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Background/Objectives Our aim was to compare factors associated with poor versus good visual outcomes in idiopathic intracranial hypertension (IIH) patients with severe papilledema at initial presentation. Subjects/Methods Retrospective review of consecutive IIH patients (1/1/2013-6/10/2023) with severe papilledema (Frisén grade 4–5 and/or atrophy in at least one eye); Patients were divided into “poor visual outcome” (poor visual acuity and constricted visual field in at least one eye) and “good visual outcome” (good visual acuity and only mild visual field changes in both eyes) at >6 months for medically-treated patients and >3 months follow-up for surgically-treated patients. Results We included 134 IIH patients with severe papilledema (70 had poor and 64 had good visual outcomes). No significant differences existed for age, gender, race, hypertension, haemoglobin, or cerebrospinal fluid opening pressure. The poor-outcome group had significantly greater BMI (mean 39.2 vs 35.1 kg/m 2 , p = 0.004), and worse initial HVF-MD (–20.04 vs –5.81 dB, p < 0.0001). Poor-outcome patients saw more prior health-care providers (4.7 vs 2.4, p < 0.0001), with delayed neuro-ophthalmology encounters (58.5 vs 15.2 weeks, p = 0.001). 41.4% of poor-outcome patients were initially seen in outside emergency departments (ED) (vs 14.1% of good-outcome patients, p = 0.0005), while only 27.1% were seen by eye-care providers (vs 53.1% of good-outcome patients, p = 0.0027). No poor-outcome patients initially consulted our institution’s ED vs 7.8% of good-outcome patients. Conclusions Although patients with poor visual outcome did not seek medical care later than good outcome patients, they had delayed diagnosis/treatment because of prior outside ED visits and lack of prior eye-care provider evaluations, suggesting that early diagnosis and specialized management of papilledema is essential for patients with symptoms of intracranial hypertension.

BackgroundNeurofilament light chain (NfL) reflects axonal damage in neurological disorders. It has recently been evaluated in idiopathic intracranial hypertension (IIH). A biomarker indicating the severity of optic nerve damage in IIH could support diagnostic accuracy and therapeutic decisions.MethodsWe retrospectively reviewed NfL concentrations in the cerebrospinal fluid (CSF) of 35 IIH patients and 12 healthy controls, who had received diagnostic workup for IIH in our clinic. The diagnosis of IIH was made according to the modified Friedman criteria for IIH and for IIH without papilledema Friedman DI et al Neurol 81:1159–1165 (2013) [1]. NfL in the CSF (CSF-NfL) was correlated with the severity of papilledema and with CSF opening pressure.ResultsCSF-NfL correlated with CSF opening pressure at the time of collection. In patients with IIH and moderate or severe papilledema, CSF-NfL was significantly increased compared to patients with mild or no papilledema. Healthy controls with raised intracranial pressure showed no relevant elevation of CSF-NfL.ConclusionCSF-NfL appears to correlate with the severity of papilledema in IIH and with CSF opening pressure and may therefore be a predictor of optic nerve damage in IIH patients.

Purpose Diagnosing pseudo-papilloedema (PPO) in children presents challenges and may lead to invasive investigations, with optic disc drusen (ODD) being the most common etiology. Other specific causes include tilted disc, optic neuritis, tumoral infiltration, vitreo-papillary traction, and Leber hereditary optic neuropathy. Peripapillary hyperreflective ovoid mass-like structures (PHOMS) are frequently observed in these cases, yet their pathophysiology remains unexplained, particularly their relation to ODD, which is still debated. Here, we explored whether patients with PPO associated with ODD, or seemingly isolated cases, could exhibit PHOMS without ODD or ODD without PHOMS, and how this might affect retinal nerve fiber layer (RNFL) thickness. Methods In this two-centre retrospective observational study, we included patients under 20 years old presenting with PPO without specific causes, with a subgroup followed for at least one year. Enhanced depth imaging optical coherence tomography was used to assess the presence and evolution of PHOMS and ODD, as well as RNFL thickness. Results We included twenty-seven patients, with thirteen followed for at least one year. In all eyes, we observed concomitant PHOMS and either deep or superficial ODD. RNFL thickness was increased in patients with deep ODD and decreased in those with superficial ODD, which was observed during follow-up. Conclusions ODD and PHOMS are concomitant features present in patients with PPO. PHOMS sometimes serve as indicators, as buried ODD are challenging to identify in young children. However, ODD tend to become more superficial over time, while RNFL thickness decreases. Key Messages What is known Diagnosing the cause of pseudo-papilledema (PPO) in children is challenging and often requires invasive investigations. The relationship between optic disc drusen (ODD) and peripapillary hyperreflective ovoid mass-like structures (PHOMS) in the context of PPO remains poorly understood. What is new PHOMS and ODD seem to consistently co-occur in pediatric PPO cases where no other specific causes are identified. Retinal nerve fiber layer (RNFL) thickness increases in the presence of deep ODD and decreases as ODD become more superficial with age. RNFL thinning progresses in correlation with the patient’s age.

Purpose Papilloedema is recognised as an indicator of raised intracranial pressure, although there is a paucity of literature describing the utility of fundoscopy in screening for raised ICP in children with craniofacial synostosis, particularly young children. We sought to investigate the association of optic disc morphology with ICP in children, and to define the sensitivity and specificity of papilloedema as a clinical indicator of raised ICP and determine if age, or underlying conditions impact the findings. Method Retrospective analysis of all patients undergoing ICP monitoring at a designated paediatric neurosurgical and craniofacial unit in the United Kingdom between October 2009 and October 2018. The fundoscopy findings and ICP monitoring data were analysed for 31 children with craniosynostosis and 29 children without craniosynostosis. Results All children who had papilloedema had raised ICP confirmed with monitoring. Across the 60-patient cohort, confirmed papilloedema on fundoscopy had Positive Predictive Value (PPV) of 1.00, Negative Predictive Value (NPV) of 0.64 with sensitivity 48% and specificity 100% for the presence of raised ICP (p =  < 0.0001). In the craniosynostosis group, PPV was 1.00, NPV was 0.39, sensitivity 48% and specificity 100% (p =  < 0.03). There is no correlation between severity of optic disc swelling using Frisen grading and elevation of ICP. Age did not affect the presence of papilloedema in those with raised ICP. Conclusion The presence of papilloedema is a strong indicator of raised ICP in a child, regardless of underlying aetiology. Detailed fundoscopy can prevent the need for further investigations including imaging-related radiation and invasive CSF pressure monitoring.

Purpose Intracranial hypertension (IH) can complicate cerebral venous thrombosis (CVT), potentially causing permanent visual loss. Current knowledge on risk factors for the development of IH following CVT is scarce. We applied a compound classifier (CSF opening pressure > 25 cmH 2 O, papilledema, or optic disc protrusion on MRI) as a surrogate for IH and studied the predictive value of thrombus location, the number of thrombosed segments, and thrombus volume. Methods We prospectively included 26 patients with acute CVT and complete MRI data. IH was defined by CSF opening pressure > 25 cmH 2 O, papilledema, or optic disc protrusion on MRI. Using high-resolution contrast-enhanced venography, we determined the thrombus location, number of thrombosed segments, and thrombus volume. We analyzed their association with IH by logistic regression, their predictive power by the area under the receiver operating characteristic curve, and their association with CSF opening pressure by linear regression. Results IH occurred in 46% of CVT patients and was associated with higher thrombus volume (AUC 0.759, p = 0.025) and superior sagittal sinus thrombosis both alone (OR 2.086, p = 0.049) and combined with transverse sinus thrombosis (OR 2.014, p = 0.028). Effects in patients presenting CSF opening pressure > 25 cm H 2 O and the compound classifier were consistent. Thrombus volume > 4 ml was the single most important predictor of higher CSF opening pressure ( ß = 0.566, p = 0.035), increasing IH risk. Conclusion Larger thrombus volume, dominant transverse sinus occlusion, and extensive superior sagittal combined with transverse sinus thrombosis were associated with IH. Thrombus volumetry might identify patients at risk for IH and direct further clinical evaluation.

Purpose To compare transorbital point-of-care ultrasound techniques —optic nerve sheath diameter (US-ONSD) and optic disc elevation (US-ODE)— with fundoscopic papilledema to detect potentially raised intracranial pressure (ICP) with treatment indication in children. Methods In a prospective study, 72 symptomatic children were included, 50 with later proven disease associated with raised ICP (e.g. pseudotumour cerebri, brain tumour, hydrocephalus) and 22 with pathology excluded. Bilateral US-ONSD and US-ODE were quantified by US using a 12-MHz-linear-array transducer. This was compared to fundoscopic optic disc findings (existence of papilledema) and, in 28 cases, invasively measured ICP values. Results The sensitivity and specificity of a cut-off value of US-ONSD (5.73 mm) to detect treatment indication for diseases associated with increased ICP was 92% and 86.4%, respectively, compared to US-ODE (0.43 mm) with sensitivity: 72%, specificity: 77.3%. Fundoscopic papilledema had a sensitivity of 46% and a specificity of 100% in this context. Repeatability and observer-reliability of US-ODE examination was eminent (Cronbach’s α  = 0.978–0.989). Papilledema was detected fundoscopically only when US-ODE was > 0.67 mm; a US-ODE > 0.43 mm had a positive predictive value of 90% for potentially increased ICP. Conclusion In our cohort, transorbital point-of-care US-ONSD and US-ODE detected potentially elevated ICP requiring treatment in children more reliably than fundoscopy. US-ONSD and US-ODE indicated the decrease in ICP after treatment earlier and more reliably than fundoscopy. The established cut-off values for US-ONSD and US-ODE and a newly developed US-based grading of ODE can be used as an ideal first-line screening tool to detect or exclude conditions with potentially elevated ICP in children.

In this study, we evaluated the topographic pattern of retinal edema in eyes with macular edema (ME) secondary to branch retinal vein occlusion (BRVO) using a widefield retinal thickness map of optical coherence tomography and its association with ME recurrence. In 87 eyes with ME secondary to BRVO who were treated with anti-vascular endothelial growth factor (VEGF) injections and followed up for ≥ 1 years, 12 × 9 mm macular volume scans of swept-source optical coherence tomography (DRI-OCT Triton; Topcon Inc, Japan) were performed and retinal thickness maps were automatically generated at baseline and follow-up visits. Topographic patterns of retinal edema on the maps at baseline and 1 month after the first anti-vascular endothelial growth factor (VEGF) treatment were classified as extramacular (outside the ETDRS grid), macular (within the grid), and combined pattern and correlated with ME recurrences. Seventy-five of 87 (86.2%) eyes with BRVO ME showed combined edema at baseline. There were 4 topographic patterns of edema at 1 month following anti-VEGF injection as follows: no residual edema, extramacular only, macular only, and combined edema. In contrast to the baseline pattern, the pattern of retinal edema 1 month following anti-VEGF therapy showed significant association with 6-month recurrence, number of ME recurrences during a 1-year period, and time to first recurrence. (all P  < 0.05) An automatically generated widefield retinal thickness map could be used to effectively visualize the topographic patterns of retinal edema in eyes with BRVO. The map can be used as a valuable tool for detection of retinal edema on widefield retinal areas and prediction of ME recurrence in eyes with BRVO.

The appearance of the optic disc is a key measure of disease status in idiopathic intracranial hypertension (IIH). The Frisén classification describes stages of optic disc swelling (grades 0–5). It is the only classification of papilloedema, and is used internationally in clinical and research practice. Despite this, there has been very limited evaluation of the scale. We assessed the inter-rater reproducibility and ability to discriminate optic disc changes over time using the Frisén classification compared with a system of ranking papilloedema severity in patients with IIH. Paired disc photographs (before and after treatment) were obtained from 47 patients with IIH (25 acute and 22 chronic). Six neuro-ophthalmologists blinded to patient identity, clinical information and chronology of the photographs reviewed the discs and allocated a Frisén grade and ranked the paired discs in order of papilloedema severity (disc ranking). A total of 188 optic disc photographs were reviewed. All six reviewers agreed in only three comparisons (1.6%) when using the Frisén classification, compared with 42 comparisons (45.2%) when using disc ranking. The probability of agreement between any two reviewers was 36.1% for Frisén grade and 70.0% for disc ranking. Disc ranking had significantly greater sensitivity for finding differences in degree of disc oedema, identifying a difference in 75.3% of paired photographs compared to 53.2% detected using the Frisén classification ( p  < 0.001). This study demonstrated the limited reproducibility and discriminative ability of the Frisén classification in identifying changes in serial optic disc photographs in IIH. Simple optic disc ranking appears to be a more sensitive and reliable tool to monitor changes in optic disc appearance. The use of disc ranking in clinical practice and research studies is recommended to monitor alterations in optic disc appearance until alternative schemes, specific to IIH, have been developed.

Objective The aim of the study was to determine the clinical and prognostic significance of craniospinal elastance (Ecs), pressure volume index (PVI), and cerebrospinal fluid (CSF) total protein data in terms of resolution of papilledema and improvement of headache in children with pseudotumor cerebri syndrome (PTCS). Methods This is a retrospective observational study of 33 children with definitive PTCS. The relationships between lumbar puncture (LP) measurements, especially Ecs and PVI, and papilledema and headache resolution time were investigated. Results In children with definite primary and secondary PTCS, higher opening pressure and decreased PVI were found to be associated with faster resolution of papilledema and faster improvement of headache ( r  = 0.904, P  < 0.01; r  = 0.894, P  < 0.01 respectively). This effect was higher in children with secondary PTCS ( P  = 0.022). While papilledema resolution time and treatment time were statistically significantly higher in the secondary group ( P  = 0.035, P  = 0.040), there was no significant difference between the two groups in terms of headache relief time ( P  = 0.051). Based on the primary and secondary groups, from the cut-off points determined, it was found that ≤ 41.60 ((AUC = 0.706, P  = 0.0420), specificity 56%, sensitivity 86.7%) for opening pressure and ≤ 69.37 ((AUC = 0.702, P  = 0.0448), specificity 48.2%, sensitivity 83.3%) for PVI were statistically significant. A very strong negative linear correlation was found between CSF total protein and lumbar puncture measurements and clinical outcomes ( P  < 0.001). Conclusion Ecs and PVI may be markers that can provide potentially important data on pediatric PTCS prognosis. CSF total protein may also provide clinical benefit.

Headache is one of the most common complaints among pediatric patients and can be due to many causes, some benign but others potentially seriously. Increased intracranial pressure, which is known to cause papilledema, is a serious cause of headache, and immediate diagnosis is critical, although difficult. The current study evaluates the diagnostic value of optic nerve sheath diameter (ONSD) and eyeball transverse diameter (ETD) ratio in pediatric patients presenting with headache and papilledema. A retrospective analysis of all pediatric patients undergoing head computed tomography scans between January 2013 and December 2015. Patients with normal brain scans were included in the study. Patients presenting with headache underwent funduscopic evaluation and grouped as either headache with papilledema or headache without papilledema. A control group of patients without headache was also included. Studies were reviewed blindly by a neuroradiologist and ONSD and ETD for both eyes were measured. ONSD/ETD index was found to have significantly higher values (p<0.001) in patients with papilledema (median 0.24, interquartile range (IQR) = 0.22-0.25) compared to patients without papilledema (median 0.18, IQR = 0.16-0.19) and the control group (median 0.17, IQR = 0.15-0.18). The ONSD/ETD index showed excellent discrimination ability for patients with headache and papilledema (AUC = 0.96, 95% CI, 0.94-0.99). The ONSD/ETD index of 0.21 was found to have a sensitivity and specificity of 82% and 93%, respectively, for identifying pediatric patients with headache and papilledema. Our study shows that ONSD/ETD index of 0.21 can be used as an easy-to-use reference tool for diagnosing papilledema and elevated intracranial pressure in pediatric patients.