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Background: Empty sella often supports a diagnosis of raised intracranial pressure (ICP) but is also seen in normal individuals. This study's objective was to determine the prevalence of empty and partially empty sella in neuro-ophthalmology patients undergoing MRI for indications other than papilledema or raised ICP., Methods: Consecutive patients without papilledema or suspected raised ICP who underwent brain MRI between August 2017 and May 2021 were included in this study. Sagittal T1 images were evaluated by 2 independent, blinded neuroradiologists who graded the sella using the published criteria (Categories 1-5, with 1 being normal and 5 showing no visible pituitary tissue). Clinical parameters were also collected., Results: A total of 613 patients (309 men; average age 56.69 ± 18.06 years) were included in this study with optic neuropathy as the most common MRI indication. A total of 176 patients had moderate concavity of the pituitary gland (Category 3), 81 had severe concavity (Category 4), and 26 had no visible pituitary tissue (Category 5). Sella appearance was mentioned in 92 patients' radiology reports (15%). There was a statistically significant difference in age between composite Categories 1 and 2 (mean 52.89 ± 18.91; P < 0.001) and composite Categories 4 and 5 (mean 63.41 ± 15.44), but not the other clinical parameters., Conclusion: Empty sella is common in neuro-ophthalmology patients without raised ICP; 17.4% of patients have severe concavity or no pituitary tissue visible. An isolated finding of empty or partially empty sella on imaging is therefore of questionable clinical value in this patient population., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by North American Neuro-Ophthalmology Society.)

Background: Idiopathic intracranial hypertension (IIH) is a condition of elevated intracranial pressure without an identifiable cause. It mostly affects young obese women but has been reported in individuals newly diagnosed with anemia or with chronic anemia. The relationship between anemia and IIH is not well elucidated. This study aims to characterize the course of IIH in anemic patients., Methods: Consecutive patients with IIH were recruited from neuro-ophthalmology clinics. Subsequent retrospective chart review obtained information on symptoms, complete blood counts (CBCs), visual acuity, visual fields, and optic disc edema at baseline and follow-up. Anemia was diagnosed by hemoglobin <120 g/L (women) and <130 g/L (men)., Results: One hundred twenty-three patients with IIH were recruited for this study, and 22.8% (28/123) had anemia. More anemic individuals had mild-to-moderate visual acuity impairment (logarithm of minimum angle of resolution 0.3-1, P = 0.01) and worse automated mean deviation ( P = 0.048). The median follow-up time was 47.4 weeks (interquartile range:20.1-91.8). Fifteen of 28 patients with anemia received anemia treatment and showed a trend toward increased hemoglobin. At follow-up, there were no differences in the visual acuity, but patients with anemia had worse automated mean deviation on visual fields ( P = 0.045)., Conclusions: IIH patients with anemia had worse visual function at presentation and worse final visual field parameters. This suggests that CBCs should be obtained for patients with papilledema because it may influence final visual outcome., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by North American Neuro-Ophthalmology Society.)

Competing Interests: The authors report no conflicts of interest.

Background: Papilledema must be managed distinctly from other causes of optic disc edema (ODE) due to its basis in raised intracranial pressure (ICP). However, evidence indicates that the term "papilledema" is widely misused across specialties to describe ODE without raised ICP. Sources of this misconception remain undiscerned. Because all physicians consult medical databases, our objective was to evaluate whether nonspecific "papilledema" subject heading definitions misleadingly associate articles on other conditions with papilledema proper., Methods: Systematic review of case reports, prospectively registered on PROSPERO (CRD42022363651). MEDLINE and Embase were searched to July 2022 for any full-length case report indexed to the "papilledema" subject heading. Studies were graded for incorrect indexing, defined as cases lacking evidence for raised ICP. Nonpapilledema diagnoses were assigned to a predefined set of diseases and pathophysiological mechanisms for subsequent comparison., Results: Incorrect indexing occurred in 40.67% of 949 included reports. Embase-derived studies were misindexed significantly less than MEDLINE-derived studies ( P < 0.01). There was also significant heterogeneity in incorrect indexing among specific diseases ( P = 0.0015) and mechanisms ( P = 0.0003). The most commonly misindexed diseases were uveitis (21.24% of errors), optic neuritis (13.47%), and instances with no mention of ODE (13.99%). The most commonly misindexed mechanisms were inflammation (34.97%), other mechanism (e.g., genetic; 25.91%), and ischemia (20.47%)., Conclusions: Database subject headings, especially from MEDLINE, do not adequately distinguish between true papilledema and other causes of ODE. Inflammatory diseases were most often incorrectly indexed among other diseases and mechanisms. Current "papilledema" subject headings should be revised to reduce the probability of misinformation., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by North American Neuro-Ophthalmology Society.)

Background: We reviewed the medical case report literature to determine the proportion of cases of idiopathic intracranial hypertension (IIH) that were either inappropriately labelled as IIH or prematurely given this diagnosis., Methods: We searched OVID MEDLINE from 2012 to 2022 to identify case reports that diagnosed patients with IIH. Case reports were assessed for diagnostic accuracy using Friedman et al.'s revised diagnostic criteria for primary pseudotumor cerebri syndrome. Our primary outcome was the crude prevalence of inappropriate or premature IIH diagnoses. Our secondary outcome was determining if inaccurate IIH diagnoses were associated with variables such as journal subscription model and impact factor, author affiliation, country of origin, and year of publication., Results: A total of 33/185 case reports (17.8%) either incorrectly labelled a patient as having IIH or did not perform all of the investigations necessary to make a diagnosis of IIH. Some of these studies (4.8%) were believed to still represent 'probable' IIH given the clinical presentation, whereas 13.0% of studies were determined to have mislabelled their patients as having IIH. The most common reason that case reports did not meet diagnostic criteria included: a lack of MRV in atypical patient cases (42.4%, n = 14), no papilledema in addition to a lack of characteristic neuroimaging features (33.3%, n = 11), intracranial hypertension being secondary to another documented cause (12.1%, n = 4), normal LP opening pressure in addition to other factors (12.1%,n = 4), no description of neuroimaging (6.1%, n = 2), and abnormal CSF composition (6.1%, n = 2). Case reports that used the term 'IIH' incorrectly had a significantly lower journal impact factor (2.0 vs. 2.6, p = 0.01)., Conclusions: There is a high prevalence of premature or inappropriate diagnoses of IIH in the peer-reviewed case report literature. Adherence to published diagnostic criteria is needed when publishing IIH case reports, and authors are expected to report all relevant data in their report to ensure that an accurate diagnosis is made., (© 2023. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Background: Idiopathic intracranial hypertension (IIH) is a condition that mostly affects obese women of childbearing age but has been reported to be temporally related to new diagnoses of anemia. Despite these reports, the association between anemia and IIH remains controversial. The present study aimed to understand the strength of association between anemia and IIH through a case-control design., Methods: Consecutive IIH patients were recruited from neuro-ophthalmology clinics and matched by age and sex to consecutive patients attending neuro-ophthalmology clinics with diagnoses other than IIH. Complete blood counts (CBCs) were reviewed within 6 months of neuro-ophthalmology presentation, and anemia was diagnosed by hemoglobin of <120 g/L in women and <130 g/L in men. Anemia was classified as microcytic (mean corpuscular volume (MCV) < 80 fL), normocytic (MCV 80-100 fL), and macrocytic (MCV > 100 fL) as well as mild (hemoglobin >110 g/L), moderate (hemoglobin 80-109 g/L), and severe (hemoglobin <80 g/L)., Results: One hundred twenty-three IIH patients and 113 controls were included in the study. More IIH patients than controls had anemia (22.8%, 28/123 vs 10.6%, 12/113, P = 0.01) with an odds ratio of 2.48 (95% CI: 1.19, 5.16). There were no differences in severity of anemia or MCV between IIH patients and controls, with anemia most often being mild (57.1%, 16/28 vs 75%, 9/12) and normocytic (50.0%, 14/28 vs 50.0%, 6/12). However, 17.9% (5/28) of IIH patients had severe anemia compared with zero in the control group. IIH patients had a higher mean platelet count than controls ( P < 0.001), and there were no differences on leukocyte counts., Conclusion: Idiopathic intracranial hypertension patients have a higher prevalence of anemia compared with non-IIH neuro-ophthalmology patients. The anemia was mostly mild and may be explained by other factors related to worse health status and obesity, including low socioeconomic status, poor nutrition, and polycystic ovary syndrome. However, as a CBC is a widely available test and anemia may influence the IIH disease course, we propose that this test be obtained in all patients with new papilledema., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by North American Neuro-Ophthalmology Society.)

Topic: The term "papilledema" is often misused in different ways when describing nonintracranial pressure-related optic disc edema (ODE) or on fundoscopic examination. However, the extent of these errors is unknown among case reports., Clinical Relevance: "Papilledema" refers to ODE secondary to raised intracranial pressure (ICP). Papilledema is a leading reason for referral to neuro-ophthalmologists. In any specialty setting, papilledema must be distinguished from other causes of ODE because it implies a unique pathophysiology and management strategy. If managed inappropriately, papilledema may cause permanent vision loss., Methods: Cross-sectional evidence-based study with protocol registered on Open Science Framework (digital object identifier: 10.17605/OSF.IO/BUQS3). From Ovid MEDLINE and EMBASE, we included case reports with "papilledema" in their title, abstract, or article keywords between January 2011 and March 2022. Studies were graded for the following errors: type 1 (using "papilledema" without evidence of elevated ICP), type 2 (declaring "papilledema" on examination before finding evidence of elevated ICP), type 3 (both errors type 1 and 2), or no error., Results: Among 722 case reports, there was a total of 482 errors (66.8%). There were 12 type 1 errors, 360 type 2 errors, 110 type 3 errors, and 240 studies with no errors. Ophthalmology, neurology, and neurosurgery were the most represented fields, yet only ophthalmology had a low prevalence of errors among all specialties. Regardless, all specialties with at least 10 included reports had a high error prevalence (>30%). Reduced error prevalence was significantly associated with higher impact factor journals, non-open-access publication models, and origin countries with an English official language. Error prevalence was not significantly associated with publication year. Also, the relationship between article citation counts and the prevalence of any type of error did not reach significance. Risk of bias analysis indicated that error occurrence was unrelated to the quality of case report study design., Conclusion: "Papilledema" is widely misused even among ophthalmology case reports. When allowed to propagate, this error can mislead care delivery to patients with ODE. Our results, although limited to case reports, indicate a need for more thorough peer-review standards and neuro-ophthalmology exposure in medical education., (Copyright © 2022 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Background: Lumbar puncture (LP) is considered an essential component of the diagnosis of idiopathic intracranial hypertension (IIH) and ruling out IIH mimics, such as meningeal inflammation and neoplastic disease. Such mimics are unlikely in patients who are systemically well and fit the clinical demographic of IIH. It is important to take into account the risks of performing a LP as patients commonly experience mild adverse effects and infrequently more serious ones including psychological distress. LP can also be difficult to obtain in some health care settings, requiring inpatient admission. We examined the clinical course of a subset of presumed patients with IIH with mild vision loss and papilledema to determine whether LP can be safely deferred in this group., Methods: This was a retrospective study looking at the clinical characteristics, final visual outcome, and diagnosis of patients with presumed IIH and papilledema determined by clinical examination who did not undergo LP. The inclusion criteria included i) no symptoms suspicious for systemic infectious/neoplastic/inflammatory processes, ii) no secondary causes of raised intracranial pressure seen on magnetic resonance imaging/magnetic resonance venography, iii) optical coherence tomography (OCT)-RNFL thickness ≤300 µm, and iv) automated mean deviation (MD) ≤ -5.00 dB v) at least one follow-up visit., Results: A total of 132 eyes of 68 patients (66 female and 2 male) were included in the study. The mean ± SD age was 31.4 ± 10.2 years, and body mass index was 35.1 ± 6.8 kg/m 2 . Systemic symptoms included headache (n = 47), pulsatile tinnitus (n = 28), transient visual obscurations (n = 10), and diplopia (n = 2). Presenting logarithm of the minimum angle of resolution visual acuity was 0.020 ± 0.090, automated MD was -2.23 ± 1.38 dB, and OCT RNFL thickness was 150.8 ± 48.4 µm. Patients were followed for a mean number of 63.3 ± 78.3 weeks. No additional cause of intracranial hypertension was discovered, and all patients remained systemically well. Two patients were started on acetazolamide, and 31 patients lost at least some weight. There was a significant improvement in the automated MD (-1.73 ± 1.74 dB; P < 0.001) and OCT RNFL thickness (128.1 ± 38.6 µm; P < 0.001) at final follow-up. Seventy-six eyes of 38 patients were considered to have resolved papilledema at the final follow-up., Conclusions: Some patients with presumed IIH may not be able to undergo LP because of patient factors such as refusal, failed attempts, or the environment in which neuro-ophthalmologists practice. This study suggests that it may be acceptable to defer LP for patients with suspected IIH who are under the care of a neuro-ophthalmologist with experience in diagnosing and managing IIH. These patients should be systemically well, in a typical demographic for IIH patients, have mild optic disc edema, and preserved visual function. Patients should be informed about the controversial nature of this decision., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by North American Neuro-Ophthalmology Society.)

Background: Idiopathic intracranial hypertension (IIH) is a condition typically affecting young, obese women. Although anemia is recognized as a risk factor of IIH from case reports, their relationship remains controversial as several comparative studies showed no significant association. This study aimed to examine the relationship between anemia and IIH., Methods: MEDLINE, Embase, Cochrane Library, and grey literature were searched to September 2020. Primary studies on patients with diagnoses of anemia of any kind and IIH were included. Primary outcomes included the total number of cases of anemia and IIH. A meta-analysis on the prevalence of anemia in IIH compared with control patients was conducted. Grading of Recommendations, Assessment, Development and Evaluations (GRADE) was used to rate the quality of evidence., Results: Overall, 62 cases and 5 observational or case-control studies were included. Pooled incidence of anemia in patients with IIH was 195/1,073 (18.2%). Patients with IIH (n = 774) had a significantly higher prevalence of anemia compared with controls (n = 230,981) (RR 1.44 [95% confidence interval 1.08-1.92]). Patients were 67.7% females and had a mean age of 22.4 years. The mean opening pressure was 37.9 cmH2O. Anemia was most commonly caused by iron deficiency (51.6%) and aplastic anemia (19.4%). Most patients (59.7%) showed improvement or resolution with anemia treatment only without intracranial pressure-lowering therapy. Evidence was limited because of high risk of reporting bias from the large number of case reports and case-control studies., Conclusions: Anemia is significantly more common in IIH compared with control patients, and case reports suggest a direct relationship. Complete blood counts should be considered in all patients with papilledema, particularly in atypical presentations (male, nonobese, nonperipapillary retinal hemorrhages, prominent risk factor for anemia) or in treatment-refractory IIH., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by North American Neuro-Ophthalmology Society.)

Competing Interests: The authors report no conflicts of interest.

Background: Acute promyelocytic leukemia (APML) is a medical emergency that can initially present with neuro-ophthalmologic signs. Early recognition is crucial, and immediate treatment with all-trans retinoic acid (ATRA) and arsenic trioxide (ATO) can be life-saving. The goal of this study was to describe patients who first presented to neuro-ophthalmology and were subsequently diagnosed with APML., Methods: We retrospectively reviewed consecutive patients seen at a tertiary neuro-ophthalmology centre. Patients with an unknown diagnosis of APML at presentation who subsequently went on to receive this diagnosis were included. Clinical characteristics, neuro-ophthalmologic findings, and outcome were retrieved., Results: A total of 3 patients (2 women and 1 men) with a mean age of 30.7 (range 24-33) years were included in the study. Neuro-ophthalmologic diagnoses at presentation were severe hemorrhagic papilledema related to dural venous sinus thrombosis, hemorrhagic bilateral optic disc edema, and left homonymous hemianopia related to an occipital lobe hemorrhage. At diagnosis, the average hemoglobin was 83.7g/L (range 78-104), and the platelet count was 39.3 × 109/L (range 15-77). All patients were treated with ATRA and ATO. One patient developed papilledema and sixth nerve palsies related to this treatment, which resolved with acetazolamide. Clinical follow-up ranged from 6 to 12 months, and all patients were in clinical remission about systemic APML., Conclusion: Neuro-ophthalmologic symptoms may be the first manifestations of APML, and a complete blood count is an essential test in patients presenting with optic disc edema, especially if hemorrhagic., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by North American Neuro-Ophthalmology Society.)

Abstract: Paracentral acute middle maculopathy (PAMM) is a relatively new optical coherence tomography finding, defined by hyperreflectivity in the inner nuclear layer. In this article, we present a case of a 73-year-old woman who presented with transient vision loss followed by the sudden onset of complete vision loss to counting fingers at 1 foot for one day in the left eye. Dilated examination showed a right cotton wool spot, left pallid optic disc edema, and retinal edema in the distribution of the cilioretinal artery. OCT demonstrated hyperreflective band at the level of the inner nuclear layer, compatible with PAMM. Clinical and laboratory findings were consistent with GCA, for which she was prescribed high-dose oral prednisone, with confirmation of GCA on a subsequent temporal artery biopsy. PAMM may be seen in the context of GCA, and OCT of the macula serves as an important adjunct to define the retinal manifestations of this condition., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by North American Neuro-Ophthalmology Society.)

A 38-year-old man presented to the emergency room with a new generalised tonic-clonic seizure. He also complained of headaches, and brain MRI/magnetic resonance venography (MRV) showed an anterior left temporal encephalocoele with gliosis and brain parenchyma herniating into the left foramen ovale. Ophthalmic examination revealed bilateral optic disc oedema and his lumbar puncture confirmed an elevated opening pressure of 48 cm of water. He was diagnosed with idiopathic intracranial hypertension (IIH) and his papilloedema resolved with weight loss and acetazolamide. Raised intracranial pressure (ICP) can be associated with encephalocoeles and lead to seizures. It is important to screen for papilloedema in these patients as they are at risk for permanent vision loss. This was a unique case in which IIH was diagnosed only after a seizure due to an encephalocoele, which was likely related to chronically undetected raised ICP., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: The authors report no conflicts of interest.

Background/objectives: To compare the yield of magnetic resonance venography (MRV)/computed tomography venography (CTV) in a group of overweight women with incidentally-discovered papilledema and symptomatic intracranial hypertension (IC-HTN) patients without risk factors for dural venous sinus thrombosis (DVST)., Design: Retrospective cohort study., Methods: Retrospective cohort study of female patients between ages 16 and 50 with papilledema, body mass index >25 kg/m 2 and MRV/CTV imaging. Patients were excluded if they had risk factors for DVST. The incidence of DVST and clinical features were retrieved. Patients were divided into those with incidentally-discovered papilledema (Group-1) and those who presented due to symptoms of IC-HTN (Group-2)., Results: One hundred three patients (45 Group-1, 58 Group-2) were included in the study. Group-2 patients were more likely to have pulsatile tinnitus (p = 0.017), transient visual obscurations, and showed a trend towards increased headache (p = 0.058). Group-2 was also more likely to have been treated with acetazolamide (p < 0.01) and undergo lumbar puncture (p = 0.02). DVST was initially reported in four patients (one in Group-1, three in Group-2), but after further review, two cases (one from Group-1, one from Group-2) were found to be false positives. The final two DVST diagnoses were in Group-2 and presented with significant neurological symptoms., Conclusions: DVST was not found among a group of 45 young, overweight women with incidentally-discovered papilledema and without DVST risk factors. We therefore believe it is reasonable practice to perform MRI alone, without dedicated MRV/CTV, in the investigation of young, overweight women with incidentally-discovered papilledema without risk factors for DVST., (© 2020. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Topic: This review presents a case series and systematic review to understand retinal changes in patients with idiopathic intracranial hypertension (IIH) using fundus photography and OCT., Clinical Relevance: IIH is a condition of raised intracranial pressure of unknown cause, usually observed in young, obese women. Ophthalmologic manifestations of IIH such as papilledema and abducens nerve palsy are well recognized, but less common retinal manifestations may occur., Methods: A retrospective institutional chart review (July 2018-March 2020) was performed on consecutive IIH patients. Fundus photographs were obtained followed by neuro-ophthalmology assessment to elicit clinical characteristics and for diagnosis. Patients who met the modified Dandy criteria were included. A systematic review of observational studies was conducted using Ovid MEDLINE and EMBASE to November 17, 2019, to supplement the case series data., Results: Of 144 consecutive IIH clinical patients reviewed, 10 (6.9%) and over 182 patients from the literature showed retinal findings (% in case series, % in literature, respectively): subretinal fluid (SRF; 30,9), chorioretinal folds (30,68), macular exudate (ME; 20,5), choroidal neovascular membrane (CNVM; 10,15), venous stasis retinopathy (VSR; 10,2), choroidal infarction (0,1), and branch retinal artery occlusion (BRAO; 0,1). Eight clinical patients were women (80%), average age was 32.00 ± 13.99 years, body mass index was 40.63 ± 7.43 kg/m 2 , baseline visual acuity (VA) was 0.79 ± 0.30 in both eyes, and visual field (VF) results were -9.89 ± 11.52 dB in both eyes. Among clinical patients, 2 (1 with SRF, 1 with CNVM) had distinctive retina-related VF defects at presentation. Outer retinal abnormalities persisted on OCT in patients after resolution of SRF and papilledema. Surgical treatment (peritoneal shunt) was required for 2 patients (1 with VSR, 1 with SRF); others were treated with weight loss and acetazolamide alone. The patient with significant ME had hypertension that was treated., Discussion: Significant retinal manifestations associated with IIH include CNVM, ME, SRF, VSR, chorioretinal folds, choroidal infarction, and BRAO. These may reduce VA or cause VF defects unrelated to papilledema, emphasizing the importance of a detailed dilated fundus examination. Consultation with a retina specialist is advised in patients with peripapillary CNVM., (Copyright © 2020 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Abstract: A 31-year-old healthy Asian man presented with new headaches and blurred vision. He was found to have bilateral optic disc edema (ODE) and peripapillary subretinal fluid and was initially investigated for causes of raised intracranial pressure. After referral to neuro-ophthalmology, he received a diagnosis of Vogt-Koyanagi-Harada (VKH) disease and his symptoms, ODE, and vision improved promptly with prednisone treatment. HLA typing was performed and returned positive for the HLA-DRB1*04 subtype. Although VKH disease usually presents with granulomatous uveitis or serous retinal detachments, ODE may manifest early in the disease course. Those with ODE in VKH disease are believed to be older and female, but this case demonstrates that these findings were also seen in a young man., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by North American Neuro-Ophthalmology Society.)

Bilateral optic disc edema (ODE) with preserved visual function is typically a result of papilledema, but the causes of this finding in the absence of raised intracranial pressure have not been systematically evaluated. The goal of this study was to determine the causes of bilateral ODE with preserved visual function not related to intracranial hypertension to help in developing a differential diagnosis for this finding. We retrospectively reviewed 221 consecutive patients presenting to a tertiary neuro-ophthalmology practice over a period of 2 years. Four patients met the inclusion criteria with a mean age of 53 years (range 43 to 63), 2 patients were men and 2 were women. The final diagnoses were bilateral optic perineuritis secondary to p-ANCA vasculitis, bilateral incipient non-arteritic anterior ischemic optic neuropathy (NAION), hypertensive emergency, and intermediate uveitis. The patient with NAION developed an inferior altitudinal defect 4 months after presentation, but all other patients maintained normal visual function. The ODE resolved in all cases at a mean follow-up of 4.5 months (range 3 to 6 months). Optic disc edema with preserved visual function may not be related to papilledema, and this series indicates that optic perineuritis, incipient NAION, hypertensive emergency, and intermediate uveitis be considered in the differential diagnosis of these cases., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Objectives: To determine whether optic disc hemorrhages (ODH) and cotton wool spots (CWS) at presentation are associated with worse visual outcomes in pediatric patients with idiopathic intracranial hypertension (IIH)., Methods: Retrospective institutional review of 100 eyes of 50 consecutive pediatric IIH patients (aged 16 years or less) who had baseline optic disc photographs before or within 30 days of their diagnostic lumbar puncture and initiation of medical treatment. Optic disc photographs were independently graded by three ophthalmologists in a standardized manner. Visual function was assessed using visual acuity (VA) and visual field grade (VFG)., Results: At least one ODH was found in 41% of eyes, at least one CWS was found in 27% of eyes, and 20% of eyes had both ODH and CWS. At presentation, Frisén grade was associated with the presence of CWS (p = 0.013) and showed no association with ODH (p = 0.060). When controlling for Frisén grade, ODH and CWS were not associated with worse VA or VFG at final follow-up. Severe ODH were associated with worse VA and VFG at presentation (p < 0.03), but not at final follow-up. Severe CWS at presentation was strongly associated with a worse Humphrey mean deviation of 5.0 dB (95% confidence interval 1.6-8.3) at final follow-up (p = 0.002)., Conclusion: When controlling for the severity of papilledema, ODH do not provide any additional prognostic value in pediatric IIH patients. Frisén grade and severe CWS at presentation were independently associated with worse visual outcomes at the final follow-up.

Use of medications including vitamin A derivatives and tetracyclines have been associated with papilledema and raised intracranial pressure. A 46-year-old woman was referred to neuro-ophthalmology for bilateral optic disc oedema and had a 7-year history of cyclosporine use after renal transplantation. She had preserved visual function and moderate bilateral optic disc oedema. Magnetic resonance imaging and magnetic resonance venography of the brain were normal apart from signs of raised intracranial pressure. Lumbar puncture revealed an elevated opening pressure of 40 cm of water with normal cerebrospinal fluid contents. Nephrology was consulted and cyclosporine was switched to tacrolimus and she was treated with acetazolamide. The papilledema resolved within 1 month of her initial visit. It is important to recognise the role that cyclosporine plays in raising intracranial pressure, especially in patients requiring immunosuppression, such as transplant patients. Tacrolimus is a suitable alternative in these cases., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Background/aims: It remains unclear whether the presence of optic disc haemorrhages (ODH) or cotton wool spots (CWS) at presentation in patients with papilloedema from idiopathic intracranial hypertension (IIH) has prognostic value. The aim of this study was to determine if optic disc appearance at presentation predicts visual outcome in patients with IIH., Methods: Retrospective study of 708 eyes of 360 consecutive patients with IIH who had baseline optic disc photographs before or within 30 days of their diagnostic lumbar puncture and initiation of medical treatment. Optic disc photographs were independently graded by three ophthalmologists in a standardised manner. Visual function was assessed using Snellen converted to logMAR visual acuity, Humphrey mean deviation and visual field grade., Results: At least one ODH was found in 201 (28.4%) eyes, at least one CWS was found in 101 (14.3%) eyes and 88 eyes had both ODH and CWS (12.4%). At presentation, Frisén grade was associated with the presence and severity of ODH and CWS (p<0.001). ODH were associated with a worse visual acuity and CWS were associated with a worse visual field grade and mean deviation at presentation (p<0.05). Frisén grade was associated with worse visual function at presentation and final follow-up (p<0.001). Neither ODH nor CWS at presentation were associated with visual function at final follow-up when controlling for the Frisén grade., Conclusions and Relevance: ODH and CWS at baseline are not independent predictors of final visual function in IIH when controlling for the severity of papilloedema., Competing Interests: Competing interest: None declared., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: Competing interests: None declared.

Competing Interests: Competing interests: None declared.