Association of empiric antibiotic selection and clinical outcomes in hospitalised children with severe orbital infections: a retrospective cohort study.

OBJECTIVE: To determine the association of initial empiric antibiotic regimens with clinical outcomes in hospitalised children with severe orbital infections. DESIGN: Multi-centre observational cohort study using data from 2009 to 2018 clinical records. SETTING: Canadian children's hospitals (7) and community hospitals (3). PATIENTS: Children between 2 months and 18 years hospitalised for >24 hours with severe orbital infections. INTERVENTIONS: Empiric intravenous antibiotic regimen in the first 24 hours of hospitalisation. MAIN OUTCOME MEASURES: Length of hospital stay and surgical intervention using multivariable median regression and multivariate logistic regression, with adjustment for covariates. RESULTS: Of 1421 patients, 60.0% were male and the median age was 5.5 years (IQR 2.4-9.9). Median length of stay was 86.4 hours (IQR 56.9-137.5) and 180 (12.7%) received surgical intervention. Patients receiving broad-spectrum empiric antibiotics had an increased median length of stay, ranging from an additional 13.8 hours (third generation cephalosporin and anaerobic coverage) to 19.5 hours (third generation cephalosporin, staphylococcal and anaerobic coverage). No antibiotic regimen was associated with a change in the odds of surgical intervention. These findings remained unchanged in sensitivity analyses restricted to more severely ill patients. There was a twofold increase in the percentage of patients receiving the broadest empiric antibiotic regimens containing both staphylococcal and anaerobic coverage from 17.8% in 2009 to 40.3% in 2018. CONCLUSIONS: Empiric use of broad-spectrum antibiotics with staphylococci and anaerobic coverage was associated with longer length of stay and similar rates of surgery in children with orbital infections. There is an urgent need for comparative effectiveness studies of various antibiotic regimes.

Abscess volume as a predictor of surgical intervention in children hospitalized with orbital cellulitis: A multicentre cohort study.

IMPORTANCE: Orbital cellulitis with subperiosteal or orbital abscess can result in serious morbidity and mortality in children. Objective volume criterion measurement on cross-sectional imaging is a useful clinical tool to identify patients with abscess who may require surgical drainage. OBJECTIVE: To determine the predictive value of abscess volume and the optimal volume cut-point for surgical intervention. DESIGN: We conducted an observational cohort study using medical records from children hospitalized between 2009 and 2018. SETTING: Multicentre study using data from 6 children's hospitals. PARTICIPANTS: Children were included if they were between 2 months and 18 years of age and hospitalized for an orbital infection with an abscess confirmed on cross-sectional imaging. EXPOSURE: Subperiosteal or orbital abscess volume. MAIN OUTCOME AND MEASURES: The primary outcome was surgical intervention, defined as subperiosteal and/or orbital abscess drainage. Multivariable logistic regression was performed to assess the association of abscess volume with surgery. To determine the optimal abscess volume cut-point, receiver operating characteristic (ROC) analysis was performed using the Youden Index to optimize sensitivity and specificity. RESULTS: Of the 150 participants (mean [SD] age, 8.5 [4.5] years), 68 (45.3%) underwent surgical intervention. On multivariable analysis, larger abscess volume and non-medial abscess location were associated with surgical intervention (abscess volume: adjusted odds ratio [aOR], 1.46; 95% CI, 1.11-1.93; abscess location: aOR, 3.46; 95% CI, 1.4-8.58). ROC analysis demonstrated an optimal abscess volume cut-point of 1.18 mL [AUC: 0.75 (95% CI 0.67-0.83) sensitivity: 66%; specificity: 79%]. CONCLUSIONS AND RELEVANCE: In this multicentre cohort study of 150 children with subperiosteal or orbital abscess, larger abscess volume and non-medial abscess location were significant predictors of surgical intervention. Children with abscesses >1.18 mL should be considered for surgery.

Variation in the Management of Hospitalized Children With Orbital Cellulitis Over 10 Years.

OBJECTIVES: No previous study has examined the management of hospitalized children with orbital cellulitis at both children's and community hospitals across multiple sites in Canada. We describe variation and trends over time in diagnostic testing and imaging, adjunctive agents, empiric antibiotics, and surgical intervention in children hospitalized with orbital cellulitis. PATIENTS AND METHODS: Multicenter cohort study of 1579 children aged 2 months to 18 years with orbital cellulitis infections admitted to 10 hospitals from 2009 to 2018. We assessed hospital-level variation in the use of diagnostic tests, imaging, antibiotics, adjunctive agents, surgical intervention, and clinical outcomes using X2, Mann-Whitney U, and Kruskal-Wallis tests. The association between clinical management and length of stay was evaluated with median regression analysis with hospital as a fixed effect. RESULTS: There were significant differences between children's hospitals in usage of C-reactive protein tests (P < .001), computed tomography scans (P = .004), MRI scans (P = .003), intranasal decongestants (P < .001), intranasal corticosteroids (P < .001), intranasal saline spray (P < .001), and systemic corticosteroids (P < .001). Children's hospital patients had significantly longer length of hospital stay compared with community hospitals (P = .001). After adjustment, diagnostic testing, imaging, and subspecialty consults were associated with longer median length of hospital stay at children's hospitals. From 2009 to 2018, C-reactive protein test usage increased from 28.8% to 73.5% (P < .001), whereas erythrocyte sedimentation rate decreased from 31.5% to 14.1% (P < .001). CONCLUSIONS: There was significant variation in diagnostic test usage and treatments, and increases in test usage and medical intervention rates over time despite minimal changes in surgical interventions and length of stay.

Magnetization transfer saturation reveals subclinical optic nerve injury in pediatric-onset multiple sclerosis.

BACKGROUND: The presence of subclinical optic nerve (ON) injury in youth living with pediatric-onset MS has not been fully elucidated. Magnetization transfer saturation (MTsat) is an advanced magnetic resonance imaging (MRI) parameter sensitive to myelin density and microstructural integrity, which can be applied to the study of the ON. OBJECTIVE: The objective of this study was to investigate the presence of subclinical ON abnormalities in pediatric-onset MS by means of magnetization transfer saturation and evaluate their association with other structural and functional parameters of visual pathway integrity. METHODS: Eleven youth living with pediatric-onset MS (ylPOMS) and no previous history of optic neuritis and 18 controls underwent standardized brain MRI, optical coherence tomography (OCT), Magnetoencephalography (MEG)-Visual Evoked Potentials (VEPs), and visual battery. Data were analyzed with mixed effect models. RESULTS: While ON volume, OCT parameters, occipital MEG-VEPs outcomes, and visual function did not differ significantly between ylPOMS and controls, ylPOMS had lower MTsat in the supratentorial normal appearing white matter (-0.26 nU, p = 0.0023), and in both in the ON (-0.62 nU, p < 0.001) and in the normal appearing white matter of the optic radiation (-0.56 nU, p = 0.00071), with these being positively correlated (+0.57 nU, p = 0.00037). CONCLUSIONS: Subclinical microstructural injury affects the ON of ylPOMS. This may appear as MTsat changes before being detectable by other currently available testing.

Machine learning classification of multiple sclerosis in children using optical coherence tomography.

BACKGROUND: In children, multiple sclerosis (MS) is the ultimate diagnosis in only 1/5 to 1/3 of cases after a first episode of central nervous system (CNS) demyelination. As the visual pathway is frequently affected in MS and other CNS demyelinating disorders (DDs), structural retinal imaging such as optical coherence tomography (OCT) can be used to differentiate MS. OBJECTIVE: This study aimed to investigate the utility of machine learning (ML) based on OCT features to identify distinct structural retinal features in children with DDs. METHODS: This study included 512 eyes from 187 (neyes = 374) children with demyelinating diseases and 69 (neyes = 138) controls. Input features of the analysis comprised of 24 auto-segmented OCT features. RESULTS: Random Forest classifier with recursive feature elimination yielded the highest predictive values and identified DDs with 75% and MS with 80% accuracy, while multiclass distinction between MS and monophasic DD was performed with 64% accuracy. A set of eight retinal features were identified as the most important features in this classification. CONCLUSION: This study demonstrates that ML based on OCT features can be used to support a diagnosis of MS in children.

Factors Associated with Surgery and Imaging Characteristics in Severe Orbital Infections.

OBJECTIVES: To evaluate risk factors associated with surgical intervention and subperiosteal/orbital abscess in hospitalized children with severe orbital infections. STUDY DESIGN: We conducted a multicenter cohort study of children 2 months to 18 years hospitalized with periorbital or orbital cellulitis from 2009 to 2018 at 10 hospitals in Canada. Clinical details were extracted, and patients were categorized as undergoing surgical or medical-only management. Primary outcome was surgical intervention and the main secondary outcome was clinically important imaging. Logistic regression was used to identify predictors. RESULTS: Of 1579 patients entered, median age was 5.4 years, 409 (25.9%) had an orbital/subperiosteal abscess, and 189 (12.0%) underwent surgery. In the adjusted analysis, the risk of surgical intervention was associated with older age (age 9 to <14: aOR 3.9, 95% CI 2.3-6.6; and age 14 to ≤18 years: aOR 7.0, 95% CI 3.4-14.1), elevated C-reactive protein >120 mg/L (aOR 2.8, 95% CI 1.3-5.9), elevated white blood cell count of 12-20 000/µL (aOR 1.7, 95% CI 1.1-2.6), proptosis (aOR 2.6, 95% CI 1.7-4.0), and subperiosteal/orbital abscess (aOR 5.3, 95% CI 3.6-7.9). There was no association with antibiotic use before hospital admission, sex, presence of a chronic disease, temperature greater than 38.0°C, and eye swollen shut. Complications were identified in 4.7% of patients, including vision loss (0.6%), intracranial extension (1.6%), and meningitis (0.8%). CONCLUSIONS: In children hospitalized with severe orbital infections, older age, elevated C-reactive protein, elevated white blood cell count, proptosis, and subperiosteal/orbital abscess were predictors of surgical intervention.

Progressive retinal changes in pediatric multiple sclerosis.

Objectives To determine to what extent acute demyelinating episodes versus chronic degenerative phenomena drive retinal neuroaxonal damage in pediatric acquired demyelinating syndromes (ADS). Methods We acquired optical coherence tomography (OCT) data (follow-up range: 2 weeks - 5 years, at variable intervals from presentation) in pediatric participants who had multiple sclerosis (MS), monophasic ADS, or were healthy. Multivariable mixed effects models were used to assess the association of the number of demyelinating episodes (either optic neuritis [ON], or non-ON relapses) with changes in retinal nerve fiber layer (RNFL) or ganglion cell layer-inner plexiform layer (GCIPL) thickness. Results 64 OCT sans from 23 MS, and 33 scans from 12 monophasic ADS participants were compared with 68 scans from 62 healthy participants. The first ON episode had the biggest impact on RNFL or GCIPL thickness in monophasic ADS (RNFL: -7.9 µm, CI=5.5, p = 0.0056; GCIPL: -8.4 µm, CI=4.4, p = 0.0002) and MS (RNFL: -16 µm, CI = 3.7, p < 10-6; GCIPL: -15 µm, CI = 2.6, p < 10-6). Non-ON relapses were also associated with small but significant retinal thickness reductions in MS (RNFL: -2.6 µm/relapse, CI = 1.4, p = 0.0003; GCIPL: -2.8 µm/relapse, CI = 0.89, p < 10-6). MS participants showed progressive GCIPL thinning independent of acute demyelinating episodes (-2.7 µm/year, CI = 1.9, p = 0.0058). Conclusions We showed a prominent impact of early ON episodes on OCT measures of neuroaxonal structure in patients with ADS. We also demonstrated negative effects of non-ON relapses, and the presence of chronic retinal neurodegenerative changes, in youth with MS.

Prevalence of Choroidal Abnormalities and Lisch Nodules in Children Meeting Clinical and Molecular Diagnosis of Neurofibromatosis Type 1.

PURPOSE: To determine the prevalence of choroidal abnormalities (CAs) and Lisch nodules (LNs) in children who met the clinical diagnostic criteria (CDC) alone and those with a molecularly confirmed diagnosis (MCD) of neurofibromatosis type 1 (NF1), and to ascertain any differences between the groups. METHODS: This was a cross-sectional observational study. All children who met the CDC and/or had MCD of NF1 and underwent eye examination were included. At least two CAs or LNs between the two eyes were set as a threshold to define the presence of either abnormality. Frequencies alongside 95% confidence intervals (CIs) were calculated. The relationship between patient age and the presence of LNs and/or CAs was estimated using logistic regression. RESULTS: The study cohort included 94 patients; CAs (64%) were more prevalent than LNs (41%) (0.22; 95% CI, 0.08-0.36; P = 0.0023). The probability of the presence of LNs was lower than that of CAs across all ages (odds ratio = 0.37; 95% CI, 0.20-0.69; P = 0.00173). CAs were exclusively found in 37% of patients and LNs in 16%; 80% had either CAs or LNs, or both. In the CDC group (n = 41), the difference in prevalence (CAs = 68%, LNs = 51%) did not attain statistical significance (0.17; 95% CI, -0.06 to 0.40; P = 0.18). In the MCD group (n = 53), the difference in prevalence (CAs = 60%, LNs = 34%) was significant (0.26; 95% CI, 0.006-0.47; P = 0.023). CONCLUSIONS: CAs were more frequent than LNs in pediatric NF1 patients regardless of age and MCD status. Combining ophthalmological exams with near-infrared imaging will increase the diagnostic reach in pediatric NF1. TRANSLATIONAL RELEVANCE: CAs detected on near-infrared imaging are objective biomarkers in NF1. They are more prevalent and detected earlier in the pediatric population compared with LNs. Hence, the presence of CAs should be routinely ascertained in children suspected with NF1.

Optic disk anatomical features of children with central retinal vein occlusion.

PURPOSE: To report a case series of children with central retinal vein occlusion (CRVO) showing a high prevalence of crowded optic disks with minimal cupping. METHODS: We retrospectively reviewed the medical records of children diagnosed with CRVO from 2008 to 2019 at a single tertiary care pediatric hospital. Clinical records, fundus photographs, and optical coherence tomography (OCT) images were reviewed. Optic disk anatomical parameters of the unaffected fellow eyes, including OCT-measured optic disk area and vertical cup:disk ratio, were collected and analyzed. RESULTS: Six patients with unilateral CRVO were identified. All patients were female. Age at presentation ranged between 9 and 17 years. Five patients were otherwise healthy, with negative systemic investigations (idiopathic group). The remaining patient had a known systemic risk factor of active Takayasu arteritis. Within the idiopathic group, "disk-at-risk" optic nerve configuration, defined as a cup:disk ratio of 0.2 or smaller, was identified in the fellow eye of all 5 patients. In the unaffected eyes, mean OCT-measured optic disk area was 1.67 ± 0.13 mm2 and mean cup:disk ratio was 0.19 ± 0.12. The patient with Takayasu arteritis had normal OCT disk area of 2.1 mm2 and cup:disk ratio of 0.61. CONCLUSIONS: We observed a high prevalence of anatomical features potentially consistent with a constrictive optic disk configuration in pediatric patients with CRVO.

An Audiovisual 3D-Immersive Stimulation Program in Hemianopia Using a Connected Device.

BACKGROUND Homonymous hemianopia is a loss of conscious vision in one hemifield, strongly affecting everyday life. Audiovisual stimulation programs improve visual perception in the blind hemifield; however, they use large equipment operated in clinical settings. Such treatments require frequent visits at the clinic, hampering the patient's adherence and compliance. In one hemianopia patient, we tested a 4-week dynamic audiovisual rehabilitation program in the stand-alone, remotely controlled, virtual-reality, head-mounted display Oculus Go and measured the effect on visual perception. CASE REPORT A 15-year-old Caucasian male was diagnosed with a right homonymous hemianopia with splitting of central fixation after a traumatic occipital contusion at age 7 months. Visual assessment showed impaired binocular contrast sensitivity and retinal sensitivity. Fixation stability and visual fields were strongly affected. After a 4-week audiovisual rehabilitation program, including 3 hours 20 minutes of stimulation, the contrast sensitivity, fixation stability, and paracentral visual perception were significantly enhanced, improving quality of life. CONCLUSIONS This pioneering work reports the use of virtual-reality in a head-mounted display to provide an audiovisual stimulation protocol for low-vision rehabilitation in a hemianopia patient. Real-time data recording and remote control of the stimulation program demonstrate that such rehabilitation treatment can be performed by the patient at home without interruption of care, decreasing the burden of disease. Beneficial effects on visual function were measured according to clinical guidelines of low-vision assessment. Improvement in visual function and quality of life challenge the prevailing belief that post-acute vision loss is both permanent and unchangeable.

Visual field outcomes in children treated for neurofibromatosis type 1-associated optic pathway gliomas: a multicenter retrospective study.

BACKGROUND: Optic pathway gliomas associated with neurofibromatosis type 1 (NF1-OPGs) may adversely affect visual acuity, but data regarding visual field (VF) outcomes after treatment in children are limited. The purpose of this study was to investigate the effects of NF1-OPGs on VF function in a large cohort of children after treatment with chemotherapy. METHODS: We performed a retrospective, international, multicenter study of VF outcomes in patients treated with chemotherapy for NF1-OPGs. RESULTS: A total of 25 participants underwent VF testing using formal perimetric techniques. At the end of treatment, 19 participants (76%) had persistent VF deficits. Formal VF testing was available for 16 participants (64%) at initiation and completion of treatment. Of the 16 children who underwent VF testing at initiation and completion of treatment, 7 (44%) showed stability of VF changes, 3 (19%) showed improvement of VF function, and 6 (38%) had worsening of VFs. Improvement or worsening of VF outcome did not always correlate with visual acuity outcome. Posterior tumor location involving the optic tracts and radiations was associated with more frequent and more profound VF defects. CONCLUSIONS: In our study cohort, children undergoing initial chemotherapy for NF1-OPGs had a high prevalence of VF loss, which could be independent of visual acuity loss. A larger, prospective study is necessary to fully determine the prevalence of VF loss and the effects of chemotherapy on VF outcomes in children with NF1-OPGs.

Care and outcomes of Canadian children hospitalised with periorbital and orbital cellulitis: protocol for a multicentre, retrospective cohort study.

INTRODUCTION: Skin and soft tissue infections of the eye can be classified based on anatomic location as either anterior to the orbital septum (ie, periorbital cellulitis) or posterior to the orbital septum (ie, orbital cellulitis). These two conditions are often considered together in hospitalised children as clinical differentiation is difficult, especially in young children. Prior studies have identified variation in management of hospitalised children with orbital cellulitis; however, they have been limited either as single centre studies or by the use of administrative data which lacks clinical details important for interpreting variation in care. We aim to describe the care and outcomes of Canadian children hospitalised with periorbital and orbital cellulitis. METHOD AND ANALYSIS: This is a multisite retrospective cohort study including previously healthy children aged 2 months to 18 years admitted to hospital with periorbital or orbital cellulitis from 2009 to 2018. Clinical data from medical records from multiple Canadian hospitals will be collected, including community and academic centres. Demographic characteristics and study outcomes will be summarised using descriptive statistics, including diagnostic testing, antibiotic therapy, adjunctive therapy, surgical intervention and clinical outcomes. Variation will be described and evaluated using χ² test or Kruskal-Wallis test. Generalised linear mixed models will be used to identify predictors of surgical intervention and longer length of stay. ETHICS AND DISSEMINATION: Approval of the study by the Research Ethics Board at each participating site has been obtained prior to data extraction. Study results will be disseminated by presentations at national and international meetings and by publications in high impact open access journals. By identifying important differences in management and outcomes by each hospital, the results will identify areas where care can be improved, practice standardised, unnecessary diagnostic imaging reduced, pharmacotherapy rationalised and where trials are needed.

Demonstration of anatomical development of the human macula within the first 5 years of life using handheld OCT.

PURPOSE: To demonstrate the anatomical development of the human macula using handheld spectral domain optical coherence tomography (SD-OCT) during the first 5 years of life. METHODS: This study is a cross-sectional, observational case series. Thirty-five normal eyes of 35 full-term/late preterm infants and children under 5 years of age were included. Handheld SD-OCT was used to image the macula of each eye. The data were analyzed using the Duke OCT Retinal Analysis Program v17 software. Retinal thickness maps were generated for the total retinal thickness (TRT), the inner retinal layers thickness (IRL), and the photoreceptor layer thickness (PRL). Based on the early treatment diabetic retinopathy study macular map, average thickness measurements were taken at 4 circles centered on the fovea (diameter): the foveal center (0.5 mm), sector 1 (S1) (1 mm), sector 2 (S2) (3 mm), sector 3 (S3) (6 mm). RESULTS: The median age at participation was 24 months (range 5-52 months). The TRT increased throughout the first 5 years of life, and this increase was statistically significant at the foveal center and S1 (p = 0.01, p = 0.016, respectively). The IRL did not show any significant change in thickness from birth and throughout the first 5 years of life. The PRL thickness showed thickening in the first 24 months of age at the foveal center and S1 which was statistically significant at S1 (p = 0.066, p = 0.016, respectively). Interestingly, this PRL thickness increase plateaus beyond 24 months of age. The photoreceptors inner segment/outer segment (IS/OS) band was identified as a distinct layer in all our subjects. CONCLUSION: Our findings conform with the literature that the anatomical development of the macular IRL completes before 5 months of age and hence before the PRL. We also identify 24 months of age as an important developmental milestone for photoreceptors development in the human macula.

VIGABATRIN TOXICITY IN INFANCY IS ASSOCIATED WITH RETINAL DEFECT IN ADOLESCENCE: A Prospective Observational Study.

PURPOSE: The purpose was to determine whether vigabatrin (VGB) (Sabril)-attributed retinal toxicity defined by electroretinogram in early childhood is associated with visual system defect in adolescents after discontinuation of VGB. METHODS: This prospective cross-sectional study included 24 children previously treated with VGB and monitored in early childhood by electroretinogram for VGB-attributed retinal defects. Ten had been diagnosed with VGB-attributed retinal defect (Group I) and 14 had no VGB-attributed retinal defect (Group II). Outcome measures were extent of monocular visual fields using Goldmann kinetic perimetry and RNFL thickness at the optic nerve head, using optical coherence tomography. RESULTS: Of those able to complete testing (6 eyes Group I and 16 eyes Group II), Goldmann results revealed results of visual field loss in Group I and not in Group II. The optical coherence tomography results demonstrated attenuation of the RNFL in all 6 eyes of Group I participants and in only 1 eye of 10 Group II participants. Optical coherence tomography data were nonoverlapping between Group 1 and Group II eyes. CONCLUSION: The VGB-attributed retinal toxicity identified by means of electroretinogram in infancy was associated with visual field loss and RNFL attenuation of the retinal nerve when tested in adolescence.

The role of MR imaging in investigating isolated pediatric nystagmus.

BACKGROUND: The use of MRI in isolated pediatric nystagmus remains a gray area in clinical management. Many clinicians prefer to order an MRI to rule out intracranial pathology despite the lack of clinically significant findings in most cases. OBJECTIVE: To assess the yield of MR imaging in isolated pediatric nystagmus and define a management algorithm to minimize avoidable MRI referrals and streamline MRI protocols. MATERIALS AND METHODS: We reviewed the charts of 148 children who underwent neuro MRI for isolated nystagmus between January 2008 and September 2014. We noted nystagmus onset and clinical characteristics and compared them with the MRI features and visual electrophysiology results. RESULTS: We included 85 boys and 63 girls (total 148, average age at MRI 4.24 ± 4.19 years). Twenty-three (15.5%) children had abnormal intracranial findings on MRI including abnormal signal lesions (4.1%; n=6), Chiari I malformations (3.4%; n=5) and optic pathway glioma (2.0%; n=3). The time of onset of nystagmus was not associated with an abnormal MRI (P=0.2). Seventy children underwent visual electrophysiology testing but this test could not predict abnormality at MRI, either (P=0.12). CONCLUSION: Among children with isolated nystagmus, 15.5% had abnormalities on neuroimaging. Neither clinical characteristics of nystagmus nor the visual electrophysiology results allowed prediction of intracranial pathology. We were unable to formulate a management algorithm for the optimal sequence of investigations (MRI preceding visual electrophysiology or vice versa), but we discuss the use of gadolinium contrast agent and orbital sequences in isolated pediatric nystagmus.

Feasibility and comparison of visual acuity testing methods in children with neurofibromatosis type 1 and/or optic pathway gliomas.

PURPOSE: Longitudinal ophthalmologic clinical trials in young children require multiple visual acuity (VA) testing methods-especially when the subjects have cognitive and developmental delay. This study evaluated the success rate and comparability of two different VA testing methods in children with neurofibromatosis type 1 (NF1) and/or optic pathway gliomas (OPGs). METHODS: Two institutions prospectively enrolled children 10 years or younger with NF1 and/or an OPG. Both Teller grating acuity (TAC) and recognition acuity using the computerized version of the Amblyopia Treatment Study VA testing protocol that limits responses to four letters (H, O, T, or V) were attempted in all subjects. The association of age and diagnosis of NF1 on success rate was analyzed. Differences in grating and recognition acuity were compared. RESULTS: One hundred twenty-seven children met inclusion criteria (median age = 5.58 years). Of 127 subjects, 11 (8.7%) could not complete monocular TAC testing in either eye; 39 (30.7%) could not complete HOTV testing and were younger than those able to complete HOTV testing (mean = 2.9 vs. 7.0 years, respectively; Z = -8.3, P < 0.01). Older age was associated with successful HOTV testing and remained significant in all regression analyses (P < 0.01). The within-subject logMAR values for TAC and HOTV testing results were significantly correlated (r = 0.69, P < 0.01). CONCLUSIONS: Young children with NF1 and/or OPGs were frequently unable to complete recognition acuity testing. These factors are important to consider when designing a clinical trial for children with NF1 and/or OPGs.

Optic pathway gliomas: a review.

Optic pathway gliomas account for 3-5% of all pediatric CNS tumors and represent the most common intrinsic optic nerve tumors. These tumors occur preferentially during the first decade of life and are particularly frequent in children with neurofibromatosis type 1. Although optic pathway gliomas are low-grade tumors, their behavior can be aggressive, and their management is often challenging. Their management includes observation, surgery, chemotherapy and radiation. The role of each modality is discussed as well as current and future developments in treatment, in particular targeted therapies that are currently being investigated.