Risk of serious intracranial pathology in children presenting with acute acquired comitant esotropia.

OBJECTIVE: Acute acquired comitant esotropia (AACE) is defined as a sudden-onset constant nonaccommodative esodeviation. The purpose of this study was to determine the risk of serious intracranial pathology in children presenting with AACE. DESIGN: Retrospective observational cohort study. SETTING: Tertiary care pediatric hospital. METHODS: The study included consecutive children who met the diagnostic criteria for AACE and had neuroimaging at a tertiary care pediatric hospital between 2000 and 2020. Patients were identified by searching the radiology database for all children who underwent neuroimaging for esotropia. The primary outcome measure was the proportion of patients with serious intracranial pathology. Secondary outcomes included risk factors for finding likely causative intracranial pathology and the proportion of patients with incidental findings. RESULTS: A total of 107 patients met the inclusion criteria. Most of the patients (75.7%) had normal neuroimaging. The next most common result was an incidental finding unrelated to the esotropia (18.7%). Five patients (4.7%) had findings with uncertain contribution to esotropia, including 3 cases of type I Chiari malformation. A serious intracranial pathology was found in 1 patient (0.9%) who had a cerebellar medulloblastoma. CONCLUSION: In this large series of pediatric patients with AACE who underwent neuroimaging, there was a small but nontrivial risk of serious intracranial pathology. In these patients, it is advisable either to obtain neuroimaging or to monitor closely for the development of concerning signs or symptoms.

The Relationship Between Choroidal Abnormalities and Visual Outcomes in Pediatric Patients With NF1-Associated Optic Pathway Gliomas.

BACKGROUND: Choroidal abnormalities (CAs) visualized on near-infrared reflectance (NIR) imaging are a new diagnostic criterion for neurofibromatosis type 1 (NF1), but the association between the presence of CAs and visual function remains unknown. This study evaluated the relationship between visual acuity (VA) with the presence, number, or total area of CAs visualized by NIR in children with NF1-associated optic pathway gliomas (NF1-OPGs). METHODS: Patients (<18 years) enrolled in a prospective longitudinal study of children with NF1-associated OPGs from 3 institutions were eligible if they had optical coherence tomography (OCT) of the macula (Heidelberg Spectralis) with ≥1 year of follow-up. The central 30° NIR images were reviewed by 2 neuro-ophthalmologists who manually calculated the number and total area of CAs. VA (logMAR) was measured using a standardized protocol. Cross-sectional associations of presence, number, and total area of CAs with VA, retinal nerve fiber layer thickness (RNFL), and ganglion cell-inner plexiform layer thickness were evaluated at the first and most recent visits using regression models. Intereye correlation was accounted for using generalized estimating equations. RESULTS: Eighty-two eyes of 41 children (56% female) were included. The mean ± SD age at the first OCT was 10.1 ± 3.3 years, with a mean follow-up of 20.4 ± 7.2 months. At study entry, CAs were present in 46% of eyes with a mean number of 2.1 ± 1.7 and a mean total area of 2.0 ± 1.7 mm 2 per eye. At the most recent follow-up, CAs were present in 48% of eyes with a mean number of 2.2 ± 1.8 lesions and a mean total area of 2.3 ± 2.1 mm 2 per eye. Neither VA nor OCT parameters at first and follow-up visits were associated with the presence, number, or total area of CAs (all P > 0.05). CONCLUSIONS: CAs are prevalent but not ubiquitous, in children with NF1-OPGs. Although CAs are a diagnostic criterion for NF1, their presence and size do not appear to be associated with visual function.

Visual outcomes in idiopathic intracranial hypertension in children.

OBJECTIVE: The purpose of this study was to report the clinical characteristics and long-term visual outcomes in a cohort of children with idiopathic intracranial hypertension (IIH). DESIGN: Retrospective, observational cohort study. PARTICIPANTS: Consecutive children who met the diagnostic criteria for definite IIH at a tertiary care pediatric hospital between 2009 and 2020. METHODS: The charts of pediatric patients with IIH were reviewed. The main outcome measure was long-term visual impairment, with an analysis of clinical features by age and risk factors for a poor visual outcome. RESULTS: There were 110 children (75 females) with IIH. At presentation, younger children with IIH were less likely to present with headaches (p = 0.01) and more likely to be asymptomatic (p = 0.03). There was a strong association with female sex (p < 0.001) and higher body mass index (p < 0.001) in adolescents in comparison to younger children. Of the 90 patients with long-term visual outcome data, only 8 (9%) had evidence of mild visual impairment (1 loss of visual acuity, 7 loss of visual field) with no cases of severe visual impairment. On risk factor analysis, the only variable associated with a poor visual outcome was greater severity of papilledema at diagnosis. CONCLUSIONS: In this large series of pediatric IIH, the long-term visual outcomes were favourable, with evidence of mild visual impairment in less than 10% of patients.

Early neuroaxonal injury is seen in the acute phase of pediatric optic neuritis.

BACKGROUND: Thinning of the retinal nerve fiber layer (RNFL) and ganglion cell/inner plexiform layer (GCIPL) occur in the chronic phase after optic neuritis (ON) in children and reflect neuroaxonal injury. The objective of this study was to describe changes in RNFL and GCIPL thickness in the acute phase following pediatric ON. METHODS: Data were collected prospectively from consecutive children presenting with ON as part of an incident acquired demyelinating event. Children with a final diagnosis of multiple sclerosis (n = 9, 10 ON-affected eyes) or monophasic demyelination (n = 16, 25 ON-affected eyes) who underwent spectral-domain optical coherence tomography (OCT) testing within 30 days of symptom onset were included. Standardized visual assessment was performed at presentation and 6-18 months follow-up. OCT measures were compared to those of healthy controls (n = 25, 50 eyes). RESULTS: Median (interquartile range [IQR]) global RNFL thickness was increased in ON-affected eyes (155 µm [114-199 µm]) compared to control eyes (104 µm [98.5-107.5 µm]; p < 0.0001). Compared to controls, fellow eyes demonstrated a reduced temporal quadrant RNFL thickness (59 µm [53-72 µm] versus 71.5 µm [65-81 µm]; p = 0.013) and lower GCIPL thickness (80.5 µm [74-88 µm] versus 87 µm [85-89 µm]; p = 0.003). The ON-affected eyes of children with monophasic demyelination demonstrated a greater global RNFL thickness (183.5 µm [146.5-206 µm]) compared to the ON-affected eyes of children with multiple sclerosis (108.5 µm [95-124 µm]; p = 0.01). OCT measures at presentation did not predict low-contrast visual acuity nor color vision at 6-18 months follow-up. CONCLUSION: Children with multiple sclerosis show less RNFL swelling in their ON-affected eyes at onset compared to children with monophasic demyelination. Lower GCIPL and temporal RNFL thickness in the clinically unaffected eyes of those children with unilateral ON suggests the presence of pre-existing neuroaxonal injury in children presenting with a first episode of ON. This finding may be driven by the subset of children with multiple sclerosis.

Functional-structural correlations in the afferent visual pathway in pediatric demyelination.

OBJECTIVE: We evaluated the relationship of optical coherence tomography (OCT)-measured ganglion cell layer (GCL) and retinal nerve fiber layer (RNFL) thickness to other functional measures of afferent visual pathway competence including high-contrast visual acuity (HCVA) and low-contrast visual acuity (LCVA), visual field sensitivity, and color vision perception in a pediatric population with demyelinating disorders. METHODS: This was a cross-sectional evaluation of 37 children, aged 8-18 years, with pediatric demyelinating disorders (n = 74 eyes), and 18 healthy controls (n = 36 eyes), who were recruited from the University of Toronto, Hospital for Sick Children and the University of Calgary, Alberta Children's Hospital, Canada. A standardized visual battery, including spectral-domain OCT, visual fields, LCVA, and HCVA, was performed in all subjects. RESULTS: Mean RNFL thickness was 26 µm (25.6%) lower in patients with demyelination (76.2 µm [3.7]) compared to controls (102.4 µm [2.1]) (p < 0.0001). Mean GCL thickness was 20% lower in patients as compared to controls (p < 0.0001). Mean GCL and RNFL thickness were strongly correlated (r = 0.89; p < 0.0001), yet in contrast to RNFL thickness, no differences in GCL thickness were noted between optic neuritis (ON) eyes and non-ON eyes of patients. HCVA and LCVA and visual field mean deviation scores decreased linearly with lower RNFL thickness. CONCLUSION: GCL thickness was decreased in patients regardless of history of ON. The retina may be a site of primary neuronal injury in pediatric demyelination.