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PurposeBrain tumors are the leading cause of death from childhood cancer. Although overall survival has improved due to earlier detection, better therapies, and improved surveillance, visual dysfunction and impaired vision-related quality-of-life (VR-QOL) are often unrecognized in children. This project investigated VR-QOL in pediatric brain tumor patients.MethodsWe evaluated visual impairment and quality-of-life (QOL) in a quality improvement project at one tertiary care center. Patients ≤ 18, greater than 6 months from diagnosis of brain tumor, excluding intrinsic anterior visual pathway tumors, underwent standardized neuro-ophthalmologic examination. Health-related QOL (HR-QOL) (PedsQL Brain Tumor Module) and VR-QOL questionnaires [CVFQ (Children’s Visual Function Questionnaire) in children < 8, and EYE-Q in children 8–18] were obtained from patients and parents.ResultsAmong 77 patients, craniopharyngiomas (n = 16, 21%) and astrocytomas (n = 15, 20%) were the most common tumors. Among 44/77 (57%) visually impaired children, 7 (16%) were legally blind. Eye-Q median score was 3.40 (interquartile range 3.00–3.75), worse than average scores for normal children. Eye-Q score decreased 0.12 with every 0.1 increase in logMAR visual acuity (p < 0.001). Patients who were legally blind had a significantly lower Eye-Q score than those who were not [0.70 vs. 3.44 (p < 0.001)]. Cognitive HR-QOL scores decreased 1.3 for every 0.1 increase in logMAR visual acuity (p = 0.02).ConclusionsPediatric brain tumor patients’ vision, HR-QOL, and VR-QOL were often severely affected even when tumors were considered cured. Visual acuity and legal blindness correlated with VR-QOL. Systematic neuro-ophthalmologic examinations in pediatric primary brain tumor patients are necessary to facilitate early preventative and corrective ophthalmologic interventions.

Neurological examination was normal. Because of concern for papilledema and raised intracranial pressure, she underwent MRI of the brain, which was initially reported as normal, but on further review showed diffuse sulcal fluid attenuated inversion recovery (FLAIR) nonsuppression with leptomeningeal enhancement within the posterior fossa (Figure 2). Primary leptomeningeal lymphoma (PLML) is a type of primary central nervous system lymphoma (PCNSL), a rare neoplasm that arises as solitary or multifocal lesions in the cerebral hemispheres in 60% of cases.1,2 While most leptomeningeal lymphomas are metastatic from another primary lymphoma,3,4 PLML without parenchymal or systemic disease accounts for 7% of all PCNSLs.1 The largest case series of PLML comprised 48 patients and found that most PLMLs, like other PCNSL, are non-Hodgkin’s lymphoma of B cell lineage.3 Most patients with PLML present with multifocal symptoms, ranging from cranial nerve palsies, lumbosacral radiculopathies, to less common ones like headaches and ataxia.3 Isolated intracranial hypertension was not described in this case series.3 Because of its wide range of presentations, PLML is commonly misdiagnosed as stroke, meningitis, polyradiculopathies, and rarely idiopathic intracranial hypertension (IIH).5 Our patient was an obese woman who presented with headache, nausea, transient visual obscurations, and papilledema, which suggested IIH.6 Symptoms and signs of IIH, if present, may only reflect those of generalized elevated intracranial pressure.7 However, in our patient, there were several atypical features that favored another diagnosis, including the rapid onset of symptoms, dot hemorrhages in the retina outside of the peripapillary area, and MRI findings of leptomeningeal enhancement. Five previously published cases of PLML have reported symptoms of intracranial hypertension.8-12 In these cases, PLML caused a clinical syndrome consistent with IIH, which is defined as intracranial hypertension without mass lesions, dural venous sinus thrombosis, or other secondary cause.13 Our case was unique since previous cases reported papilledema and intracranial hypertension in men,8,9,11 had significant associated neurological symptoms such as obtundation,10 significantly compromised visual function,12 or the main location of disease was in the spine.9 Patients may present with only blurred vision without headache5 and initial MRI brain may be normal.6 The importance of CSF studies in cases atypical for IIH including male patients was emphasized by a case where a 49-year-old man with 6 weeks of headaches and transient visual obscurations with a normal MRI brain underwent transverse sinus stenting without LP.6 It was only 2 years later when he represented with seizures, balance problems, and progressive vision loss that the diagnosis of PLML was established. Leptomeningeal enhancement on MRI was found in 74% of patients with PLML, most commonly in the spinal cord and nerve roots.3 Patients with PLML usually have high protein and low glucose on CSF studies.3 CSF detection of PLML can be increased by doing a combination of cytology (malignant lymphocytes in 67% of PLML cases), flow cytometry (monoclonal population in 80% of cases), and receptor gene rearrangement studies (positive in 71% of cases).3 Serial LPs are indicated in patients who are suspected of PLML as initial CSF studies can be negative.9,11 If CSF remains nondiagnostic, a leptomeningeal biopsy is required.3 PLML is usually a diagnosis of exclusion, and it was ruled in for our patient after PET-CT scan showed no systemic involvement, MRI of brain found no parenchymal involvement, and bone marrow biopsy was negative for lymphoma.

Background/aimsIt 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.MethodsRetrospective 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.ResultsAt 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 relevanceODH and CWS at baseline are not independent predictors of final visual function in IIH when controlling for the severity of papilloedema.

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.

Idiopathic intracranial hypertension (IIH) is a condition of elevated intracranial pressure (ICP) of unknown cause. In adults, this is associated with female gender and obesity. Symptoms may include headaches, diplopia, pulsatile tinnitus, and vision loss due to papilledema. Children may develop IIH, although the demographics of prepubescent children with IIH appear to be distinct from older children, being independent of gender and obesity. Recent diagnostic criteria have been established specifically for children with IIH, including a different normal range of ICP in children. Treatments may include weight loss, carbonic anhydrase inhibitors, or surgical procedures. The visual prognosis of children treated appropriately is generally good.