedly enhanced diagnostic sensitivity. The proposed mechanism of paracentral acute middle maculopathy is related to ischemia at the level of the superficial and deep retinal capillary plexi. This is a case report of a patient who developed an acute macular neuroretinopathy after an uneventful angioplasty with stents in the coronary artery.

RESUMO Sistemas de imagem sofisticados ajudaram a redefinir a apresentação clínica da neurorretinopatia macular aguda e têm sensibilidade diagnóstica marcadamente aumentada. A maculopatia média aguda paracentral tem sido relacionada à isquemia ao nível dos plexos capilares superficial e profundo da retina. Este é um relato de caso de paciente que desenvolveu uma neurorretinopatia macular aguda após uma cirurgia de angioplastia com stents da artéria coronária sem complicações.

Intravitreal and posterior subtenon triamcinolone acetonide for severe acute posterior multifocal placoid pigment epitheliopathy / Triancinolona intravítrea e sub-tenoniana para epiteliopatia pigmentar placóide multifocal posterior aguda grave

ABSTRACT A 21-year-old man presented with visual acuity of 20/200 in both eyes. The fundus picture, fluorescein angiography, and optical coherence tomography revealed severe bilateral acute posterior multifocal placoid pigment epitheliopathy and serous macular detachments. We treated the patient with triamcinolone acetonide, an intravitreal injection (4 mg/0.1 mL) in one eye and a posterior subtenon injection (40 mg/1 mL) in the other eye. Within 2 weeks the visual acuity was 20/80 in both eyes. At the 8-week follow-up visit his vision was 20/63 bilaterally. One year later the vision remained 20/63 in both eyes. In this patient, the triamcinolone acetonide injections, whether administered intravitreally or via the posterior subtenon route, achieved similar anatomic and functional recovery results.

RESUMO Um homem de 21 anos apresentou acuidade visual de 20/200 em ambos os olhos. O quadro de fundo de olho, angiofluoresceinografia e a tomografia de coerência óptica revelaram epiteliopatia pigmentar placóide multifocal posterior aguda e descolamento macular seroso. Tratamos o paciente com triancinolona acetonida, uma injeção intravítrea (4 mg/0,1 ml) em um olho e uma injeção subtenoniana posterior (40 mg/1 ml) no outro olho. Após 2 semanas, a acuidade visual foi de 20/80 em ambos os olhos. Na visita de acompanhamento de 8 semanas, sua visão foi de 20/63 bilateralmente. Um ano depois, a visão permaneceu 20/63 em ambos os olhos. Neste paciente, as injeções de triancinolona, administradas por via intravítrea ou por via subtenoniana posterior, obtiveram resultados semelhantes na recuperação anatômica e funcional.