The sensitivity and specificity of retinal and choroidal folds to distinguish between mild papilloedema and pseudopapilledema

Reggie, Sara N.; Avery, Robert A.; Bavinger, J. Clay; Jivraj, Imran; Alfaro, Cesar; Pistilli, Maxwell; Kohli, Anita; Liu, Grant T.; Shindler, Kenneth S.; Ross, Ahmara G.; Kardon, Randy H.; Sibony, Patrick A.; Tamhankar, Madhura A.

doi:10.1038/s41433-020-01368-y

Cited by 5 publications

(5 citation statements)

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“…On rare occasions, low intraocular pressure can lead to choroidal folds, for example following surgery; however, evidence suggests intraocular pressure is not decreased during long-duration spaceflight . The presence of choroidal folds, retinal folds, or peripapillary wrinkles in terrestrial patients with optic disc edema may indicate elevated intracranial pressure (ICP) when other known causes such as orbital disease, tumor, posterior scleritis, and hypotony are ruled out …”

Section: Introductionmentioning

confidence: 99%

“…14,15 The presence of choroidal folds, retinal folds, or peripapillary wrinkles in terrestrial patients with optic disc edema may indicate elevated intracranial pressure (ICP) when other known causes such as orbital disease, tumor, posterior scleritis, and hypotony are ruled out. 16,17 Folds develop within the retina due to changes in mechanical loading conditions and biomechanical tissue properties. 8,18 Thus, the timing, location, orientation, and pattern of fold presentation within the retina may provide insight into the underlying pathophysiology.…”

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confidence: 99%

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Incidence and Progression of Chorioretinal Folds During Long-Duration Spaceflight

et al. 2023

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ImportanceThe primary contributing factor for development of chorioretinal folds during spaceflight is unknown. Characterizing fold types that develop and tracking their progression may provide insight into the pathophysiology of spaceflight-associated neuro-ocular syndrome and elucidate the risk of fold progression for future exploration-class missions exceeding 12 months in duration.ObjectiveTo determine the incidence and presentation of chorioretinal folds in long-duration International Space Station crew members and objectively quantify the progression of choroidal folds during spaceflight.Design, Setting, and ParticipantsIn this retrospective cohort study, optical coherence tomography scans of the optic nerve head and macula of crew members completing long-duration spaceflight missions were obtained on Earth prior to spaceflight and during flight. A panel of experts examined the scans for the qualitative presence of chorioretinal folds. Peripapillary total retinal thickness was calculated to identify eyes with optic disc edema, and choroidal folds were quantified based on surface roughness within macular and peripapillary regions of interest.Interventions or ExposuresSpaceflight missions ranging 6 to 12 months.Main Outcomes and MeasuresIncidence of peripapillary wrinkles, retinal folds, and choroidal folds; peripapillary total retinal thickness; and Bruch membrane surface roughness.ResultsA total of 36 crew members were analyzed (mean [SD] age, 46 [6] years; 7 [19%] female). Chorioretinal folds were observed in 12 of 72 eyes (17%; 6 crew members). In eyes with early signs of disc edema, 10 of 42 (24%) had choroidal folds, 4 of 42 (10%) had inner retinal folds, and 2 of 42 (5%) had peripapillary wrinkles. Choroidal folds were observed in all eyes with retinal folds and peripapillary wrinkles. Macular choroidal folds developed in 7 of 12 eyes (4 of 6 crew members) with folds and progressed with mission duration; these folds extended into the fovea in 6 eyes. Circumpapillary choroidal folds developed predominantly superior, nasal, and inferior to the optic nerve head and increased in prevalence and severity with mission duration.Conclusions and RelevanceChoroidal folds were the most common fold type to develop during spaceflight; this differs from reports in idiopathic intracranial hypertension, suggesting differences in the mechanisms underlying fold formation. Quantitative measures demonstrate the development and progression of choroidal folds during weightlessness, and these metrics may help to assess the efficacy of spaceflight-associated neuro-ocular syndrome countermeasures.

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Section: Introductionmentioning

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Incidence and Progression of Chorioretinal Folds During Long-Duration Spaceflight

et al. 2023

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“…In discs with papilledema, transverse axial and en face OCT reveal structural changes, including anterior displacement of Bruch's membrane opening, peripapillary folds or wrinkles, and peripapillary fluid accumulation. [37][38][39] Quantitative OCT measures of the peripapillary RNFL thickness also may be helpful in the evaluation and monitoring of the optic disc swelling of papilledema. [40][41][42][43] However, there are several limitations in clinical use, including the considerable overlap of RNFL thickness values among normal optic discs, discs with deep ODD or mild papilledema, 44,45 absence of normative RNFL thickness values in children and in ocular conditions such as high myopia, falsely normative values of RNFL thickness when there is concurrent optic disc edema and optic atrophy, and false positively thickened RNFL from other disc abnormalities (e.g., gliosis, hyperopia).…”

Section: Discussionmentioning

confidence: 99%

A Deep Learning Approach to Accurately Discriminate Between Optic Disc Drusen and Papilledema on Fundus Photographs

Sathianvichitr

Najjar

Tang

et al. 2023

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Objective: To assess the performance of a deep learning system (DLS) to discriminate between optic disc drusen (ODD) and papilledema caused by intracranial hypertension, using standard color ocular fundus photographs collected in a large international multi-ethnic population. Design: Retrospective study. Participants: The study included 4,508 color fundus images in 2,180 patients from 30 neuro-ophthalmology centers (19 countries) participating in the Brain and Optic Nerve Study with Artificial Intelligence (BONSAI) Group. Methods: We trained, validated, and tested a dedicated DLS for binary classification of ODD vs. papilledema (including various subgroups within each category), on conventional mydriatic digital ocular fundus photographs. For training and internal validation, we used 857 ODD images and 3,230 papilledema images, in 1,959 patients. External-testing was subsequently performed on an independent dataset (221 patients) including 207 images with ODD (96 visible and 111 buried), provided by 3 centers of the Optic Disc Drusen Studies Consortium, and 214 images of papilledema (92 mild-to-moderate and 122 severe) from a previously validated study. Main outcome measures: Area under the receiver operating characteristic curve (AUC), accuracy, sensitivity, and specificity were used to discriminate between ODD and papilledema. Results: Overall, the DLS could accurately distinguish between all ODD and papilledema (all severities included): AUC 0.97 (95% confidence interval [CI], 0.96 to 0.98), accuracy 90.5% (95% CI, 88.0% to 92.9%), sensitivity 86.0% (95% CI, 82.1% to 90.1%), and specificity 94.9% (95% CI, 92.3% to 97.6%). The performance of the DLS remained high for discrimination of buried ODD from mild-to-moderate papilledema: AUC 0.93 (95% CI, 0.90 to 0.96), accuracy 84.2% (95% CI, 80.2%-88.6%), sensitivity 78.4% (95% CI, 72.2% to 84.7%), and specificity 91.3% (95% CI, 87.0% to 96.4%). Conclusions: A dedicated DLS can accurately distinguish between ODD and papilledema caused by elevated intracranial pressure, even when considering buried ODD vs mild-to-moderate papilledema. Future studies are required to validate the utility of this DLS in clinical practice.

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“…Shallow elevations of the RPE known as the ‘double layer sign’, subretinal pigment detachment ring-like lesion associated with thumb-like PED has high specificity and sensibility for the diagnosis of PVC [ 31 , 32 ]. For chorioretinal folds, fundoscopy is usually sensitive enough to detect, but FA and OCT may aid information on choroidal folds when peripapillary wrinkles and retinal folds are not present, supporting its diagnosis [ 33 , 34 ]. Therefore, the use of OCT and OCT-A in IIH is essential not only to evaluate the degree of papilledema and ganglion cell loss, but also for early detection of complications as the development of chorioretinal folds and polypoidal lesions.…”

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confidence: 99%

Chorioretinal abnormalities in idiopathic intracranial hypertension: case reports

et al. 2022

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Background Papilledema is the main ocular finding in patients with idiopathic intracranial hypertension (IIH) although several chorioretinal abnormalities may also occur and contribute to visual loss. The purpose of this paper is to describe two cases of chorioretinal abnormalities associated with idiopathic intracranial hypertension: one with choroidal folds and another with polypoidal choroidal vasculopathy, an extremely unusual ocular complication in the disease. Case presentation Case 1: A 47-year-old woman previous diagnosed with idiopathic intracranial hypertension treated with weight loss and acetazolamide that over the following 6 months had optic disc edema gradually resolved. The patient was follow-up for a period of 10 years and the papilledema disappeared, but choroidal folds remained unchanged. Case 2: A 61-year-old female patient was seen as a follow-up examination of a 5-year history of IIH that presented with papilledema. The patient was asymptomatic but fundoscopy evaluation revealed a yellowish white peripapillary subretinal nodular lesion temporally in OD. Multimodal imaging studies were made, and the patient was diagnosed with a rare and just recent described association of IIH and polypoidal choroidal vasculopathy. Conclusion Papilledema, RNFL and retinal ganglion cell loss are the most common structural complications of IIH, but chorioretinal complications are important findings and should be carefully evaluated in such patients. Awareness of such occurrence and the use of appropriated clinical and multimodal imaging studies are of great importance for its early detection, leading to proper treatment and prevention of further visual loss.

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The sensitivity and specificity of retinal and choroidal folds to distinguish between mild papilloedema and pseudopapilledema

Cited by 5 publications

References 16 publications

Incidence and Progression of Chorioretinal Folds During Long-Duration Spaceflight

Incidence and Progression of Chorioretinal Folds During Long-Duration Spaceflight

A Deep Learning Approach to Accurately Discriminate Between Optic Disc Drusen and Papilledema on Fundus Photographs

Chorioretinal abnormalities in idiopathic intracranial hypertension: case reports

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