Atypical Presentation of Foster Kennedy Syndrome due to Neurocysticercosis: A Rare Case Report.

This case highlights the atypical presentation of Foster-Kennedy syndrome (FKS) associated with Neurocysticercosis (NCC), a prevalent cause of space-occupying lesions in areas endemic to the parasite. We report a newly diagnosed case of NCC in a 13-year-old boy who presented with a one-day history of abnormal movements of the left side of the body and no ocular complaints. Fundus examination of the patient revealed temporal disc pallor and a cup disc ratio (CDR) of 0.6 in the right eye suggesting unilateral optic disc atrophy and a hyperaemic disc with CDR 0.3 and blood vessel tortuosity in the left eye suggesting contralateral impending disc edema, mimicking the classic triad of FKS. He was diagnosed with NCC based on clinical features and radiological findings and was started on Carbamazepine (400 mg), Prednisolone (60 mg), Albendazole (400 mg), Acetazolamide (750 mg), and Vitamin B12 complex. Abbreviations: BCVA = Best Corrected Visual Acuity, CDR = Cup-Disc Ratio, CT = Computed Tomography, FKS = Foster Kennedy Syndrome, IDSA = Infectious Diseases Society of America, ICP = Intracranial Pressure, IOP = Intraocular Pressure, MRI = Magnetic Resonance Imaging, NCC = Neurocysticercosis, OOC = Orbital/Ocular Cysticercosis, OD = Right Eye, OS = Left Eye, OU = Both Eyes, RNFL = Retinal Nerve Fibre Layer, WNL = Within Normal Limits.

Applicability of the New Myelin Oligodendrocyte Glycoprotein Antibody-associated Disease Diagnostic Criteria in an Israeli Cohort.

BACKGROUND: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a rare autoimmune demyelinating disorder of the central nervous system. Optic neuritis (ON) is the most common clinical manifestation of MOGAD in adults. In 2023, new MOGAD diagnostic criteria were proposed, highlighting the importance of supplemental criteria when MOG-immunoglobulin G (IgG) titers are unavailable. OBJECTIVES: To investigate the applicability of the 2023 MOGAD criteria in patients diagnosed with MOGAD and treated before the availability of MOG-IgG titers. METHODS: We conducted a retrospective chart review of patients classified as MOGAD between 2010 and 2023 at Rabin Medical Center. Patient demographics as well as clinical and imaging data were collected, including visual acuity, expanded disability status score, core demyelinating events, antibody status, and brain and optic nerve magnetic resonance imaging data. Patients fulfilling the 2023 MOGAD criteria were reported as definite MOGAD. RESULTS: Fifteen patients met the 2023 MOGAD diagnostic criteria despite lack of MOG-IgG titer. The most common supplemental criterion meeting the 2023 MOGAD criteria was optic disc edema (n=12, 80%), followed by longitudinal optic nerve involvement (53%), bilateral ON (40%), and perineural optic sheath enhancement (33%). CONCLUSIONS: All patients with a clinical diagnosis of MOG-ON in our cohort fulfilled the 2023 MOGAD criteria despite the lack of antibody titers. The 2023 MOGAD criteria can be reliably applied to Israeli cohorts, prior to availability of MOGAD IgG titers, with particular attention to additional supplemental criteria. Since the 2023 MOGAD criteria were published, MOGAD IgG titers have been added to routine testing at our facility.

[Idiopathic intracranial hypertension. Review of our experience in the last eight years (2016-2023)]. / Hipertensión intracraneal idiopática. Revisión de nuestra experiencia en los últimos ocho años (2016-2023).

INTRODUCTION: Idiopathic intracranial hypertension (IIH) or benign intracranial hypertension is a rare disease in childhood. The clinical presentation in pediatric patients can be very variable, being more unespecific in younger patients. PATIENTS AND METHODS: A retrospective descriptive study was carried out on patients diagnosed of IIH in the last eight years (2016-2023) in the neuropediatrics unit of a tertiary hospital. In the present study, the clinical-epidemiological characteristics and the diagnostic-therapeutic procedure carried out in each case were analyzed. RESULTS: We studied 14 patients, 57% were women. The average age at diagnosis was 9 years, headache was the most common reason for consultation. In all patients, papilledema was found in the fundus and neuroimaging didn´t find alterations. Optical coherence tomography has been carried out in 78.5% of the sample, > 80% of patients showed thickening of the retinal nerve fiber layer. All patients had a high cerebrospinal fluid opening pressure (>25 cmH2O). 57% of patients required treatment with acetazolamide, a carbonic anhydrase inhibito. In all patients the resolution was complete, however almost 30% of them have presented recurrences during follow-up. CONCLUSIONS: In recent years there has been an increase in the incidence of this entity, making early diagnosis and treatment essential to avoid possible irreversible damage.

TITLE: Hipertensión intracraneal idiopática. Revisión de nuestra experiencia en los últimos ocho años (2016-2023).Introducción. La hipertensión intracraneal idiopática (HII), o hipertensión intracraneal benigna, es una enfermedad poco frecuente en la infancia. La presentación clínica en pacientes pediátricos puede ser muy variable, y es más inespecífica a menor edad. Pacientes y métodos. Se ha realizado un estudio descriptivo retrospectivo de los pacientes diagnosticados de HII en los últimos ocho años (2016-2023) en la consulta de neuropediatría de un hospital de tercer nivel. En el presente estudio se analizaron las características clinicoepidemiológicas y el procedimiento diagnosticoterapéutico llevado a cabo en cada caso. Resultados. Se estudió a 14 pacientes, de los cuales el 57% eran mujeres. La edad media en el momento del diagnóstico fue de 9 años, y la cefalea fue el motivo de consulta más habitual. En todos los pacientes se constató papiledema en el fondo de ojo y una prueba de neuroimagen sin alteraciones. Se llevó a cabo una tomografía de coherencia óptica en el 78,5% de la muestra, y >80% de los pacientes presentaba engrosamiento de la capa de fibras nerviosas retiniana. La totalidad de los pacientes presentaba una presión de apertura de líquido cefalorraquídeo elevada (> 25 cmH2O). El 57% de los pacientes precisó como tratamiento acetazolamida, un inhibidor de la anhidrasa carbónica. En todos los pacientes la resolución fue completa; sin embargo, casi el 30% de ellos presentó recurrencias durante el seguimiento. Conclusiones. En los últimos años se ha mostrado un aumento en la incidencia de esta entidad, y es fundamental un diagnóstico y un tratamiento precoces para evitar posibles secuelas irreversibles.

Diagnostic dilemma of papilledema and pseudopapilledema.

BACKGROUND: Papilledema is the optic disc swelling caused by increased intracranial pressure (ICP) that can damage the optic nerve and cause subsequent vision loss. Pseudopapilledema refers to optic disc elevation without peripapillary fluid that can arise from several optic disc disorders, with optic disc drusen (ODD) being the most frequent cause. Occasionally, pseudopapilledema patients are mistakenly diagnosed as papilledema, leading to the possibility of unneeded procedures. We aim to thoroughly examine the most current evidence on papilledema and pseudopapilledema causes and several methods for distinguishing between both conditions. METHODS: An extensive literature search was conducted on electronic databases including PubMed and google scholar using keywords that were relevant to the assessed pathologies. Data were collected and then summarized in comprehensive form. RESULTS: Various techniques are employed to distinguish between papilledema and pseudopapilledema. These techniques include Fundus fluorescein angiography, optical coherence tomography, ultrasonography, and magnetic resonance imaging. Lumbar puncture and other invasive procedures may be needed if results are suspicious. CONCLUSION: Papilledema is a sight-threatening condition that may lead to visual affection. Many disc conditions may mimic papilledema. Accordingly, differentiation between papilledema and pseudopailledema is crucial and can be conducted through many modalities.

The additive value of complementing diagnostic idiopathic intracranial hypertension criteria by MRI - an external validation study.

BACKGROUND: Recently, diagnostic criteria including a standardized MRI criterion were presented to identify patients suffering from idiopathic intracranial hypertension (IIH) proposing that IIH might be defined by two out of three objective findings (papilledema, ≥ 25 cm cerebrospinal fluid opening pressure (CSF-OP) and ≥ 3/4 neuroimaging signs). METHODS: To provide independent external validation, we retrospectively applied the proposed diagnostic criteria to our cohort of patients with clinical suspicion of IIH from the Vienna IIH database. Neuroimaging was reevaluated for IIH signs according to standardized definitions by a blinded expert neuroradiologist. We determined isolated diagnostic accuracy of the neuroimaging criterion (≥ 3/4 signs) as well as overall accuracy of the new proposed criteria. RESULTS: We included patients with IIH (n = 102) and patients without IIH (no-IIH, n = 23). Baseline characteristics were balanced between IIH and no-IIH groups, but papilledema and CSF-OP were significantly higher in IIH. For the presence of ≥ 3/4 MRI signs, sensitivity was 39.2% and specificity was 91.3% with positive predictive value (PPV) of 95.2% and negative predictive value (NPV) 25.3%. Reclassifying our cohort according to the 2/3 IIH definition correctly identified 100% of patients without IIH, with definite IIH and suggested to have IIH without papilledema by Friedman criteria, respectively. CONCLUSION: The standardized neuroimaging criteria are easily applicable in clinical routine and provide moderate sensitivity and excellent specificity to identify patients with IIH. Defining IIH by 2/3 criteria significantly simplifies diagnosis without compromising accuracy.

The Pseudotumor Cerebri Syndrome.

Pseudotumor cerebri syndrome is a syndrome of increased cerebrospinal fluid pressure without ventriculomegaly, mass lesion, or meningeal abnormality. It is either primary (idiopathic intracranial hypertension, IIH) or secondary. A secondary cause is unlikely when adhering to the diagnostic criteria. Permanent visual loss occurs if undetected or untreated, and the associated headaches may be debilitating. Fulminant disease may result in blindness despite aggressive treatment. This study addresses the diagnosis and management of IIH including new insights into the pathobiology of IIH, updates in therapeutics and causes of overdiagnosis.

The impact of eating disorders on idiopathic intracranial hypertension.

BACKGROUND: Idiopathic intracranial hypertension (IIH) occurs more frequently in obese females of childbearing age. A link between eating disorders and poor outcome has been suggested but remains unproven. METHODS: This prospective field study at two tertiary headache centers included patients with clinically suspected IIH after standardized diagnostic work-up. Eating disorders were evaluated using validated questionnaires (EDQs). Primary outcome was the impact of eating disorders on IIH severity and outcome, secondary outcome was the prevalence and type of eating disorders in IIH compared to controls. RESULTS: We screened 326 patients; 143 patients replied to the EDQs and were classified as 'IIH' or 'non-IIH' patients. The demographic profile of EDQ-respondents and non-respondents was similar. Presence of an eating disorder did not impact IIH severity (lumbar puncture opening pressure (p = 0.63), perimetric mean deviation (p = 0.18), papilledema (Frisén grad 1-3; p = 0.53)) nor IIH outcome (optic nerve atrophy (p = 0.6), impaired visual fields (p = 0.18)). Moreover, we found no differences in the prevalence and type of eating disorders when comparing IIH with non-IIH patients (p = 0.09). CONCLUSION: Eating disorders did not affect IIH severity or outcome. We found the same prevalence and distribution pattern of eating disorders in IIH and non-IIH patients advocating against a direct link between IIH and eating disorders.

Spontaneous onset and resolution of optic disk pit maculopathy in a young patient.

We report the case of an 8-year-old boy who presented with an optic disk pit and subsequently developed optic disk pit maculopathy, consisting of cystoid retinal edema in the peripapillary space and in the papillomacular bundle, which slowly and spontaneously resolved without intervention.

Clinical Course and Visual Outcomes of Papilledema in Pediatric Cerebral Venous Sinus Thrombosis.

PURPOSE: Cerebral venous sinus thrombosis (CVST) is a rare but life-threatening event with significant neurologic and visual morbidity. In this study, we report on the natural history and visual outcomes of papilledema in children with CVST. DESIGN: Retrospective case series. METHODS: Patients with CVST evaluated by the Department of Ophthalmology between 2000 and 2023 were included. Records were reviewed for presence and course of papilledema, treatment, and final visual outcomes following papilledema resolution. RESULTS: The study included 35 patients with a mean age of 9 ± 5 years and 40% were female. The most common risk factors for CVST were infection (69%), dehydration (26%), and hypercoagulability (23%). 31 patients (89%) had papilledema. Of these patients, 9 (29%) had progression of papilledema despite treatment, 17 patients (55%) did not have progression, and 5 patients (16%) lacked follow-up records. Initial Frisén grade among all cases was 2 ± 1, and cases with progression reached a grade of 4 ± 1 between 10 and 32 days following initial identification. Most patients (97%) were treated with anticoagulation and 100% required acetazolamide and/or lumbar puncture. Among 26 patients with follow-up, papilledema resolved in 107 ± 128 days. Fifty-four percent of patients had permanent ophthalmic sequelae. An initial Frisén grade ≥3 (odds ratio 7.54, 95% confidence interval 6.53-8.70, P< .001) was significantly associated with eventual optic atrophy. CONCLUSIONS: Children with CVST are at high risk for ophthalmologic sequelae. Papilledema can progress despite appropriate therapy. Our results highlight the importance of ophthalmologic follow-up during treatment course to prevent irreversible vision loss.

Persistent Elevation of Opening Pressure Despite Treatment and Symptom Resolution in Pediatric Intracranial Hypertension.

BACKGROUND: Currently no guidelines for repeating a lumbar puncture to guide management in primary intracranial hypertension (PIH) exist. METHODS: An institutional database of patients 18 years and younger followed in the institution's pediatric intracranial hypertension clinic was examined for opening pressure changes in PIH at diagnosis, before medication wean, and following medication wean, as well as to examine whether measurements at the time of diagnosis differed between those with and without disease recurrence. RESULTS: Forty-two patients were included in this study; 36% were male and the mean age at diagnosis was 11.01 years. Treatment duration averaged 9.68 months in those without recurrence and 8.5 months in those with recurrence. Average body mass index percentile of patients with disease recurrence was 83.7 and 72.1 in those without recurrence (P = 0.16). Average opening pressure values of all patients at diagnosis, prewean, and postwean was 36.53 cm H2O, 30.7 cm H2O, and 31.1 cm H2O, respectively. There was no statistically significant difference in opening pressures across these time points (P = 0.14). The change in opening pressure from diagnosis to postwean was statistically significant with a reduction of 5.18 cm H2O (P = 0.04). There was no statistical difference between change in opening pressure at diagnosis versus postwean between those with and without recurrence (P = 0.17). CONCLUSIONS: This clinical observational study suggests that mean opening pressure measurements in patients with PIH remain elevated both before and after medication wean despite papilledema resolution and patient-reported PIH symptoms. Clinically, this suggests that other features such as signs of optic disc edema and symptoms should be used to inform a clinical determination of disease recurrence and treatment course.

[Pulmonary and testicular sarcoidosis revealed by optic disc edema]. / Cas clinique. Sarcoïdose pulmonaire et testiculaire révélée par un oedème de la tête du nerf optique.

Sarcoidosis is a chronic inflammatory granulomatous disease of unknown aetiology. Although the lungs and thoracic lymph nodes are the most frequently affected structures, every tissue can be affected. Ophthalmological involvement occurs in 25 % of cases. We report a clinical case in which the diagnosis of sarcoidosis was revealed by isolated unilateral optic disc edema.

La sarcoïdose est une pathologie inflammatoire granulomateuse chronique dont l'étiologie reste inconnue. Bien que les poumons et les ganglions thoraciques soient les structures les plus fréquemment atteintes, tous les tissus peuvent être affectés. L'atteinte ophtalmologique est présente dans 25 % des cas. Nous rapportons un cas clinique dont le diagnostic de sarcoïdose fut révélé par un œdème papillaire unilatéral isolé.

Vision loss due to atypical bilateral edema of the optic nerve in a patient with hereditary angioedema: A case report.

PURPOSE: To describe a rare case of vision loss due to bilateral edema of the optic nerve in a patient with Hereditary Angioedema, treated with prophylactic C1-esterase inhibitor. METHODS: A 60-year-old Caucasian male affected by Hereditary Angioedema with unknown genetic defect (HAE- UNK) was admitted to our hospital presenting bilateral vision loss (best corrected visual acuity of 20/32 in the right eye and hand motion in the left eye) during an HAE attack. Intravenous administration of C1- esterase inhibitor (C1-INH, 1500 IU, Berinert, CSL Behring) determined the resolution of facial and periorbital swelling, however visual impairment persisted, in contrast with previous attacks experienced by the patient. Fundus examination revealed a vital optic disc without papilledema in both eyes. Magnetic resonance imaging (MRI) of the head and orbits showed bilateral edema of the optic nerve sheath. Treatment with intravenous and oral steroids was ineffective. Subsequently, a prophylactic treatment strategy with subcutaneous C1-esterase inhibitor was started (7000 IU every four days). RESULTS: Complete regression of edema of the optic nerves was observed by imaging at two months of follow-up after chronic treatment with C1-esterase inhibitor (7000 IU every four days). Complete restoration of visual acuity was achieved (BCVA 20/20 in both eyes) and multimodal imaging of the optic nerves demonstrated the absence of anatomical and functional damage. CONCLUSION: Patients affected by HAE may show atypical presentation with edema of the optic nerves without involvement of the optic nerve head. They may significantly benefit from prophylactic and chronic treatment with C1-esterase inhibitor.

Concomitant optic disk drusen and papilledema due to idiopathic intracranial hypertension in a pediatric cohort.

BACKGROUND: Optic disk drusen (ODD) in pediatric patients typically presents with pseudopapilledema. Diagnosing concomitant papilledema due to idiopathic intracranial hypertension (IIH) in these patients can be challenging. The purpose of this study was to evaluate the incidence and clinical features of papilledema due to IIH among pediatric patients with a new diagnosis of ODD and to discuss the clinical and paraclinical findings that helped diagnose this group. METHODS: The medical records of children <15 years of age with ODD confirmed by B-scan ultrasound at their first visit over a 4-year period (2019-2022) were reviewed retrospectively. Patients with concurrent IIH were identified, and the demographic and clinical characteristics were reviewed. RESULTS: A total of 83 children with confirmed ODD at the initial presentation were included, of whom 4 (4.8%) were diagnosed with concomitant IIH. Patients ranged in age from 7 to 15 years; 3 of the 4 were female, and 3 had IIH-related symptoms at presentation (1 was asymptomatic). None of the 4 patients had papilledema greater than Frisen grade 2. CONCLUSIONS: We recommend that clinicians review pertinent IIH symptoms and risk factors in children with ODD and follow the standard workup for IIH in suspicious cases. In asymptomatic patients with a new diagnosis of ODD, we recommend obtaining a follow-up optic nerve evaluation and optical coherence tomography scan to detect any significant interval change that might serve as a possible indicator of concomitant papilledema.

The BONSAI (Brain and Optic Nerve Study with Artificial Intelligence) deep learning system can accurately identify pediatric papilledema on standard ocular fundus photographs.

BACKGROUND: Pediatric papilledema often reflects an underlying severe neurologic disorder and may be difficult to appreciate, especially in young children. Ocular fundus photographs are easy to obtain even in young children and in nonophthalmology settings. The aim of our study was to ascertain whether an improved deep-learning system (DLS), previously validated in adults, can accurately identify papilledema and other optic disk abnormalities in children. METHODS: The DLS was tested on mydriatic fundus photographs obtained in a multiethnic pediatric population (<17 years) from three centers (Atlanta-USA; Bucharest-Romania; Singapore). The DLS's multiclass classification accuracy (ie, normal optic disk, papilledema, disks with other abnormality) was calculated, and the DLS's performance to specifically detect papilledema and normal disks was evaluated in a one-vs-rest strategy using the AUC, sensitivity and specificity, with reference to expert neuro-ophthalmologists. RESULTS: External testing was performed on 898 fundus photographs: 447 patients; mean age, 10.33 (231 patients ≤10 years of age; 216, 11-16 years); 558 normal disks, 254 papilledema, 86 other disk abnormalities. Overall multiclass accuracy of the DLS was 89.6% (range, 87.8%-91.6%). The DLS successfully distinguished "normal" from "abnormal" optic disks (AUC 0.99 [0.98-0.99]; sensitivity, 87.3% [84.9%-89.8%]; specificity, 98.5% [97.6%-99.6%]), and "papilledema" from "normal and other" (AUC 0.99 [0.98-1.0]; sensitivity, 98.0% [96.8%-99.4%]; specificity, 94.1% (92.4%-95.9%)]. CONCLUSIONS: Our DLS reliably distinguished papilledema from normal optic disks and other disk abnormalities in children, suggesting it could be utilized as a diagnostic aid for the assessment of optic nerve head appearance in the pediatric age group.

[An analysis of the etiology composition and clinical characteristics of bilateral optic disc swelling].

Objective: To investigate the etiology composition and clinical characteristics of bilateral optic disc swelling(ODS). Methods: The medical records of all newly diagnosed bilateral ODS patients admitted to the neurology ward of Beijing Tongren Hospital from January 2017 to June 2021 were retrospectively searched to classify the etiology, obtain demographic and clinical information, and compare the differences in clinical characteristics. Results: A total of 131 patients with bilateral ODS were included, including 56 males and 75 females, aged 15-73 (39±14) years. The most common cause of the bilateral ODS was increased intracranial pressure (ICP)(56/131, 42.7%), followed by optic neuritis (ON)(40/131, 30.5%). Other causes included vascular optic neuropathy (13/131, 9.9%), pseudopilledema (9/131, 6.9%); uveitis (6/131, 4.6%), toxic optic neuropathy (3/131, 2.3%) and unknown causes (4/131, 3.1%). Idiopathic intracranial hypertension (IIH) (43/56, 76.8%) was the most common etiology for papilledema. In the comparison of the two main causes of intracranial hypertension and the clinical characteristics of ON in ODS, there was no statistically significant difference in the incidence of age, gender, complaints of ocular pain or headache, and hemorrhage of optic disc(P>0.05). Visual acuity abnormalities and low vision were more common in ON group than the increased ICP group[36/40(90%) vs 33/56(58.9%), P=0.001; 35/80(43.8%) vs 22/112(19.6%), P<0.001], while severe papilledema was more common in increased ICP group[38/112(33.9%)vs 9/80(11.3%), P<0.001]. Conclusions: The most common cause for bilateral ODS is increased ICP, but it can also be triggered by a variety of other causes. Optic neuritis(ON) is the most important differentiating disease in the study of Chinese patients.

[Importance of fluorescein angiography for differentiating between early papilledema and papillitis]. / Stellenwert der Fluoreszenzangiographie zur Differenzierung zwischen einer frühen Stauungspapille und einer Papillitis.

BACKGROUND: Optic disc swelling poses a diagnostic challenge due to its multiple underlying pathological causes. This study aimed to investigate the use of fluorescein angiography (FLA) in combination with optical coherence tomography (OCT) as a diagnostic tool for differentiating between papilledema and papillitis in cases of optic disc swelling. MATERIAL AND METHODS: A total of 12 patients were included in the study in whom both FLA and OCT of the optic disc were performed to evaluate the optic disc swelling in cases of papilledema (7 patients, 14 eyes) and papillitis (5 patients, 7 eyes). The fluorescence behavior of the optic disc during late phase FLA was examined in relation to papillary thickness measured by OCT. RESULTS AND DISCUSSION: In the papilledema group OCT revealed a mean papillary thickness of 873 µm. In 6 patients FLA detected a ring-shaped papillary hyperfluorescence with papillary thicknesses ranging from 611 µm to 972 µm. Another patient with chronic and marked papilledema exhibited bilateral panpapillary leakage in FLA and papillary thicknesses of 1287 µm (right eye) and 1526 µm (left eye). In the papillitis group FLA showed panpapillary leakage in all cases. The mean papillary thickness was 865 µm (range 632-1195 µm). CONCLUSION: In acute optic disc swelling and a papillary prominence less than 1000 µm in OCT, a difference in FLA was noticeable between papilledema and papillitis. While acute and mild papilledema exhibited a ring-shaped hyperfluorescence, papillitis showed a panpapillary leakage in late phase FLA in the examined cases. This difference could not be seen in the case of papilledema with chronic and severe swelling.

Which OCT Measure of the Optic Nerve Head Improves Fastest? Towards Optimizing Early Detection of Resolving Papilledema in Children.

Purpose: Optical coherence tomography (OCT) has been used to monitor papilledema. This study aims to determine which OCT-derived measures of the optic nerve head (ONH) detect resolving papilledema in children faster than standard OCT measures. Methods: Children (≤18 years of age) with papilledema who completed optic nerve SD-OCT pretreatment and had evidence of treatment response on one or more follow-up OCTs within 4 months were included. Standard (mean circumpapillary retinal nerve fiber layer [cpRNFL] thickness), device-derived (per-quadrant cpRNFL) and custom (ONH height, maximum Bruch's membrane displacement [BMD], ONH volume [ONHV], and BMD volume) OCT measures were calculated. Per-eye generalized estimating equations (GEEs) modelled changes in device-derived and custom measures as a function of mean cpRNFL to identify those measures that resolved faster during early (0-2 months) follow-up. Mean cpRNFL coefficients of greater than 1 indicated faster resolving papilledema. Results: We included 52 eyes of 29 children (mean age, 12.8 years; 72.4% female). In analysis of early follow-up visits (38 eyes from 22 children), nasal cpRNFL and maximum BMD in each quadrant resolved faster than mean cpRNFL (GEE coefficients range, 1.14-3.37). Inferior cpRNFL, superior, nasal, and inferior ONH heights and ONHV resolved slower than mean cpRNFL (GEE coefficients range, 0.67-0.87). Conclusions: Nasal cpRNFL is a promising device-derived OCT measure for the early detection of resolving papilledema in children compared with mean cpRNFL. Maximum BMD, a custom measure, also shows promise, but its calculation has not yet been incorporated into commercial OCT devices. Translational Relevance: This study guides the optimal use of OCT in capturing resolving papilledema in children.

Evaluation of Peripapillary and Macular Optical Coherence Tomography Angiography Characteristics in Different Stages of Papilledema.

BACKGROUND: Prospective evaluation of optical coherence tomography (OCT) and OCT angiography (OCT-A) characteristics in different stages of papilledema in idiopathic intracranial hypertension (IIH). METHODS: In this prospective, observational study patients of IIH with papilledema were recruited and divided into 3 groups-early/established (Group 1), chronic (Group 2), and atrophic papilledema (Group 3). Peripapillary retinal nerve fiber layer (RNFL) and macular ganglion cell inner plexiform layer (GC-IPL) were recorded on OCT. Peripapillary and macular perfusion was documented at superficial retinal, deep retinal, and choriocapillary level using OCT-A. The investigations were repeated at 3 months. RESULTS: RNFL showed significant thinning in all groups on follow-up with the atrophic group showing maximum thinning ( P = 0.01-Group 3). GC-IPL was significantly reduced in all stages of papilledema at baseline compared with the controls. Thinnest GC-IPL was noted in the atrophic group (52.75 ± 7.44 µm; P = 0.00 in Group 3 vs controls) that showed further deterioration on follow-up. On Image J analysis, significant decrease was noted at various levels in the peripapillary and macular perfusion at baseline especially in the atrophic group which showed further deterioration noted on follow-up. The final visual acuity showed a statistically significant weak negative correlation with baseline RNFL (r = -0.306) and GC-IPL (r = -0.384) and moderately negative correlation with baseline superficial peripapillary retinal perfusion (r = -0.553). A significant negative correlation was seen between increasing grade of papilledema and superficial peripapillary retinal perfusion with both Image J and automated indices (r = -0.46; r = -0.61), respectively. CONCLUSIONS: GC-IPL may help identify early damage in papilledema even in the presence of thicker RNFL. Significant vascular changes can be observed on OCT-A that may help predict the final visual outcome in papilledema due to IIH.