Quantitative Analysis of Ellipsoid Zone in Acute Posterior Multifocal Placoid Pigment Epitheliopathy
Ontology highlight
ABSTRACT:
SUBMITTER: Browne A
PROVIDER: S-EPMC8171299 | biostudies-literature | 2020 Feb
REPOSITORIES: biostudies-literature
Similar Datasets
Project description:PurposeTo reassess the underlying pathophysiology of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and relentless placoid chorioretinitis (RPC) through comparison with the non-inoculated eye of the von Szily animal model of neurotropic viral retinal infection.MethodsNarrative review.ResultsLiterature reports of isolated neurotropic viral entities and rising serological viral titers in APMPPE after presentation support a potential direct infective etiology. In general, viral transport along axons results in mitochondrial stasis and disruption of axoplasmic flow. Clinical manifestations of axoplasmic flow disruption in APMPPE/RPC may signify the passage of virus along the neuronal pathway. From a case series of 11 patients, we demonstrate a timely, spatial, and proportional association of optic disc swelling with APMPPE lesion occurrence. Signs within the inner retina appear to precede outer retinal lesions; and acute areas of outer nuclear layer (ONL) hyperreflectivity appear to be the result of coalescence of multiple hyperreflective foci resembling axonal spheroids (which occur as a consequence of axoplasmic disruption) and follow the Henle fiber layer neurons. Underlying areas of retinal pigment epithelium (RPE) hyper-autofluorescence follow ONL hyperreflectivity and may signify localized infection. Areas of apparent choriocapillaris hypoperfusion mirror areas of RPE/Bruch's membrane separation and appear secondary to tractional forces above. Increases in choroidal thickness with lesion occurrence and focal areas of choriocapillaris hypoperfusion are observed in both APMPPE/RPC and the von Szily model.ConclusionsThe neurotrophic infection model provides significant advantages over the existing primary choriocapillaris ischemia hypothesis to account for the range of imaging signs observed in APMPPE and RPC.
| S-EPMC11182168 | biostudies-literature
Project description:PurposeTo report an atypical case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with central nervous system (CNS) vasculitis and recurrent strokes.ObservationsA 57 year-old female presented with APMPPE after a febrile illness and rash. She developed an acute infarct on magnetic resonance imaging. Computed tomography angiography of the cerebral vasculature was normal. Cerebrospinal fluid (CSF) analysis and an extensive serum lab workup were also unremarkable. She was treated with high-dose corticosteroids and eventually transitioned to methotrexate. A month after being on treatment she developed a second stroke. A cerebral angiogram was obtained and did not show evidence of CNS vasculitis. The methotrexate was eventually stopped and the prednisone was tapered. Approximately 3 months later she developed a third stroke and worsening APMPPE-associated maculopathy in both eyes. She was eventually started on oral cyclophosphamide.Conclusions & importanceAlthough rare, CNS vasculitis is a known complication of APMPPE. This case is atypical given the development of multiple recurrent strokes, lack of inflammatory evidence on CSF analysis, and normal imaging of the cerebral vasculature. This report highlights the need for a high level of clinical suspicion for CNS vasculitis with APMPPE despite noncontributory cerebral angiographic imaging and normal CSF analysis.
| S-EPMC6872853 | biostudies-literature
Project description:The purpose of this study was to evaluate potential insights into the pathogenesis of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) using multimodal diagnostic imaging and laboratory evaluation in long-term follow-up. A retrospective, single-center case series was conducted on seven consecutive patients (14 eyes) who were given a diagnosis of APMPPE from March 1, 2011, through June 30, 2019 with at least three months of follow-up. Clinical characteristics (age, symptoms, visual acuity [VA]), laboratory testing including coxsackievirus titers, and multimodal imaging from fundus photography, spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), fluorescein angiography (FA), and indocyanine green angiography (ICG) were analyzed for each patient. The initial median VA was 20/71 and final median VA was 20/22. Coxsackievirus B (CVB) titers were elevated (≥ 1:80) in six of seven patients, with a four-fold increase in convalescent titers seen in two patients suggestive of recent infection. All patients were treated with oral corticosteroids, and five patients underwent corticosteroid-sparing immunomodulatory therapy. Initially, multifocal deep choroidal lesions were observed in the posterior pole corresponding to patches of hypocyanescence on ICG. Overlying retinal pigment epithelium (RPE) disease was observed on FAF, although this finding was not universally observed, suggesting that RPE disease may occur as a sequelae to unchecked choroidal inflammation. SD-OCT architectural changes confirmed outer retina and ellipsoid zone disruption. FA of active lesions showed early hypofluorescence and late hyperfluorescence with surrounding leakage while inactive disease showed areas of staining. Long-term follow-up of multimodal diagnostic imaging in APMPPE revealed that choroidal inflammation likely precedes RPE change and photoreceptor damage. Elevation of coxsackievirus titers with seroconversion may be associated with an infectious trigger in concert with immune-mediated disease in this posterior uveitis syndrome.
| S-EPMC7446910 | biostudies-literature
Project description:BackgroundAcute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare inflammatory eye disorder that is characterized by the presence of multiple placoid lesions in the posterior pole of the eye. Relentless placoid chorioretinitis (RPC) is an inflammatory chorioretinopathy that combines clinical features of APMPPE and serpiginous chorioretinitis, which is a progressive condition with a high risk of visual disability. Patients with COVID-19 can develop various ocular manifestations, however, there have been limited reports of APMPPE and RPC associated with the infection. We report a case of a patient who developed APMPPE after a COVID-19 infection and subsequently progressed into RPC.Case presentationA 17-year-old male presented with a one-week history of painless gradual visual loss in both eyes. Two months prior to the visual symptoms, the patient had a SARS CoV-2 infection, confirmed by polymerase chain reaction test. Clinical findings with fundoscopy, optical coherence tomography and fluorescein angiography were consistent with APMPPE. Due to the severely affected vision in both eyes, the patient was started on 50 mg oral prednisolone daily, after which vision began to improve rapidly. Two months after symptom onset during steroid taper, the impression of continued inflammatory activity and new lesions in the retinal periphery of both eyes suggested RPC. Adalimumab 40 mg every other week was initiated with 12.5 mg prednisolone daily followed by slow tapering. Vision improved and five months after the start of the adalimumab treatment, the steroid was discontinued and there were no signs of active inflammation. The patient has been followed for a total of 21 months since presentation, had full visual recovery and good tolerance of the immunosuppressive treatment.ConclusionCOVID-19 might cause long-lasting activity of APMPPE. The scarcity of reports compared with the number of confirmed COVID-19 infections worldwide suggests a rare entity. The association of APMPPE with a variety of infections may suggest a common immunological aberrant response that might be triggered by various factors. Further examinations and case reports are needed to understand the role of biological therapy in the treatment of such cases.
| S-EPMC10766895 | biostudies-literature
Project description:PurposeTo report a case of acute syphilitic posterior placoid chorioretinopathy (ASPPC) that demonstrated partial resolution with immunosuppressive therapy secondary to a misdiagnosis as Behçet's disease followed by a relapse which was successfully treated with the appropriate treatment.ObservationsA 34-year-old female patient presented to our service with complaints of decreased vision in the left eye (OS). She initially developed similar symptoms seven months prior to presentation and was diagnosed as Behçet's disease based on the clinical picture of papillitis, vasculitis and placoid chorioretinitis in the posterior pole of OS. She was started on daily oral prednisone 60 mg and weekly methotrexate 10mg by her rheumatologist. The patient's ocular symptoms improved one month prior to presentation with resolution of the placoid lesion but persistence of vasculitis and papillitis. At that time, the dose of the prednisone was decreased to 30 mg which resulted in a relapse of the placoid chorioretinal lesions and worsened visual acuity at the time of presentation to us. Extensive laboratory workup demonstrated positive serology for syphilis. A diagnosis of syphilitic placoid chorioretinitis was made and the patient was treated with intravenous penicillin G for 2 weeks. The vitritis, papillitis, and placoid chorioretinitis resolved along with improvement in vision following the treatment.Conclusions and importanceOcular findings in syphilis are heterogeneous and may mimic variety of ocular diseases. ASPPC is a rare ocular manifestation of syphilis and its natural course and underlying pathophysiology is not well understood. However, irrespective of the underlying mechanism of the disease, all patients with ASPPC should receive treatment to prevent recurrence and long-term functional damage.
| S-EPMC6423698 | biostudies-literature
Project description:PurposeTo investigate the natural course of pachychoroid pigment epitheliopathy (PPE).DesignA retrospective cohort study.SubjectsFrom the Kyoto central serous chorioretinopathy (CSC) cohort consisting of 548 patients with CSC as of September 2020, we included consecutive unilateral patients with acute or chronic CSC between January 2013 and December 2016.MethodsAll patients underwent complete ophthalmic examination, including multimodal imaging such as fundus autofluorescence, spectral-domain optical coherence tomography, and fluorescein angiography/indocyanine green angiography and/or optimal coherence tomography angiography. The fellow eyes of eyes diagnosed with CSC were screened for PPE, and their natural course was evaluated. We also evaluated the association of ARMS2 rs10490924, CFH rs800292, TNFRSF10A rs13278062, and GATA5 rs6061548 genotypes with the natural course.Main outcome measuresIncidence of CSC, pachychoroid neovasculopathy, and pachychoroid geographic atrophy (GA).ResultsIn total, 165 patients with unilateral CSC (mean age, 55.7 ± 12.6 years; female, 22.4%) were included from the Kyoto CSC cohort. Among them, 148 (89.7%) were diagnosed as having PPE in their non-CSC eye. Survival analysis revealed that 16.8% of PPE eyes developed CSC during the 6-year follow up, whereas non-PPE eyes did not. Although genetic factors did not have significant association with CSC development (P > 0.05, log-rank test), choroidal vascular hyperpermeability (CVH) and subfoveal choroidal thickness (SFCT) were significantly associated with CSC incidence (P = 0.001, log-rank test). Survival analysis showed that eyes without CVH and eyes with SFCT < 300 μm did not develop CSC during the 6-year follow-up. Pachychoroid neovasculopathy developed in only 1 eye with PPE during a follow-up of 46.4 months. Pachychoroid GA did not develop in any of the studied eyes.ConclusionsThis study revealed a natural history of PPE in a relatively large Japanese cohort. Choroidal vascular hyperpermeability and SFCT were significant risk factors for the development of CSC in PPE eyes. Although the current results cannot be generalized for all eyes with PPE, these findings present an important clinical implication.
| S-EPMC9754975 | biostudies-literature
Project description:BackgroundTo quantitatively assess outer retinal layers in eyes with hydroxychloroquine (HCQ) toxicity.MethodsA retrospective case-control study was performed to identify eyes with HCQ retinopathy/toxicity at Cleveland Clinic. A clinical diagnosis of HCQ retinopathy was made based on clinical and imaging features including the presence of parafoveal ellipsoid zone (EZ) loss on spectral-domain optical coherence tomography (OCT) and visual field defects. All participants underwent macular cube scan using the Cirrus HD-OCT (Zeiss, Oberkochen, Germany). Quantitative assessment of outer nuclear layer (ONL)/Henle fibre layer complex (HFL) metrics and EZ mapping were performed with a novel software platform and compared with age-matched controls. HCQ toxicity group was divided into three subgroups based on the severity.ResultsThere were 14 eyes from 14 patients in HCQ toxicity group (mean age 57.0±18.6 years), and 14 eyes from 14 subjects in age-matched control group (mean age 59.4±18.6 years). Multiple outer retinal parameters including ONL/HFL-EZ volume, parafoveal ONL/HFL-EZ thickness and EZ-retinal pigment epithelium (RPE) volume were significantly reduced in all HCQ toxicity subgroups (early, moderate and advanced toxicity) compared with controls. Semiautomated layer segmentation tool produced en face representation of EZ-RPE mapping and allowed unique visualisation of EZ attenuation in eyes with HCQ toxicity. The longitudinal analysis of HCQ toxicity group demonstrated progressive decline in some outer retinal parameters.ConclusionHCQ toxicity resulted in significant outer retinal layer volumetric thinning compared with controls. Quantitative assessment of outer retinal parameters and EZ mapping on SD-OCT may become a useful biomarker to identify and monitor HCQ toxicity.
| S-EPMC6397800 | biostudies-literature
Project description:PurposeThe purpose of this study was to define structure-function correlation of geographic atrophy (GA) on optical coherence tomography (OCT) and functional testing on microperimetry (MP) based on deep-learning (DL)-quantified spectral-domain OCT (SD-OCT) biomarkers.MethodsPatients with GA were prospectively examined by SD-OCT (Spectralis, 97 B-scans) and two microperimetry devices (MP3 and MAIA) in two combined test runs each. DL-algorithms measured the ellipsoid-zone thickness (EZT), ellipsoid-zone loss (EZL), hyper-reflective-foci (HRF) volume, drusen-volume (DV), and retinal-pigment-epithelium loss (RPEL) area. Pointwise co-registration was established between all stimuli and the location on OCT. A multivariable mixed-effect model with variable selection was used to identify pointwise retinal sensitivity (PWS) changes for each biomarker, accounting for age and eccentricity.ResultsThree thousand six hundred stimuli points were collected and correlated with 1940 OCT B-scans in 20 eyes of 20 patients. PWS was significantly lower in stimuli with EZL without RPEL (-2.81, 95% confidence interval [CI] = -3.72 to -1.91 decibel [dB], 0 degrees, P < 0.0001) and in areas with both EZL and RPEL (-10.03, 95% CI = -10.96 to -9.11 dB, 0 degrees, P < 0.0001) compared to areas without any atrophy. Increased EZT had a significant positive effect on PWS (0.34, 95% CI = 0.32 to 0.36 dB/µm, P < 0.0001). Structure-function correlations were consistent throughout all levels of eccentricity with P < 0.001. Drusen and HRF volume, but not age, were associated with reduced PWS.ConclusionsFunctional impairment by MP was associated with defined morphological changes as quantified by DL on OCT. PR degeneration seen as EZL alone impairs the function on MP examinations. The combination of DL-based SD-OCT biomarker assessment and MP appear suited for evaluation of retinal function beyond visual acuity for disease monitoring.
| S-EPMC11918028 | biostudies-literature
Project description:PurposeTo assess longitudinally the effect of anti-vascular endothelial growth factor (VEGF) treatment on ellipsoid zone (EZ) integrity, subretinal hyperreflective material (SHRM), and the sub-retinal pigment epithelium (sub-RPE) compartment in eyes with neovascular age-related macular degeneration (nAMD).DesignPost hoc analysis of the OSPREY clinical trial, a prospective, double-masked, phase 2 study comparing brolucizumab 6 mg with aflibercept 2 mg over 56 weeks.ParticipantsParticipants with treatment-naïve nAMD at the initiation of the trial were included in the analysis.MethodsEyes were evaluated with spectral-domain OCT at 4-week intervals in the OSPREY trial (n = 81). Spectral-domain OCT scans collected from each visit were segmented automatically using a proprietary, machine learning-enabled higher-order feature-extraction platform for retinal layer, SHRM, and sub-RPE boundary lines, which were evaluated and corrected as needed by masked trained graders. The current analysis focused only on patients evaluated with the Cirrus (Zeiss) platform (n = 28).Main outcome measuresOutcome measures included change from baseline in EZ-RPE (i.e., photoreceptor outer segment) volume, EZ-RPE central subfield thickness (CST), total EZ attenuation, SHRM volume, SHRM CST, and total sub-RPE volume. The correlation between each of these measures and best-corrected visual acuity (BCVA) at each visit was evaluated.ResultsEZ-RPE volume and EZ-RPE CST showed significant increases, and total EZ attenuation, SHRM volume, SHRM CST, and total sub-RPE volume showed significant decreases from baseline at each visit from weeks 4 through 56 (P < 0.05 at each visit). Ellipsoid zone integrity measures and SHRM volume correlated significantly with BCVA at most visits (P < 0.05). No significant correlation was found between total sub-RPE volume and BCVA.ConclusionsEZ integrity, SHRM, and sub-RPE disease features in eyes with nAMD showed improvement as early as week 4 of anti-VEGF treatment. EZ integrity measures and SHRM volume were predictors of visual acuity over the first year of treatment.
| S-EPMC8387490 | biostudies-literature
Project description:PurposeThe relative ellipsoid zone reflectivity (rEZR) has been proposed as an innovative biomarker for photoreceptor integrity. This study evaluates the rEZR in macular telangiectasia type 2 (MacTel) eyes of different disease stages.MethodsThe mean rEZR (ratio ellipsoid zone [EZ]/external limiting membrane [ELM] reflectivity [arbitrary units {AUs}], grey level range = 0-1) was analyzed for an entire spectral domain optical coherence tomography volume scan (global) and for each subfield of the Early Treatment Diabetic Retinopathy Study (ETDRS) grid (topographic) in patients with MacTel and controls. MacTel disease severity was classified according to Gass and Blodi.ResultsLinear mixed-model analysis of 145 eyes of 74 patients and 50 eyes of 25 controls revealed globally lower, yet not statistically significant, rEZR values in MacTel eyes. Topographically, most pronounced decreases were found in stages 3 and 4/5 for the temporal inner (coefficient estimates [CEs] = -25.4 [-38.2; -12.6] and -34.1 [-48.7; -19.6] AU, both: P < 0.001), the inferior inner (-29.9 [-44.6; -15.6] and -35.3 [-52.1; -18.5] AU, both: P < 0.001), the nasal inner (-21.5 [-35.52; -7.4] and -31.6 [-47.6; -15.6] AU, P = 0,003 and P < 0.001), and in the superior inner subfield of stage 4/5 (-25.0 [-42.0; -7.9] AU, P = 0.004).ConclusionsThe rEZR showed association with disease severity and the predilection area of MacTel. Given the current understanding of the pathophysiological concept of MacTel, these findings underscore the value of the rEZR as a potential novel biomarker for outer retinal integrity. Longitudinal studies are demanded to better characterize its value as a biomarker for early photoreceptor alterations and disease progression in MacTel.
| S-EPMC10362918 | biostudies-literature