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Background: Optic Pathway Gliomas (OPG) are the most common brain tumor in Neurofibromatosis 1 patients (NF1). They are found along the optic pathway and may involve the optic nerves, chiasm, retro-chiasmatic structures, and the optic radiations. NF1 associate OPG (NF1-OPG) have variable presentation, disease course and response to treatment. The optimal management is patient-specific and should be tailored by a multidisciplinary team. Age, sex, histology, and molecular markers may be important factors in the individualized decision-making process. Chemotherapy is the first-line treatment in cases of progressive tumors, and visual preservation is the main goal of treatment. Purpose: In this paper we will review the disease, practical management, and recent advances of NF1-OPG. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this paper is to provide novel insight into the rare pediatric meningiomas. Methods: We retrospectively analyzed pediatric surgical cases of meningioma during 2002 to 2017 in our institution. The preoperative, intraoperative, and the postoperative status were collected to find any unique features that has not reported in the past. Results: Nine surgeries out of 5 patients were identified. The mean age was 7 years old (range 1–14 years old). Four patients were females. The mean tumor diameter was 52 mm (range 23–81 mm). The tumor locations were optic nerve sheath, Sylvian fissure, parasagittal, trigone of the lateral ventricle, and cerebellopontine angle. The Sylvian fissure meningioma without dural attachment (MWODA) was found in a 15-month-old female. A relapsed parasagittal meningioma showed regression in histological grade and residual tumor demonstrated spontaneous regression. In the initial surgeries, Simpson grade 1 resection was achieved in 2 cases. The pathological diagnoses were 1 meningothelial, 1 metaplastic, 2 atypical, and 1 clear cell meningiomas. The mean postoperative follow-up period was 71 months. Three patients experienced recurrence of the tumor. At the latest follow-up, all patients were free of radiological tumor recurrence or regrowth with a mean follow-up of 4 years (range 1–6.9 years). All patients were in the modified Rankin scale of 0–1. Conclusions: MWODA is not considered to be rare in pediatric meningioma and should be included in the differential diagnosis. We presented a histologically regressed relapsed meningioma, which spontaneously regressed after subtotal resection. In the case of recurrent meningioma, surgical resection and adjuvant radiation therapy could be effective for long-term control of the tumor. [ABSTRACT FROM AUTHOR]

Introduction: Secondary craniosynostosis subsequent to shunting is one of the late complications of ventricular shunt placement in the early childhood. Several interventions have been used to treat high intracranial pressure associated with this condition. This study aimed to evaluate the patients' clinical symptoms and head circumference before and after a method of decompressive craniotomy, coined as external–internal cranial expansion (EICE). Methods: A retrospective study was conducted, and the patients who had undergone EICE for the treatment of post-shunt craniosynostosis between 2010 and 2020 were enrolled. This approach was a combination of a hinge multiple-strut decompressive craniectomy and internal cranial flap thinning by drill. Data, extracted from medical records, were used to evaluate the patients' symptoms and head circumferences before and 12 months after surgery. Results: A total of 16 patients were enrolled in the study, of which eight were females. Before the surgery, 9 patients (56.2%) suffered from visual impairment, and all had intractable headache. Papilledema was recorded in all, with 3 cases having optic disc paleness. After cranial expansion, only two patients had headaches, diagnosed as migraine-type and psychosomatic headaches, respectively. In two patients, progressive visual impairments got worsening after surgery, which would be due to severe preoperative optic nerve atrophy. Patients' head circumferences significantly increased after the surgery (mean of 48.97 ± 4.28 cm vs. 45.78 ± 4.31 cm; P value < 0.0001). Conclusion: In lower resource countries, where newer technologies like distraction osteogenesis is not easily available, external–internal cranial expansion can be considered an effective alternative for patients with post-shunt craniosynostosis. [ABSTRACT FROM AUTHOR]

Introduction: Plexiform neurofibromas (PNF) in neurofibromatosis type 1 (NF1) are usually diagnosed in childhood and can grow rapidly during this period. In 10% of patients, PNF involve the orbital-periorbital area and may cause visual problems including glaucoma, visual loss from amblyopia (deprivational, strabismic, or refractive), optic nerve compression, or keratopathy. Ptosis, proptosis, and facial disfigurement lead to social problems and decreased self-esteem. Complete surgical removal involves significant risks and mutilation, and regrowth after debulking is not uncommon. Inhibitors of the RAS/MAPK pathway have recently been investigated for their activity in PNF. We administered the oral MEK inhibitor trametinib to five young children with NF1 and PNF of the orbital area, with visual compromise and progressive tumor growth; and followed them clinically and by volumetric MRI. Methods: Treatment was initiated at a mean age of 26.8 months (SD ± 12.8) and continued for a median 28 months (range 16–51). Doses were 0.025 mg/kg/day for children aged > 6 years and 0.032 mg/kg/day for those aged < 6 years. Results: Volumetric MRI measurements showed a reduction of 2.9–33% at 1 year after treatment initiation, with maximal reductions of 44% and 49% in two patients, at 44 and 36 months, respectively. No change in visual function was recorded during treatment. One child reported decreased orbital pain after 2 weeks; and another, with involvement of the masseters, had increased ability to chew food. Toxicities were mostly to skin and nails, grades 1–2. Conclusions: Trametinib can decrease tumor size in some young children with orbital PNF and may prevent progressive disfigurement. [ABSTRACT FROM AUTHOR]

Purpose: Intracranial pressure (ICP) > 20 mmHg is associated with worse outcomes in children. The gold standard for monitoring ICP is invasive, has complications such as bleeding and infection, and may not be available in resource limited areas. Ultrasound of the optic nerve sheath diameter (ONSD) has been studied as a noninvasive way to evaluate for elevated ICP in adults. Its utility in pediatrics remains unclear. Methods: A prospective study was performed in a pediatric intensive care unit in children ≤ 18 years old. ONSD ultrasound was performed using a 13–6 MHz linear probe with the patient's invasively measured ICP simultaneously recorded. Linear mixed effects models were used to evaluate the association between ONSD and ICP. Results: One hundred thirty-six measurements were obtained from 16 patients. ONSD was not significantly associated with ICP (p = 0.51). A ROC curve assessing ONSD to determine elevated ICP > 20 mmHg had an area under the curve of 0.52 (95%CI = 0.32–0.72). There was no difference in measurements obtained between the left and right ONSD at the same time (p = 0.82). Conclusions: In conclusion, this study demonstrated no association between ONSD measurement and invasively monitored ICP in critically ill children. [ABSTRACT FROM AUTHOR]

Optic pathway glioma (OPG) is a common and significant complication of neurofibromatosis 1 (NF-1) that might lead to vision loss. The main reason to treat OPG is to preserve vision. Tumor location along the visual pathway largely dictates the presenting signs and symptoms. Clinical ophthalmic evaluation is focused on optic nerve functions including evaluation of pupils' reaction to light, visual acuity, color vision, and visual field, as well as optic nerve appearance. An important relatively new ancillary test is optic coherence tomography (OCT) that measures the volume of retinal nerve fiber layer around the optic nerve and the ganglion cell layer-inner plexiform layer (GCL-IPL) of the macula, both proved to be strongly associated with losing vision in OPG. Accurate evaluation of vision functions plays a critical role in the decision of treatment. In this review, we describe the ophthalmological assessment including new biomarkers in clinical use. We also outline prognostic factors and current recommendations for surveillance and indications for treatment. [ABSTRACT FROM AUTHOR]

Purpose: Measurement of optic nerve sheath diameter (ONSD) with ocular ultrasonography (USG) is a noninvasive technique that can be readily used to determine clues of increased intracranial pressure. In this study, we aimed to determine the role of optic nerve sheath diameter measurements in the diagnosis and follow-up of pediatric patients with idiopathic intracranial hypertension (IIH). Methods: Eight patients with a diagnosis of IIH with a median age of 11.7 (range 4.5–17) years were examined prospectively. During follow-up, orbital ultrasonography (USG) was performed immediately prior to lumbar puncture (LP) and at 24 h, at 1 week, and between 1 and 18 months after LP. Cranial MRI examinations and automated visual field assessments were performed at baseline and at 3 months, and both measurements were compared with each other. Results: The mean cerebrospinal fluid opening pressure (37.75 ± 12.64 cm H2O) and the mean ONSD (5.94 ± 0.46 mm) were correlated. The median follow-up was 16 (range, 12–18 months), and ONSD regressed gradually consistent with clinical and radiologic improvement during follow-up. Conclusions: To the best of our knowledge, this is the first prospective pilot study performed on pediatric patients with IIH using orbital USG for ONSD measurements. Despite the small sample size, the present study indicated that orbital USG may be used as a promising noninvasive tool to diagnose increased intracranial pressure and for monitoring treatment efficacy in this special patient population. [ABSTRACT FROM AUTHOR]

Introduction: The goal of this study was to evaluate the utility of orbital ultrasonography and magnetic resonance imaging in the diagnosis of idiopathic intracranial hypertension (IIH). Method: We reviewed the medical records of patients referred to our department for suspected IIH. Results: Seven children were diagnosed with IIH. Nine children revealed pseudopapilledema by optic coherence tomography and/or orbital ultrasonography. When the axial sequences were reexamined, patients with papilledema had optic nerve sheath (ONS) enlargement (6.62 ± 0.70 mm); patients with pseudopapilledema had ONS diameter as 4.62 ± 0.64 mm. There was a significant correlation between the CSF opening pressure and ONS diameter ( p < 0.005, r = 0.661). In the papilledema group, the presence of proposed subtle markers as increased tortuosity in the optic nerve was found in six patients. Five of seven patients had a target sign, intraocular protrusion of the optic nerve, and posterior globe flattening. Discussion: Ophthalmological review is important to avoid unnecessary procedures for detection of true papilledema. ONS diameter is a reliable neuroimaging marker as other subtle markers. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the accuracy of high resolution transbulbar sonography for the estimation of intracranial pressure (ICP) in children. Methods: In children and adolescents with acute neurologic symptoms of various origin, transbulbar sonography was performed. Besides measurement of the optic nerve sheath diameter (ONSD), the ultrastructure of the subarachnoid space of the optic nerve sheath was evaluated. The results of transbulbar sonography were correlated with clinical data based on cross-sectional imaging, ICP measurement, and ophthalmologic examination. Results: Eighty-one patients (age 3-17.8 years, mean 11.7 years) were included. In 25 children, cross-sectional imaging and ICP measurement revealed increased intracranial pressure. The mean ONSD was 6.85 ± 0.81 mm. Twenty patients (20/25, 80 %) had a microcystic appearance of the subarachnoid space of the optic nerve. In 56 children without evidence of increased intracranial pressure, the mean ONSD was 5.77 ± 0.48 mm. Forty-nine patients (49/56, 87.5 %) had a normal homogenous appearance of the subarachnoid space. The ONSD in children with increased intracranial pressure was significantly higher than in patients without ( p < 0.001). Conclusion: High resolution transbulbar sonography of the optic nerve is a useful technique for the rapid and non-invasive estimation of intracranial pressure in children. Besides measurement of the optic nerve sheath diameter, evaluation of the ultrastructure of the subarachnoid space of the optic nerve is a helpful parameter. [ABSTRACT FROM AUTHOR]

Purpose: Unlike pilocytic astrocytomas in other parts of the brain, optic pathway gliomas (OPG) are usually diffuse lesions involving the anterior optic pathways and hypothalamus. Their infiltrative nature often precludes complete surgical resection. We sought to determine whether careful magnetic resonance (MR) analysis, correlated with visual deficits, could be sufficient to identify those focal lesions that may be amenable to more aggressive surgical resection at presentation. Methods: We retrospectively reviewed the medical records of patients from two sites: children under 20 years of age treated for OPG between 1985 and 2009 at St Jude's Children's Research Hospital and children under 16 years of age treated at Great Ormond Street Hospital, London, UK, between 1984 and 2011. Patients with isolated optic nerve tumors were excluded. Visual acuity and visual field data at presentation were reviewed and correlated with MR characteristics, including extent of optic pathway involvement, symmetry, and lateral extension. Results: Two hundred and one children were treated for OPG between 1984 and 2011 in the two institutions; 74 had neurofibromatosis 1 (NF1). At presentation, visual loss was symmetrical in 132 patients and asymmetrical in 69. Potential correlation between pattern of visual loss and tumor characteristics on routine MRI was found in only 13 patients with asymmetrical vision. There was no difference between patients with and without NF1. Conclusion: The decision for aggressive surgical resection for optic pathway gliomas should be based on clinical criteria, particularly in children with good vision in one eye and poor vision in the other, as current MRI results do not reliably predict visual field deficits. [ABSTRACT FROM AUTHOR]

Purpose: Hamartomas are well described but yet incompletely understood sporadic benign lesions that can arise in various locations. Hypothalamic hamartomas of infancy are often associated with severe developmental disturbances. We present a case of an infant boy with a hamartoma that arises from the optic nerve and lead to progressive unspecific seizure activity, behavioral problems and precautious puberty. Methods: A 1-year-old male patient was presented with horizontal nystagmus and developmental retardation. Magnetic resonance imaging (MRI) with contrast revealed an isointense mass ventral of the chiasm consistent with a hamartoma. Soon after the MRI, the mother of the patient reported gelastic-like seizures. The patient was evaluated by an interdisciplinary team, and surgery was recommended. Intraoperatively, a firm attachment to the optic nerve was recognized and a thin remnant layer of tissue was left behind. Results: After an uncomplicated near total resection, the patient improved significantly. After 6 months, the frequency of seizures reoccurred, which were again unresponsive to antiepileptic medication. In a second operation, a complete resection of the remnants was performed, and the patient showed lasting clinical improvement. Conclusion: We conclude that hamartomas mimicking hypothalamic symptoms can also arise from the optic pathway and that a reoperation, if feasible, of even small remnants is essential in order to achieve lasting symptom relieve. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this study was to investigate the value of the change in optic nerve sheath diameter (ONSD) as a radiological marker of endoscopic third ventriculostomy (ETV) outcome in children. Methods: Magnetic resonance imaging (MRI) scans of patients on whom ETVs were performed between the periods of January 2009 and June 2013 were reviewed. ONSD measurements on pre- and post-operative images were performed by two blinded observers, and the relationship between the change in these measurements and outcome from ETV were investigated. These findings were then also compared to conventional imaging features associated with ETV outcome. Results: MRI scans of 24 patients were adequate to measure the ONSD pre- and post-operatively. In patients with successful ETV ( n = 19), the mean change in ONSD was 0.73 mm and in patients with a failed ETV ( n = 5), the mean change in ONSD was 0.18 mm ( p = 0.0007). A change in ONSD of 7.5 % of the initial measurement demonstrated a sensitivity of 92.9 % and a sensitivity of 85.7 % for ETV outcome (area under the receiver operating characteristic curve (AUROC) = 0.96). Conclusion: Change in ONSD is a useful radiological marker of ETV outcome and may be used in combination with conventional radiological parameters to aid decision-making in this difficult group of patients. [ABSTRACT FROM AUTHOR]

Introduction: Serial change in ventricular size is recognized as an imperfect indicator of ongoing hydrocephalus in children. Potentially, other radiographic features may be useful in determining the success of hydrocephalus interventions. In this study, optic nerve sheath diameter (ONSD), optic nerve tortuosity, and optic disk bulging were assessed as indicators of hydrocephalus control in children who underwent endoscopic third ventriculostomy (ETV) or posterior fossa tumor resection. Methods: Sixteen children underwent ETV or tumor resection for treatment of hydrocephalus. T2-weighted axial magnetic resonance images of the orbit were obtained, and the ONSD was measured posterior to the optic globe, pre- and post-intervention. Evidence of optic disk bulging and optic nerve tortuosity was also assessed. Ventricular size was estimated using the frontal and occipital horn ratio (FOR). Results: There was a significant reduction in the ONSD post-ETV ( n = 9) and after tumor resection ( n = 7). Average preoperative ONSD was 6.21 versus 5.71 mm postoperatively ( p = 0.0017).There was also an 88 % ( p = 0.011) and 60 % ( p = 0.23) reduction in optic disk bulging and tortuosity, respectively. The FOR normalized in the tumor resection group but not the ETV group. After intervention, all patients showed improvement in signs and symptoms of hydrocephalus. Conclusion: In our study population, ONSD decreased in response to measures to reduce hydrocephalus. Optic disk bulging also appears to resolve. Serial reduction in ONSD, and optic disk bulging may be indicators of improved hydrocephalus following pediatric neurosurgical interventions. [ABSTRACT FROM AUTHOR]

Introduction: Neuroendoscopic approaches to Sylvian arachnoid cysts (SACs) constitute an alternative treatment option to craniotomy for fenestration and shunting procedures. In this study, the authors discuss their experience on pure neuroendoscopic technique in the treatment of SACs in children. Results: The results of treatment of 20 children (range of age, between 7 months and 17 years) with Galassi type II ( n, 5) or III ( n, 15) SACs who were subjected to pure neuroendoscopic fenestration procedure were presented. It was possible to perform the cystocisternostomy endoscopically in all children with several stomies. The site of the opening was between the optic nerve and the carotid artery in 19, between the carotid artery and the oculomotor nerve in 17, and below the oculomotor nerve in 7. The stomies were enlarged in all cases using the double balloon. Three of the cases required repetition of the operation and two cases required 'cystoperitoneal shunt' implantation. There was one minor complication in a patient who had an asymptomatic postoperative subdural effusion, which resolved spontaneously. Of the 18 cases, in which the neuroendoscopic procedures succeeded, 10 showed a reduction in cyst size. The mean follow-up period was 53 months. Discussion: Our results suggest that 'pure neuroendoscopic' approach can be used safely in the management of SACs in children. We recommend at least two fenestration sites for an effective marsupialization of the cyst within the basal cisterns. In pediatric cases, the use of a small diameter rigid endoscope allows to reach safely the planned target areas. [ABSTRACT FROM AUTHOR]

Meningocele of the optic nerve is a rare condition defined as dilatation of the optic nerve by spinal fluid not due to other pathology, classically presenting with headaches or progressive visual decline and usually follows a rapidly progressive course. Surgical decompression is the standard treatment with improvement or arrest of progression in most cases. We describe a case of a child with multiple congenital anomalies including a left optic nerve meningocele that progressively expanded and caused displacement of the orbit laterally resulting in severe cosmetic deformity and complete blindness in the left eye. We describe our surgical decompression as well as review the literature on optic nerve meningocele. [ABSTRACT FROM AUTHOR]

AbstractIntroduction??Orbital emphysema can be associated with optic nerve and vascular compromise, with subsequent potentially irreversible visual loss.Materials and methods??We report a 4-year-old girl who presented right orbital emphysema after posterior fossa surgery for pilocytic astrocytoma resection in the sitting position. Endoscopic third ventriculostomy (ETV) had been previously carried out for the treatment of symptomatic obstructive hydrocephalus. Our hypothesis is that periorbital emphysema developed due to air passing from the intracranial to the extracranial compartment through the ETV burr hole.Result and discussion??The emphysema resolved spontaneously without any sequelae. To our knowledge, this is the first description of orbital emphysema after posterior fossa surgery in the sitting position. [ABSTRACT FROM AUTHOR]