A series at a single clinical centre recently demonstrated an association between the interstitial cystitis drug pentosan polysulfate sodium (PPS) and a vision-threatening pigmentary maculopathy. The ...aim of this study was to determine if an association exists between PPS use and macular disease in a large national cohort.
A retrospective, matched cohort study using data from a large US medical claims database from 2002 to 2016 was performed. A total of 3012 and 1604 PPS users were compared with 15 060 and 8017 matched controls at 5 and 7 years, respectively. The primary outcome measures included (1) any new diagnosis of a hereditary or secondary pigmentary maculopathy (atypical maculopathy outcome), and (2) any new diagnosis of dry age-related macular degeneration (AMD) or drusen in addition to the aforementioned diagnoses (atypical maculopathy+AMD outcome).
At the 5-year and 7-year follow-up, 9 (0.3%) and 10 (0.6%) PPS patients progressed to the atypical maculopathy outcome compared with 32 (0.2%) and 25 (0.3%) control patients, respectively. 103 (3.4%) and 87 (5.4%) PPS patients developed the atypical maculopathy+AMD outcome compared with 440 (2.9%) and 328 (4.1%) control patients at 5 and 7 years, respectively. At 5 years, multivariate analysis showed no significant association (p>0.13). At 7 years, PPS users had significantly increased odds of having the atypical maculopathy+AMD outcome (OR=1.41, 95% CI 1.09 to 1.83, p=0.009).
PPS exposure was associated with a new diagnosis of macular disease at the 7-year follow-up in a large national cohort.
The purpose of this study was to describe the ocular findings, structural ocular complications, and vision impairment in a cohort of Lassa fever survivors in Kenema, Sierra Leone. A retrospective, ...uncontrolled, cross-sectional study of 31 Lassa fever survivors (62 eyes) who underwent an ophthalmic evaluation in January 2018 at the Kenema Government Hospital in Kenema, Sierra Leone was performed. Data collection included demographic information, ocular/systemic symptoms, visual acuity (VA), and ophthalmic examination findings. Main outcome measures included anterior and posterior segment ophthalmic manifestations and level of VA impairment in Lassa fever survivors. Anterior segment findings included cataract (18%) and pterygium (2%), while posterior segment manifestations consisted of glaucoma (6%), preretinal hemorrhage (2%), and lattice degeneration (2%). Findings suggestive of prior sequelae of uveitis included chorioretinal scarring (5%), retinal fibrosis (3%), and vitreous opacity (2%). Visual acuity was normal/mildly impaired in 53 eyes (85%), moderately impaired in 6 eyes (10%), and 3 eyes (5%) were considered blind by the World Health Organization (WHO) criteria. Median VA was worse in Lassa fever survivors with ophthalmic disease findings (p<0.0001) for both anterior segment (p<0.0001) and posterior segment disease (p<0.013). Untreated cataract was a significant cause of visual acuity impairment (p<0.0001). Lassa fever survivors in this cohort were found to have cataract and posterior segment findings that potentially represent sequelae of uveitis associated with visual impairment. Future studies are warranted to improve our understanding of the spectrum of ocular disease in this emerging infectious disease of public health consequence.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
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 (greater than or equal to 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.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
To determine the incidence of worsening vitreoretinal traction after laser treatment for familial exudative vitreoretinopathy (FEVR) and to determine whether any baseline clinical features are ...associated with worsening.
Retrospective cohort comparison study in a university tertiary referral center. Methods: All patients 0-21 years of age treated with laser from January 1, 2001, to June 1, 2018, were studied. Worsening traction after treatment was defined as the occurrence within 6 months of worsening or development of tractional retinal detachment, folds, dragging, breaks, rhegmatogenous detachment, or worsening tractional membranes. Comparisons of baseline features between groups with and without worsening were performed to determine features associated with higher risk.
A total of 46 eyes from 28 patients met inclusion criteria. Of the 46 eyes, 6 (13%) had worsening after treatment. There were no significant differences in age or other baseline demographics between the cohorts with and those without worsening traction. The presence of proliferative tissue in contact with the lens was found in 2 of 6 patients with worsening compared to 1 of 40 (3%) without worsening (P = .04). Mean follow-up was 57.8 months (range, 6.6-134 months). At the 6-month follow-up, median logMAR visual acuity in the cohorts with and without worsening was 1.7 (Snellen 20/1002; n = 5) and 0.24 (Snellen 20/35; n = 16), respectively.
Laser treatment resulted in worsening traction in a substantial proportion of eyes with FEVR. Children with proliferative tissue in contact with the lens may be at higher risk of worsening after laser. Potential measures to reduce risk will require further study to establish efficacy.
The goal of this study was to document current clinical practice and report patient outcomes in presurgical language functional MRI (fMRI) for epilepsy surgery. Epilepsy surgical programs worldwide ...were surveyed as to the utility, implementation, and efficacy of language fMRI in the clinic; 82 programs responded. Respondents were predominantly US (61%) academic programs (85%), and evaluated adults (44%), adults and children (40%), or children only (16%). Nearly all (96%) reported using language fMRI. Surprisingly, fMRI is used to guide surgical margins (44% of programs) as well as lateralize language (100%). Sites using fMRI for localization most often use a distance margin around activation of 10mm. While considered useful, 56% of programs reported at least one instance of disagreement with other measures. Direct brain stimulation typically confirmed fMRI findings (74%) when guiding margins, but instances of unpredicted decline were reported by 17% of programs and 54% reported unexpected preservation of function. Programs reporting unexpected decline did not clearly differ from those which did not. Clinicians using fMRI to guide surgical margins do not typically map known language‐critical areas beyond Broca's and Wernicke's. This initial data shows many clinical teams are confident using fMRI not only for language lateralization but also to guide surgical margins. Reported cases of unexpected language preservation when fMRI activation is resected, and cases of language decline when it is not, emphasize a critical need for further validation. Comprehensive studies comparing commonly‐used fMRI paradigms to predict stimulation mapping and post‐surgical language decline remain of high importance.
To report multimodal imaging of novel retinal findings in a case of syndactyly, telecanthus, anogenital, and renal malformations (STAR) syndrome.
A 5-year old patient with STAR syndrome, an ...ultra-rare developmental disorder composed of syndactyly, telecanthus, anogenital, and renal malformations, was found to have bilateral macular yellow pigmentary changes and peripheral retinal pigment epithelial changes in a radial pattern highlighted by fundus autofluorescence (FAF) imaging. Optical coherence tomography (OCT) of the macula revealed foveal hypoplasia, ellipsoid zone disruption, and outer retinal atrophy suggestive of a retinal degeneration. OCT angiography found no significant abnormalities, and oral fluorescein angiography revealed staining in areas of atrophy in both eyes.
This case displays the first report of multimodal imaging of retinal manifestations in STAR syndrome, revealing bilateral foveal hypoplasia, outer retinal macular atrophy, and peripheral retinal pigment epithelial changes. Further studies and long-term follow-up are warranted to determine if patients with STAR syndrome have an underlying progressive retinal degeneration.
Little is known about how language functional MRI (fMRI) is executed in clinical practice in spite of its widespread use. Here we comprehensively documented its execution in surgical planning in ...epilepsy. A questionnaire focusing on cognitive design, image acquisition, analysis and interpretation, and practical considerations was developed. Individuals responsible for collecting, analyzing, and interpreting clinical language fMRI data at 63 epilepsy surgical programs responded. The central finding was of marked heterogeneity in all aspects of fMRI. Most programs use multiple tasks, with a fifth routinely using 2, 3, 4, or 5 tasks with a modal run duration of 5 min. Variants of over 15 protocols are in routine use with forms of noun–verb generation, verbal fluency, and semantic decision‐making used most often. Nearly all aspects of data acquisition and analysis vary markedly. Neither of the two best‐validated protocols was used by more than 10% of respondents. Preprocessing steps are broadly consistent across sites, language‐related blood flow is most often identified using general linear modeling (76% of respondents), and statistical thresholding typically varies by patient (79%). The software SPM is most often used. fMRI programs inconsistently include input from experts with all required skills (imaging, cognitive assessment, MR physics, statistical analysis, and brain–behavior relationships). These data highlight marked gaps between the evidence supporting fMRI and its clinical application. Teams performing language fMRI may benefit from evaluating practice with reference to the best‐validated protocols to date and ensuring individuals trained in all aspects of fMRI are involved to optimize patient care.
Diagnosis of Acute Retinal Necrosis The diagnosis of ARN is classically made through clinical examination based on the following criteria defined by the Executive Committee of the American Uveitis ...Society in 1994: at least one area of peripheral retinal necrosis with circumferential spread, anterior chamber and vitreous inflammation, occlusive vasculopathy, and rapid disease progression in the absence of therapeutic intervention.1 Maintaining a clinical suspicion for ARN is critical, and prompt treatment should be administered when the clinical exam is suggestive of ARN. The results of these studies suggest that oral valacyclovir achieves comparable outcomes to intravenous acyclovir and can be utilized as the initial induction therapy for ARN, potentially reducing health care costs associated with inpatient treatment. By contrast, Risseeuw et al. performed an analysis adjusting for severity of disease and did not find a risk reduction in the rate of rhegmatogenous RD (RRD) in ARN patients treated with prophylactic laser.12 Tibbetts et al.7 similarly found that prophylactic laser did not seem to affect the rate of RD, as RD occurred in 58% (11 of 19) of eyes treated with prophylactic laser versus 46% (18 of 39) that did not have laser treatment (P = .40). Risseeuw et al. similarly did not find a statistically significant association between prophylactic vitrectomy and reduction in the rate of RRD.12 Currently, there is insufficient evidence supporting the role for early vitrectomy to prevent RD and severe vision loss in ARN patients.
To develop automated algorithms for the detection of posterior vitreous detachment (PVD) using OCT imaging.
Evaluation of a diagnostic test or technology.
Overall, 42 385 consecutive OCT images (865 ...volumetric OCT scans) obtained with Heidelberg Spectralis from 865 eyes from 464 patients at an academic retina clinic between October 2020 and December 2021 were retrospectively reviewed.
We developed a customized computer vision algorithm based on image filtering and edge detection to detect the posterior vitreous cortex for the determination of PVD status. A second deep learning (DL) image classification model based on convolutional neural networks and ResNet-50 architecture was also trained to identify PVD status from OCT images. The training dataset consisted of 674 OCT volume scans (33 026 OCT images), while the validation testing set consisted of 73 OCT volume scans (3577 OCT images). Overall, 118 OCT volume scans (5782 OCT images) were used as a separate external testing dataset.
Accuracy, sensitivity, specificity, F1-scores, and area under the receiver operator characteristic curves (AUROCs) were measured to assess the performance of the automated algorithms.
Both the customized computer vision algorithm and DL model results were largely in agreement with the PVD status labeled by trained graders. The DL approach achieved an accuracy of 90.7% and an F1-score of 0.932 with a sensitivity of 100% and a specificity of 74.5% for PVD detection from an OCT volume scan. The AUROC was 89% at the image level and 96% at the volume level for the DL model. The customized computer vision algorithm attained an accuracy of 89.5% and an F1-score of 0.912 with a sensitivity of 91.9% and a specificity of 86.1% on the same task.
Both the computer vision algorithm and the DL model applied on OCT imaging enabled reliable detection of PVD status, demonstrating the potential for OCT-based automated PVD status classification to assist with vitreoretinal surgical planning.
Proprietary or commercial disclosure may be found after the references.
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