Quantitative Analysis of Ellipsoid Zone in Acute Posterior Multifocal Placoid Pigment Epitheliopathy
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SUBMITTER: Browne A
PROVIDER: S-EPMC8171299 | biostudies-literature |
REPOSITORIES: biostudies-literature
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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: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:BACKGROUND:To quantitatively assess outer retinal layers in eyes with hydroxychloroquine (HCQ) toxicity. METHODS:A 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. RESULTS:There 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. CONCLUSION:HCQ 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:PurposeTo assess en face ellipsoid zone (EZ) maps of remaining retinal structure as outcome measures for the future clinical research in patients with choroideremia.MethodsTwenty eyes from 12 patients with a confirmed genetic diagnosis of choroideremia were included retrospectively from a single site. From spectral domain-optical coherence tomography volume scans, slabs including the EZ were manually segmented to create the en face EZ maps. The preserved EZ area was measured by two graders. Lengths of the EZ were recorded at 0°, 45°, 90°, and 135°. The intraclass correlation coefficients and Bland-Altman plots were used to show intergrader agreement. The Pearson correlation coefficient evaluated the correlation between length and area. A Bland-Altman plot compared en face EZ and the preserved fundus autofluorescence area.ResultsMeasurements of EZ area by two graders showed excellent agreement with an intraclass correlation coefficient of 0.992 (95% confidence interval, 0.980-0.997). A Pearson correlation analysis showed that the existing marker for preserved photoreceptor (horizontal EZ length) was correlated with the area (r = 0.722). The average EZ length in four meridians showed a much better correlation with the EZ area (r = 0.929). The fundus autofluorescence area was found to be a mean of 0.45 ± 0.99 mm2 greater than the EZ area.ConclusionsEZ area measurement provides excellent intergrader reliability, although the process is time consuming. We propose a less time-consuming alternative to estimate the EZ by using the average EZ band length in meridians. Our data also suggest that the loss of photoreceptor inner segments is an early change in choroideremia and may happen before the loss of the retinal pigment epithelium.Translational relevanceEn face EZ mapping is a potential tool for future clinical trials to quantify preserved photoreceptor structure in choroideremia.
| S-EPMC8132016 | biostudies-literature
Project description:PURPOSE:To investigate the progression characteristics of ellipsoid zone (EZ) loss in eyes with macular telangiectasia type 2 (MacTel) as reflected by area and linear measurements, and their relevance for visual acuity. METHODS:Participants were selected from the MacTel Study cohort. Linear and area measurements of EZ loss were performed in Spectral-Domain Optical Coherence Tomograph (SD-OCT) volume scans. Progression characteristics and correlations between linear and area measurements were analysed using linear mixed effects models. RESULTS:A total of 134 eyes of 70 patients were included (85 eyes with follow-up, mean 4.7 years, range: 1.4-8 years). Ellipsoid zone (EZ) loss significantly progressed at a mean annual increment of 0.057 mm2 (p = 0.005). The progression rate was non-linear and interacted significantly with initial EZ lesion size indicating an exponential growth before reaching a plateau. There was a strong heterogeneity in area sizes between fellow eyes. EZ break length had a significant linear effect on EZ break area (b = 1.06, p < 0.001) and could predict it. The location of the EZ break had a significant impact on visual acuity. CONCLUSION:Ellipsoid zone (EZ) loss in MacTel has a non-linear progression characteristic, and its rate depends on area size at baseline, which must be taken into account at sample selection in clinical trials. Our results show a good correlation of linear and area measures of EZ loss and a segregation of best-corrected visual acuity by EZ location, which may help routine clinical practice.
| S-EPMC6785352 | biostudies-literature
Project description:PurposeTo examine repeatability and reproducibility of ellipsoid zone (EZ) width measurements in patients with retinitis pigmentosa (RP) using a longitudinal reflectivity profile (LRP) analysis.MethodsWe examined Bioptigen optical coherence tomography (OCT) scans from 48 subjects with RP or Usher syndrome. Nominal scan lengths were 6, 7, or 10 mm, and the lateral scale of each scan was calculated using axial length measurements. LRPs were generated from OCT line scans, and the peak corresponding to EZ was manually identified using ImageJ. The locations at which the EZ peak disappeared were used to calculate EZ width. Each scan was analyzed twice by each of two observers, who were masked to their previous measurements and those of the other observer.ResultsOn average, horizontal width (HW) was significantly greater than vertical width (VW), and there was high interocular symmetry for both HW and VW. We observed excellent intraobserver repeatability with intraclass correlation coefficients (ICCs) ranging from 0.996 to 0.998 for HW and VW measurements. Interobserver reproducibility was also excellent for both HW (ICC = 0.989; 95% confidence interval [CI] = 0.983-0.995) and VW (ICC = 0.991; 95% CI = 0.985-0.996), with no significant bias observed between observers.ConclusionsEZ width can be measured using LRPs with excellent repeatability and reproducibility. Our observation of greater HW than VW is consistent with previous observations in RP, though the reason for this anisotropy remains unclear.Translational relevanceWe describe repeatability and reproducibility of a method for measuring EZ width in patients with RP or Usher syndrome. This approach could facilitate measurement of retinal band thickness and/or intensity.
| S-EPMC5989764 | biostudies-literature
Project description:Background: To analyze the pain modulation capacity profile in a Brazilian population, the relationship between opioid receptor (OPRM1) and Catechol-O-methyltransferase (COMT) 1polymorphisms and pain modulation capacity was determined through preoperative pain modulation tests and acute postoperative pain control evaluation, swelling, and trismus in 200 volunteers undergoing lower third molar removal. Methods: Psychologic and clinical parameters were measured. Patient DNA was sequenced for single nucleotide polymorphisms in OPRM1 and COMT, and the salivary concentration of interleukin (IL)-2 (IL)-6, interferon (IFN)-γ and tumor necrosis factor (TNF)-α was evaluated. Primary outcomes were the influence of all predictors on the fluctuation of pain intensity using a visual analogue scale (VAS), and swelling and trismus on the 2nd and 7th postoperative days. Preoperative pain modulation capacity (CPM), pain catastrophizing scale (PCS), body mass index (BMI), and surgery duration and difficulty were evaluated. Results: Salivary concentration of IFN-γ and IL-2 as well as the duration of surgery influenced the fluctuation of postoperative pain in the VAS, and in the sum of the differences in pain intensity test at 8, 48, and 96 h. BMI influenced swelling, while both BMI and COMT haplotype influenced trismus on the 2nd postoperative day. Conclusion: Polymorphisms in COMT, salivary concentrations of IL-2 and IFN-γ, BMI, and duration of surgery were predictors for pain fluctuation, swelling, and trismus on the 2nd day after lower third molar extraction. This therapy was effective in controlling inflammatory symptomatology after lower third molar extraction and ibuprofen was well tolerated by patients. Clinical Trial Registration: www.ClinicalTrials.gov, identifier NCT03169127.
| S-EPMC8082138 | biostudies-literature
Project description:PURPOSE:To compare images of photoreceptor layer disruptions obtained with optical coherence tomography (OCT) and adaptive optics scanning light ophthalmoscopy (AOSLO) in a variety of pathologic states. METHODS:Five subjects with photoreceptor ellipsoid zone disruption as per OCT and clinical diagnoses of closed-globe blunt ocular trauma (n = 2), macular telangiectasia type 2 (n = 1), blue-cone monochromacy (n = 1), or cone-rod dystrophy (n = 1) were included. Images were acquired within and around photoreceptor lesions using spectral domain OCT, confocal AOSLO, and split-detector AOSLO. RESULTS:There were substantial differences in the extent and appearance of the photoreceptor mosaic as revealed by confocal AOSLO, split-detector AOSLO, and spectral domain OCT en face view of the ellipsoid zone. CONCLUSION:Clinically available spectral domain OCT, viewed en face or as B-scan, may lead to misinterpretation of photoreceptor anatomy in a variety of diseases and injuries. This was demonstrated using split-detector AOSLO to reveal substantial populations of photoreceptors in areas of no, low, or ambiguous ellipsoid zone reflectivity with en face OCT and confocal AOSLO. Although it is unclear if these photoreceptors are functional, their presence offers hope for therapeutic strategies aimed at preserving or restoring photoreceptor function.
| S-EPMC4843118 | biostudies-literature
Project description:PurposeRod-cone dystrophy (RCD) is characterized by centripetal loss of rod followed by cone photoreceptors. In this prospective, observational cohort, we used flood-illumination adaptive optics (AO) imaging to investigate parafoveal cone loss in regions with preserved ellipsoid zone (EZ) in patients with RCD.MethodsEight patients with RCD and 10 age-matched healthy controls underwent spectral-domain optical coherence tomography and AO imaging. The RCD cohort underwent a follow-up examination after 6 months. Cone density (CD) and intercone distance (ICD) measurements were performed at 2° temporal from the fovea. Baseline CD and ICD values were compared between the control and patient groups, and longitudinal changes were calculated in the patient group. Residual EZ span in patients was measured in horizontal foveal B-scans.ResultsBetween the control and patient groups, there was no significant difference in the baseline CD (2094 vs. 1750 cells/deg2, respectively; P = 0.09) and ICD (1.46 vs. 1.62 arcmin, respectively; P = 0.08). Mean CD declined by 198 cells/deg2 (-11.3%; P < 0.01), and mean ICD increased by 0.09 arcmin (+5.6%; P = 0.01) at the 6-month follow-up in the patient group. Mean baseline and follow-up residual EZ spans in the six patients with EZ defect were 3189 µm and 3065 µm, respectively (-3.9%; P = 0.08).ConclusionsAO imaging detected significant parafoveal cone loss over 6-month follow-up even in regions with preserved EZ. Further studies to refine AO imaging protocol and validate cone metrics as a structural endpoint in early RCD are warranted.Translational relevanceCD and ICD may change prior to EZ span shortening in RCD.
| S-EPMC8684316 | biostudies-literature