Outcome In Idiopathic Intracranial Hypertension Research Articles

Clinical profile and treatment outcomes of idiopathic intracranial hypertension: a multicenter study from Korea

BackgroundCurrently, there is a relative lack of detailed reports regarding clinical presentation and outcome of idiopathic intracranial hypertension in Asians. This study aims to describe the clinical features and treatment outcomes of Korean patients with idiopathic intracranial hypertension.MethodsWe prospectively recruited patients with idiopathic intracranial hypertension from one hospital and retrospectively analyzed the medical records of 11 hospitals in Korea. We collected data regarding preceding medical conditions or suspected medication exposure, headache phenotypes, other associated symptoms, detailed neuroimaging findings, treatments, and outcomes after 1–2 and 3–6 months of treatment.ResultsFifty-nine (83.1% women) patients were included. The mean body mass index was 29.11 (standard deviation, 5.87) kg/m2; only 27 patients (45.8%) had a body mass index of ≥ 30 kg/m2. Fifty-one (86.4%) patients experienced headaches, patterns of which included chronic migraine (15/51 [29.4%]), episodic migraine (8/51 [15.7%]), probable migraine (4/51 [7.8%]), chronic tension-type headache (3/51 [5.9%]), episodic tension-type headache (2/51 [3.9%]), probable tension-type headache (2/51 [3.9%]), and unclassified (17/51 [33.3%]). Medication overuse headache was diagnosed in 4/51 (7.8%) patients. After 3–6 months of treatment, the intracranial pressure normalized in 8/32 (25.0%), improved in 17/32 (53.1%), no changed in 7/32 (21.9%), and worsened in none. Over the same period, headaches remitted or significantly improved by more than 50% in 24/39 patients (61.5%), improved less than 50% in 9/39 (23.1%), and persisted or worsened in 6/39 (15.4%) patients.ConclusionOur findings suggest that the features of Asian patients with idiopathic intracranial hypertension may be atypical (i.e., less likely obese, less female predominance). A wide spectrum of headache phenotypes was observed. Medical treatment resulted in overall favorable short-term outcomes; however, the headaches did not improve in a small proportion of patients.

The Vienna idiopathic intracranial hypertension database-An Austrian registry.

Idiopathic intracranial hypertension (IIH) is becoming increasingly more prevalent bearing the risk of visual impairment and affecting the quality of life. Clinical presentation and outcome are heterogeneous. Large, well-characterized cohorts are scarce. To characterize the clinical spectrum, diagnostic findings, therapeutic management, and outcome of IIH. We identified patients with IIH according to modified Friedman criteria treated at our center between 2014 and 2021. The Vienna IIH database is described in detail. Of 113 patients 89% were female (mean age 32.3years). Median body mass index (BMI) was 31.8, with 85% overweight (BMI > 25) and 5% were classified as IIH without papilledema. Headache was present in 84% and showed migraine features in 43%. Median opening pressure in lumbar puncture was 31 cmH2O. Pharmacotherapy (predominantly acetazolamide) was established in 99%, 56% required at least 1 therapeutic lumbar puncture and 13% a surgical intervention. After a median 3.7years follow-up, 57% had achieved significant weight loss, papilledema was present in 59% and headache in 76% (58% improved). Comparing initial presentation to follow-up, perimetry was abnormal in 67% vs. 50% (8% worsened, 24% improved) and transorbital sonography in 87% vs. 65% with amedian optic nerve sheath diameter of 5.4 mm vs. 4.9 mm. Median peripapillary retinal nerve fiber layer thickness decreased from 199 µm to 99 µm and ganglion cell layer volume from 1.13 mm3 to 1.05 mm3. The large representative Vienna IIH cohort characterizes IIH-related symptoms, diagnostic findings, treatment, and outcome emphasizing substantial long-term sequelae of IIH. Future analyses will aim to refine phenotyping and identify factors predicting outcome.

Idiopathic intracranial hypertension: Evaluation of births and fertility through the Hospital Episode Statistics dataset.

Idiopathic intracranial hypertension (IIH) predominantly affects women of reproductive age with obesity, and these women have a distinct profile of hyperandrogenism and insulin resistance. Polycystic ovary syndrome (PCOS) has an established adverse fertility phenotype that typically affects obese women. As IIH may impact reproductive health, we sought to evaluate fertility, gestational complications and pregnancy outcome in IIH. Prospective cohort study from English Hospital Episode Statistics dataset. English hospitals, UK. Women aged 18-45 years seen in English hospitals between 1 April 2002 and 31 March 2019. Patients were required to have an IIH diagnosis and were compared with those with PCOS and general population female controls. Pregnancies resulting in live births, complications of gestational diabetes and pre-eclampsia, and method of delivery. Data was collected from 17 587 IIH, 199633 PCOS and 10947 012 women in the general population. The live birth rate, adjusted for age, was significantly lower among women with IIH (54.1%) than PCOS (67.9%), p< 0.0001 and the general population (57.7%), p< 0.0001. Pre-eclampsia and gestational diabetes risks were higher following a diagnosis of IIH (5.3-fold and 2.7-fold, respectively, p< 0.0001) compared with the general population controls. Following a diagnosis of IIH, elective caesarean section rates were more than twice that of general population (odds ratio [OR] 2.4) and prior to a diagnosis of IIH (OR 2.2). These data indicate there are lower age-adjusted total pregnancy rates, increased risk of pre-eclampsia and gestational diabetes, and a doubling of elective caesarean section rates in those with a diagnosis of IIH.

Course and Predictors of Visual Outcome of Idiopathic Intracranial Hypertension

ABSTRACT Idiopathic intracranial hypertension (IIH) is a clinical syndrome characterised by headache and papilloedema that can lead to significant visual morbidity. There are few studies in the literature about the visual outcome of IIH. We have reviewed the record of 76 patients with IIH according to the modified Dandy criteria. There was a significant improvement in the Humphrey 24-2 mean deviation (MD) in the study eyes (worse affected eye at presentation) in both the medically treated group (+2.0 dB; from −5.60 dB at baseline to −3.60 dB at final follow-up, p < .01) and in the fellow eyes in the medically treated group (+1.70 dB, from −4.40 dB at baseline to −2.74 dB at final follow-up, p < .01). Higher papilloedema grade (beta −0.66, p < .001) and age (p < .02) were inversely correlated with the final visual field MD in the study eye. The visual outcome for the IIH patients in our study was predominantly favourable, but patients with high-grade papilloedema had a worse visual prognosis and required more aggressive treatment.

Outcome of idiopathic intracranial hypertension after laparoscopic sleeve gastrectomy.

Idiopathic intracranial hypertension (IIH) predominantly affects young, obese women and presents with signs and symptoms of increased intracranial pressure, such as headaches and visual impairment. We aim to present our experience in the management of IIH. University Hospital. Obese IIH patients who had a laparoscopic sleeve gastrectomy during the study period (2 years) were included. Data were retrieved from prospectively collected database. Headaches, visual alterations, and medications or interventions used to treat are discussed. The study included 16 obese women with IIH. Mean age was 31 ± 2 years (range, 25-44 yr) and mean body mass index was 46 ± 4 kg/m2 (range, 42-53 kg/m2). Main symptoms and signs were chronic headaches (14), impaired vision (15), vision loss (1), papilledema (6), and field defects in 4 patients. Symptoms were present for a mean of 5 years (4-11). History of medical treatment with carbonic anhydrase inhibitor (acetazolamide) and thecoperitoneal shunting was present in 12 and 9 patients, respectively. Mean lumbar puncture opening pressure was 41.2 ± 21- (range, 30-64) cm water. At 12 months after laparoscopic sleeve gastrectomy, body mass index and percentage excess weight loss were 27.8 ± 1 kg/m2 and 75.2 ± 2%, respectively. Symptoms gradually improved with complete resolution in all but 2 patients (87.5%). The present work emphasizes the role of bariatric surgery in the management of obese patients with IIH. Larger, prospective, controlled studies are needed.

Visual Field Mean Deviation at Diagnosis of Idiopathic Intracranial Hypertension Predicts Visual Outcome.

A robust predictor of visual outcome in idiopathic intracranial hypertension (IIH) would be useful in management, but there is limited information on this point. The purpose of this study was to ascertain whether visual field mean deviation on standard static perimetry performed at diagnosis in a large patient cohort is a reliable predictor of visual outcome. We retrospectively reviewed the automated visual field mean deviations at diagnosis and at final encounter in 79 patients with IIH examined in the neuro-ophthalmology clinics at a single academic medical center from 1999 to 2015. Of the 79 study patients, 66 (84%) entered with visual field mean deviations of -7 dB or better. Of those 66 patients, 59 (89%) had final mean deviations of -4 dB or better and 33 (56%) had final mean deviations of -2 dB or better. The single patient who had an initial mean deviation of -7 dB or better and a poor final mean deviation (-32 dB) was nonadherent to prescribed medication. Of the 13 (21%) patients who entered with mean deviations worse than -7 dB, 11 (85%) ended up with poor visual outcomes, their final mean deviations ranging from -5 dB to -32 dB. Over half of those 13 patients had required surgery for IIH, often within 3 weeks of diagnosis, owing to severe papilledema and visual dysfunction at the time of diagnosis. Based on this retrospective study, patients with IIH who have relatively mild visual dysfunction at diagnosis are likely to have a favorable visual outcome, provided they are adherent to recommended treatment. Many of those with poor visual function at diagnosis will have unfavorable visual outcomes despite aggressive treatment.

Prognostic Factors of Poor Outcome in Idiopathic Intracranial Hypertension (P1.9-008)

Prognostic Factors of Poor Outcome in Idiopathic Intracranial Hypertension (P1.9-008)

Clinical and Neuro-ophthalmologic Predictors of Visual Outcome in Idiopathic Intracranial Hypertension

ABSTRACTDespite the potential of Idiopathic Intracranial Hypertension (IIH) to cause visual morbidity, limited literature is available focussing on predictors of visual outcome in IIH. This study was planned to assess visual morbidity in patients of IIH in terms of clinical and neuro-ophthalmo- logical parameters. In this prospective study of 40 patients of IIH, neuro-ophthalmological parameters were noted in the form of visual acuity, visual field, contrast sensitivity, Retinal Nerve Fibre Layer thickness, and visual evoked potential. Visual outcome was defined in using specific criteria. Final visual outcome of patients was compared with clinical and neuro-ophthalmologic para- meters to determine any correlation. The most common presenting clinical symptoms were headache (85%) and Transient visual obscurations (TVOs) (52.5%). In univariate analysis severity of visual loss, Cerebro Spinal Fluid (CSF) pressures and abnormal Visual evoked potential (VEP) were associated with worse visual outcome or need of aggressive management. When adjusted for severity of visual loss no independent clinical/neuroophthalmic predictor could be established. High CSF opening pressure, worsening vision/papilledema, greater Retinal Nerve Fiber Layer (RNFL) thickness and abnormal VEPs may be some of the alarming signs for physicians, but none of these parameters can be used as an independent predictor for visual outcome in isolation. Visual loss at presentation is probably the most important predictor of the final visual outcome in these patients. This may also suggest that patients presenting in an advanced disease course (with worse visual status) fair badly despite best medical/surgical management. Early diagnosis and prompt management is the cornerstone of management.

Weight reduction for a better visual outcome in idiopathic intracranial hypertension.

To evaluate the correlation between weight reduction and visual outcome in overweight patients with idiopathic intracranial hypertension. Thirty-nine newly diagnosed, overweight (body mass index >25 kg/m2) patients with idiopathic intracranial hypertension were studied retrospectively. All patients underwent medical treatment with acetazolamide, and a weight reduction program was also offered. Patients were grouped according to their compliance with this weight reduction program into the diet-success (Group 1) and diet-failure groups (Group 2). Body mass index, papilledema, visual acuity, and perimetric mean deviation were compared at the end of the 6-month study period. Groups 1 and 2 did not differ regarding the baseline mean body mass index (32.63 ± 5.61, 32.35 ± 5.06 kg/m2), visual acuity (0.080 ± 0.13, 0.130 ± 0.24 logMAR), perimetric mean deviation (-9.978 ± 0.68, -12.86 ± 8.91), or papilledema grade (2.94 ± 0.22, 2.90 ± 0.30), respectively (p>0.05). During the 6 months' follow-up, Group 1 patients, who complied with both medical and diet therapy, improved significantly in all parameters, including body mass index (p<0.001), visual acuity (p=0.001), perimetric mean deviation (p=0.016), and papilledema grade (p<0.001). Conversely, Group 2 patients, who only underwent medical therapy, improved only in papilledema grade (p<0.001). However, coincident development of optic disc pallor was observed in three patients. Further, they also had significant loss in visual acuity (p=0.047) during the study period. Weight reduction combined with medical treatment is associated with significantly better improvement in visual acuity, visual field, and papilledema in idiopathic intracranial hypertension patients. Compliance with an efficient diet program should be encouraged in overweight patients with idiopathic intracranial hypertension.

Optic disc haemorrhages at baseline as a risk factor for poor outcome in the Idiopathic Intracranial Hypertension Treatment Trial

BackgroundThe risk of optic disc haemorrhages on visual outcome in idiopathic intracranial hypertension (IIH) is unknown. We report the type and frequency of optic disc haemorrhages and other funduscopic abnormalities...

Clinical and neuro-ophthalmologic predictors of visual outcome in idiopathic intracranial hypertension (IIH)

Clinical and neuro-ophthalmologic predictors of visual outcome in idiopathic intracranial hypertension (IIH)

Intracranial idiopathic hypertension: 1-year follow-up study.

Standard guidelines for ongoing management, as well as definitive data about the long-term course of idiopathic intracranial hypertension (IIH) are not available. The aim of this study was to compare several clinical and instrumental variables as assessed at the time of diagnosis and then after 1 year in a sample of IIH patients. A total of 21 patients were studied. Our results confirmed that headache and TVO are the most frequent symptoms in IIH patients, and that overweight is a very common feature. A trend towards a favorable outcome in patients followed for 1 year and treated by usual medical therapy was found: intracranial pressure was lower at follow-up; improvement of headache and transient visual obscurations, as well as of papilledema, was reported in most patients. On the other hand, neuroradiological findings (such as empty sella, perioptic subarachnoid space distension, narrowing of the transverse sinuses) were substantially stable at follow. These findings may be relevant for future research as far as understanding the role of different clinical and instrumental findings as diagnostic items as well as predictors of outcome in IIH.

Meningoceles in Idiopathic Intracranial Hypertension

MRI abnormalities have been described in patients with increased intracranial pressure (ICP), including in those with idiopathic intracranial hypertension (IIH). Spontaneous CSF-filled outpouchings of the dura (meningoceles) and secondary CSF leaks can occur from elevated ICP in patients with IIH; however, few studies have evaluated these findings. Our objective was to evaluate the frequency of spontaneous intracranial meningoceles among IIH patients and determine their association with visual outcome. We performed a retrospective case-control study of consecutive IIH patients between 2000 and 2011 who underwent MRI that included T2-weighted imaging. Demographics, presenting symptoms, CSF opening pressure, and visual outcome were collected for the first and last evaluations. Control subjects included patients without headache or visual complaints who had normal brain MRI results. Stratified analysis was used to control for potential confounding by age, sex, race, and body mass index. We included 79 IIH patients and 76 control subjects. Meningoceles were found in 11% of IIH patients versus 0% of control subjects (p<0.003). Prominent Meckel caves without frank meningoceles were found in 9% of IIH patients versus 0% of control subjects (p<0.003). Among IIH patients, the presence of meningocele or prominent Meckel caves was not associated with demographics, symptoms, degree of papilledema, CSF opening pressure, visual acuity, or visual field defect severity. Meningoceles are significantly more common in IIH patients than in control subjects and can be considered an additional imaging sign for IIH. Meningoceles are not, however, associated with decreased CSF opening pressure or better visual outcome in IIH.

Association of MRI Findings and Visual Outcome in Idiopathic Intracranial Hypertension: Saindane A, Bruce B, Riggeal B, Newman NJ, Biousse V. AJR Am J Roentgenol 2013;201:412–8

Association of MRI Findings and Visual Outcome in Idiopathic Intracranial Hypertension: Saindane A, Bruce B, Riggeal B, Newman NJ, Biousse V. AJR Am J Roentgenol 2013;201:412–8

Association of MRI Findings and Visual Outcome in Idiopathic Intracranial Hypertension

Patients with idiopathic intracranial hypertension (IIH) have elevated intracranial pressure (ICP) without an identifiable cause. The clinical course is variable, resulting in irreversible vision loss in some and a benign course in others. Although MRI findings have been described in IIH, their association with visual outcome has not been evaluated to date. Forty-six patients with IIH underwent funduscopic evaluation, visual field testing, lumbar puncture with opening pressure (OP) measurement, and MRI. Patients were stratified into the following groups by visual outcome: group 1, no vision loss (n = 28); group 2, some vision loss (n = 10); and group 3, severe vision loss (n = 8). MRI findings in the orbits, pituitary gland, and optic canals and the frequency of skull base cephaloceles and of transverse sinus (TS) stenosis were assessed by a reviewer blinded to the patients' visual outcome. Demographic, clinical, and MRI findings were evaluated for association with visual outcome. Patients in group 3 (worst visual outcome) were significantly younger (p = 0.03) and had higher OP (p = 0.04) than patients in the other groups. There were no significant differences in sex, race, or body mass index. Despite worse visual outcomes and sometimes fulminant vision loss, there were no differences in the frequency of orbital MRI findings or TS stenosis, optic canal diameter, and pituitary appearance among the three groups. Group 3 had significantly lower cephalocele frequency than the other groups (p = 0.04). Although MRI findings may suggest elevated ICP and the diagnosis of IIH, they are not predictive of visual outcome in patients with IIH.

Idiopathic intracranial hypertension in pediatric population: Case series from India

Idiopathic intracranial hypertension (IIH) is a well described entity in adults. In pediatric age group the presentation of disease can vary depending on the age of patients and is less frequently reported. The aim of this study is to describe the clinical features, investigations, treatment and outcome of IIH in pediatric population (age <18 years). This retrospective hospital based study was carried out on 25 children with diagnosis of IIH based on modified Dandys criteria. Their clinical, investigation, treatment, outcome and follow-up for 2 year period were analyzed. Out of the 25 children, the youngest child was 4-month-old infant. The commonest symptom was headache (76%) followed by vomiting and papilledema (72%). The mean cerebrospinal fluid (CSF) pressure was 330 mm of H 2 O. In Infants irritability and bulging anterior fontanelle was seen. A total of 24 patients showed a complete resolution of symptom. None of patient had recurrence over a period of 2 years follow-up. IIH can present at any age group. This is the largest series of IIH reported in pediatric population in India. The clinical features are similar to adult patients except in infants. Absence of papilledema does not exclude the diagnosis of IIH. CSF pressure monitoring is needed in suspected cases of IIH. Early and prompt treatment can prevent deficits.

Clinical course of idiopathic intracranial hypertension with transverse sinus stenosis

Transverse sinus stenosis (TSS) is common in idiopathic intracranial hypertension (IIH), but its effect on the course and outcome of IIH is unknown. We evaluated differences in TSS characteristics between patients with IIH with "good" vs. "poor" clinical courses. All patients with IIH seen in our institution after September 2009 who underwent a high-quality standardized brain magnetic resonance venogram (MRV) were included. Patients were categorized as having a good or poor clinical course based on medical record review. The location and percent of each TSS were determined for each patient, and were correlated to the clinical outcome. We included 51 patients. Forty-six patients had bilateral TSS. The median average percent stenosis was 56%. Seventy-one percent of patients had stenoses >50%. Thirty-five of the 51 patients (69%) had no final visual field loss. Eight patients (16%) had a clinical course classified as poor. There was no difference in the average percent stenosis between those with good clinical courses vs those with poor courses (62% vs. 56%, p = 0.44). There was no difference in the percent stenosis based on the visual field grade (p = 0.38). CSF opening pressure was not associated with either location or degree of TSS. TSS is common, if not universal, among patients with IIH, and is almost always bilateral. There is no correlation between the degree of TSS and the clinical course, including visual field loss, among patients with IIH, suggesting that clinical features, not the degree of TSS, should be used to determine management in IIH.

Does the Presence of Transverse Sinus Stenosis (TSS) Influence the Clinical Presentation and Outcome of Idiopathic Intracranial Hypertension (IIH) (P07.266)

Objective: To evaluate differences in characteristics and visual outcomes between IIH patients with and without TSS. Background TSS is common in IIH, but its effect on the course and outcome of IIH is unknown. Design/Methods: All IIH patients seen in our institution between 9/2009 and 9/2011 who underwent brain MRV or CTV were included. Patients were separated into two groups based on the presence or absence of TSS. Patient characteristics, presenting symptoms/signs, and visual outcomes were recorded. All visual fields (VF; HVF/GVF) were graded as [1]=normal; [2]=blind spot enlargement; [3]=nasal/temporal defect; or [4]=diffuse constriction. Univariate statistics and linear regression were applied. Results: 134 patients were included: 49 with TSS (48 bilateral), 85 without TSS. There were no differences between the two groups with respect to mean age (29vs.27years, p=0.26), black race (53%vs.42%, p=0.23), female sex (92%vs.84%, p=0.17), mean BMI (36vs.37, p=0.38), or mean CSF-OP (38cm vs. 38 cm, p=0.99). Headache frequency in the two groups was similar at baseline (82%vs.81%, p=0.95) and at last follow up (41%vs.50%, p=0.36). Grade of papilledema, visual acuity, and VF were similar at baseline, but initial HVF mean deviation (HVF-MD) was better in the TSS group than in the no-TSS group (-4.8vs.-6.8, p=0.08). There was no medical or surgical therapy difference by TSS status. At last follow-up, grade of papilledema, visual acuity, and VF were similar. However, the no-TSS group had significantly more improvement in VF-grade (-0.60vs-0.28, p=0.049) and HVF-MD (3.0vs.1, p=0.026) than the TSS group. Linear regression suggested that the worse HVF-MD among no-TSS patients at baseline better predicted their degree of improvement than the patient9s TSS status. Conclusions: While TSS was found in 37% of IIH patients, the presence of TSS did not have a substantial impact on outcomes. Our findings do not support routine TSS stenting in IIH. Disclosure: Dr. Riggeal has nothing to disclose. Dr. Bruce has nothing to disclose. Dr. Saindane has nothing to disclose. Dr. Kelly has nothing to disclose. Dr. Ridha has nothing to disclose. Dr. Newman has received personal compensation for activities with Biogen Idec. Dr. Biousse has nothing to disclose.

Clinical profile, evaluation, management and visual outcome of idiopathic intracranial hypertension in a neuro-ophthalmology clinic of a tertiary referral ophthalmic center in India

Aim:To discuss the clinical features and management of patients who presented with optic disc edema and had features of presumed idiopathic intracranial hypertension (IIH).Materials and Methods:Case series of all patients diagnosed to have IIH from January 2000 to December 2003 in the neuro-ophthalmology clinic of a tertiary referral ophthalmic institution, were retrospectively analyzed. Analysis was done for 50/106 patients who fulfilled modified Dandy's criteria and had optic disc edema and a minimal follow-up period of two years.Results: Most (40/50, 80%) of the patients were females and the mean age of presentation for all the 50 patients was 32.89 years. Chief complaints were headache in 38 (76%) patients, 24(48%) patients had transient visual obscuration, 24 (48%) patients had reduced vision, 15 (30%) patients had nausea, vomiting, 4(8%) patients had diplopia. Bilateral disc edema was seen in 46 (92%) patients and unilateral disc edema in 4 (8%) patients. 60 eyes had enlarged blind spot as the common visual field defect. Neuroimaging revealed prominent perioptic CSF spaces in 14 patients and empty sella in three patients. CSF opening pressure was 250–350 mm H2O (water) in 39 patients and was >350 mm H2O in 11 patients. Medical treatment was started for all patients; whereas 35 [70%] patients responded, 15 [30%] patients had to undergo LP shunt.

Idiopathic intracranial hypertension in pediatric patients

To evaluate retrospectively the features, treatment, and outcome of idiopathic intracranial hypertension (IIH) in children. Nine patients, 15 years and younger, diagnosed with IIH. Inclusion criteria were papilledema, normal brain computer tomography or magnetic resonance imaging, cerebrospinal fluid pressure greater than 250 mm H(2)O, normal cerebrospinal fluid content, and a nonfocal neurologic examination except for sixth nerve palsy. Of the nine patients, eight were girls. Five girls were overweight and one boy was obese. The most common presenting symptom was headache (5 patients). Diplopia or strabismus did not occur in our group. Visual field abnormalities were present in all eyes, and severe visual loss resulting in light perception vision occurred in both eyes of one patient. Eight patients were treated medically with acetazolamide alone, and one girl needed a combination of acetazolamide and corticosteroids. This girl also required optic nerve sheath decompression surgery. Resolution of papilledema and recovery of visual function occurred in all patients. Idiopathic intracranial hypertension in prepubertal children is rather uncommon. Prompt diagnosis and management are important to prevent permanent visual loss.