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Neurofibromatosis type 1 (NF1), the most frequent phakomatosis and one of the most common inherited tumor predisposition syndromes, is characterized by several manifestations that pervasively involve central and peripheral nervous system structures. The disorder is due to mutations in the NF1 gene, which encodes for the ubiquitous tumor suppressor protein neurofibromin; neurofibromin is highly expressed in neural crest derived tissues, where it plays a crucial role in regulating cell proliferation, differentiation, and structural organization. This review article aims to provide an overview on NF1 non-neoplastic manifestations of neuroradiological interest, involving both the central nervous system and spine. We also briefly review the most recent MRI functional findings in NF1.

MNTI is a rare tumor of indeterminate histogenesis and molecular signature. We performed methylation and copy number variation (CNV) profiles in patients with MNTI ( n = 7) and PAT ( n = 1) compared to the methylation brain tumor classifier v11b4 (BT-C) and the medulloblastoma (MB) classifier group 3/4 v1.0 (MB3/4-C). The patients' mean age was 8 months (range: 4-48). The BT-C classified five MNTIs and one PAT (relapse) as class family MB-G3/G4, subclass group 3 (score: >0.9). The remaining two MNTIs and PAT (primary) were classified as class family plexus tumor, subclass pediatric (scores: >0.45). The MB3/4-C classified all MNTIs as high-risk MB-G3, Subtype II (score: >0.45). The primary PAT was classified as subtype III (score: 0.99) and its relapse as subtype II/III. MNTI and PAT clustered close to MB-G3. CNV analysis showed multiple rearrangements in one PAT and two MNTIs. The median follow-up was 54 months (four MNTIs in remission, one PAT died). In conclusion, we demonstrated that MNTI shares a homogenous methylation profile with MB-G3, and possibly with PAT. The role of a multipotent progenitor cell (i.e., early cranial neural crest cell) in their histogenesis and the influence of the anatomical site, tumor microenvironment, and other cytogenetic events in their divergent biologic behavior deserve further investigation.

Purpose: Moyamoya syndrome (MMS) is a rare intracranial arterial vasculopathy which can occur in neurofibromatosis type 1 (NF1) disease, representing a cause of cerebrovascular reserve (CVR) impairment, possibly leading to ischemic stroke. Here, we evaluated noninvasive imaging techniques used to assess CVR in MMS patients, describing clinical and imaging findings in patients affected by MMS-NF1., Methods: Following strict inclusion and exclusion criteria, in this retrospective observational study, we evaluated imaging data of nine consecutive MMS-NF1 patients (M/F = 5/4, mean age: 12.6 ± 4.0). Subjects underwent a multimodal evaluation of cerebral vascular status, including intracranial arterial MR Angiography (MRA), MRI perfusion with dynamic susceptibility contrast (DSC) technique, and 99mTc-hexamethylpropyleneamine oxime (HMPAO) SPECT., Results: In 8 out 9 patients (88.8%, 6/8 symptomatic), time-to-peak maps were correlated with the involved cerebral hemisphere, while in 6 out 9 patients (66.6%, 5/6 symptomatic), mean transit time (MTT) maps showed correspondence with the affected cerebrovascular territories. Cerebral blood flow (CBF) calculated using DSC perfusion failed to detect the hypoperfused regions instead identified by SPECT-CBF in all patients, while MTT maps overlapped with SPECT-CBF data in all cases and time-to-peak maps in 60.0%., Conclusions: Although SPECT imaging still represents the gold standard for CBF assessment, our results suggest that data obtained using DSC perfusion technique, and in particular MTT maps, might be a very useful and noninvasive tool for evaluating hemodynamic status in MMS-NF1 patients.

Introduction: Neuroendoscopic biopsy represents the procedure of choice for pure intraventricular lesions. Instead, in case of deep-seated paraventricular tumors, with intact ependyma, the advantage of neuroendoscopy over stereotactic biopsy is not so evident, because the lesion is not under direct vision; the tissue sample may be limited to more superficial ependymal layer, and bleeding may obscurate vision. Also, stereotactic biopsy may reserve additional problems for these lesions: inaccuracy caused by leak of cerebrospinal fluid and increased risk of severe hemorrhage due to damage of the ependymal vessels., Case Reports: We report two cases of young children affected by thalamic tumors that were biopsied using a modification of a recently proposed technique: endoscopic visual control, neuronavigated needle biopsy., Conclusion: This technique may combine the accuracy of a stereotactic needle biopsy with the advantage of visual control on site of ependymal puncture and possibility of immediate bleeding control.

Introduction: Tuberous sclerosis complex (TSC) is a rare autosomal dominant disorder affecting multiple systems, due to inactivating mutations of TSC1 or TSC2 mTOR pathway genes. Neurological manifestations are observed in about 95% cases, representing the most frequent cause of morbidity and one of the most common causes of mortality., Background: Neuroimaging is crucial for early diagnosis, monitoring, and management of these patients. While computed tomography is generally used as first-line investigation at emergency department, magnetic resonance imaging is the reference method to define central nervous system involvement and investigate subtle pathophysiological alterations in TSC patients., Purpose: Here, we review the state-of-the-art knowledge in TSC brain imaging, describing conventional findings and depicting the role of advanced techniques in providing new insights on the disease, also offering an overview on future perspectives of neuroimaging applications for a better understanding of disease pathophysiology.

Epilepsy is one of the possible neurological manifestations of the neurofibromatosis type 1 (NF1) that represents the most common neurocutaneous disorder. We performed a systematic review of the literature on epilepsy associated with NF1 since 1995 in order to better define prevalence and describe type and causes of seizures. Data on type, nature of studies, number of patients, gender, and inheritance of NF1 were recorded as well as data on causes, type, EEGs, brain imaging, intellectual disability (ID), surgical treatment, and outcome of epilepsy. We identified a total of 141 references through the literature search of Pubmed and Embase. After screening, 42 records were identified, including 11617 individuals with NF1 (53% of males). Overall prevalence was estimated at 5.4% lifelong with values that seemed to be slightly lower in children, 3.7% (p 0.0016). Neither gender differences nor correlation with NF1 inheritance was found. Focal with or without bilateral tonic-clonic seizures were the most common seizure type encountered (60.9%). Structural causes were identified in half of cases (114/226). Low-grade gliomas were the most frequent associated lesions followed by mesial temporal sclerosis, malformation of cortical development, dysembryoplastic neuroepithelial tumor, and cerebrovascular lesions. In these cases, the surgical approach improved the epileptic outcome. Prevalence of epilepsy is higher in subjects with NF1 respect of the general population, with values apparently significantly lower in pediatric age. Brain tumors and cytoarchitectural abnormalities are the most frequent causes of epilepsy in this population, although many other brain complications should be taken in account.

The prevalence of hydrocephalus among patients with neurofibromatosis type I (NF1) is estimated to be between 1 and 13%. Most hydrocephalic causes are obstructive-aqueductal webs, chiasmatic-hypothalamic tumors, and thalamic mass effect related to NF changes. Other NF1-related conditions may mimic the clinical presentation of hydrocephalus and should be ruled out while evaluating children with headaches. These include brain tumors and moyamoya syndrome. Treatment of NF1-related hydrocephalus should be personally tailored, including tumor resection or debulking, shunts, and endoscopic procedures such as septostomy and third ventriculostomy. Despite these personalized treatments, many of the primary treatments (including shunts and endoscopic procedures) fail, and patients should be screened and followed accordingly. In the current manuscript, we review the causes of NF1-related hydrocephalus, as well as treatment options.

Lumbar pseudomeningocele is a common complication of spinal surgery. It is caused by dural defect, and it consists of extradural collection of cerebrospinal fluid communicating with subarachnoid space. Usually it is small, asymptomatic, and self-limiting. In case of persistence, common treatments include bedrest, puncture and compressive dressing, subcutaneous or subarachnoid lumbar drainage, surgical re-exploration for durotomy closure, and blood patch. Fast-resorbing polymer mesh has been recently used for closure osseous gap in adult patients who developed giant pseudomeningocele following laminectomy. We report the case of a 16-year-old girl, affected by spinal dysraphism, who developed a persistent giant lumbar pseudomeningocele after repeated surgery for detethering of the spinal cord and removing of spinal lipomyelomeningocele. She was finally treated with fast-resorbing polymer mesh, to fill the gap of the posterior osseous defect and to provide support for the thecal sac. For our best knowledge, this is the first pediatric case reported in literature on this kind of reconstruction. In complex cases of pseudomeningocele, implant of fast-resorbing polymer mesh may represent an option also in pediatric patients.

Background: Pediatric spinal epidural abscess is a major suppurative infection of the central nervous system. It is an extremely rare pathology carrying serious risk of permanent neurological sequelae if is not properly treated., Methods and Results: All the pertinent literature was analyzed, focused on pediatric cases of spinal epidural abscess and its peculiar features. Two illustrative cases are also presented. The first case is that of a 9-year old girl who took medical attention, when she was already paraplegic. Despite prompt surgical evacuation and good neuroradiological outcome and intensive rehabilitation, motor deficits did not recover after surgery. The second case was that of a 14-year old girl who presented with fever, neck pain, and torticollis. Prompt diagnosis, decompressive surgery, and 6 weeks of antibiotics allowed good neurological outcome., Conclusions: The management of spinal epidural abscess includes evacuation of the abscess with decompression of the spinal cord and prolonged antibiotic treatment. The presence of neurological deficit and the delay in the initiation of proper treatment are the two factors that more worsen prognosis.

Context: Up to 20% of children with neurofibromatosis type 1 (NF1) develop low-grade optic pathway gliomas (OPGs) that can result in endocrine dysfunction. Data on prevalence and type of endocrine disorders in NF1-related OPGs are scarce., Objectives: The aim of the study was to determine the prevalence of endocrine dysfunctions in patients with NF1 and OPGs and to investigate predictive factors before oncological treatment., Design: Multicenter retrospective study., Settings and Patients: Records were reviewed for 116 children (64 females, 52 males) with NF1 and OPGs followed at 4 Italian centers., Main Outcome Measures: We evaluated endocrine function and reviewed brain imaging at the time of OPG diagnosis before radio- and chemotherapy and/or surgery. OPGs were classified according to the modified Dodge classification., Results: Thirty-two children (27.6%) with a median age of 7.8 years had endocrine dysfunctions including central precocious puberty in 23 (71.9%), growth hormone deficiency in 3 (9.4%), diencephalic syndrome in 4 (12.5%), and growth hormone hypersecretion in 2 (6.2%). In a multivariate cox regression analysis, hypothalamic involvement was the only independent predictor of endocrine dysfunctions (hazard ratio 5.02 [1.802-13.983]; P = .002)., Conclusions: Endocrine disorders were found in approximately one-third of patients with Neurofibromatosis type 1 and OPGs before any oncological treatment, central precocious puberty being the most prevalent. Sign of diencephalic syndrome and growth hormone hypersecretion, although rare, could be predictive of optic pathway gliomas in NF1. Tumor location was the most important predictor of endocrine disorders, particularly hypothalamic involvement., (© Endocrine Society 2020. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

s: The natural history of non-optic central nervous system (CNS) tumors in neurofibromatosis type 1 (NF1) is largely unknown. Here, we describe prevalence, clinical presentation, treatment, and outcome of 49 non-optic CNS tumors observed in 35 pediatric patients (0-18 years). Patient- and tumor-related data were recorded. Overall survival (OS) and progression-free survival (PFS) were evaluated. Eighteen patients (51%) harbored an optic pathway glioma (OPG) and eight (23%) had multiple non-optic CNS lesions. The majority of lesions (37/49) were managed with a wait-and-see strategy, with one regression and five reductions observed. Twenty-one lesions (42.9%) required surgical treatment. Five-year OS was 85.3%. Twenty-four patients progressed with a 5-year PFS of 41.4%. Patients with multiple low-grade gliomas progressed earlier and had a lower 5-year PFS than those with one lesion only (14.3% vs. 57.9%), irrespective of OPG co-presence. Non-optic CNS tumors are common in young patients with NF1. Neither age and symptoms at diagnosis nor tumor location influenced time to progression in our series. Patients with multiple lesions tended to have a lower age at onset and to progress earlier, but with a good OS., Competing Interests: The authors declare no conflict of interest.

Background: Midbrain and surrounding areas are among the most difficult regions to surgically approach in neurosurgery. The subtemporal approach is a well-known neurosurgical approach that is rarely described in children., Objective: To assess the feasibility, advantages, and disadvantages of subtemporal approach in children., Methods: A total of 20 pediatric patients with intra-axial tumors involving the lateral midbrain or superior cerebellar peduncle or with extra-axial tumors in the middle incisural space/Meckel cave underwent subtemporal transtentorial approaches with the aim to remove (16 cases) or biopsy (4 cases) their lesions. These cases were retrospectively reviewed and the surgical approach described., Results: Total resection was achieved in 11 patients. In the 4 patients who underwent biopsies, a diagnosis was obtained. A total of 5 patients presented non-neoplastic lesions (dermoid tumors or cavernomas). A total of 9 patients harbored low-grade tumors and 6 high-grade tumors. Patients with low-grade tumors and non-neoplastic lesions are all alive with no evidence of disease or stable residue. Four patients affected by high grade tumors died. No patient suffered permanent neurological deficits related to the surgical approach. Three patients presented temporary neurological deficits following the procedure: one case of strabismus, one case of aphasia and hemiparesis, secondary to delayed, transient thrombosis of the Labbé vein, and 1 case of trigeminal neuralgia., Conclusion: The subtemporal approach represents a feasible approach for mesencephalic and perimesencephalic lesions in children. It provides an ample and direct access, with excellent outcomes and acceptable postoperative morbidity., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Introduction: Cranioplasty in children is a controversial and challenging issue, since there is still no consensus on the ideal material. Main problems in paediatric age are represented by the child's growing skull, the lower bone thickness and the high incidence of cerebrospinal fluid (CSF) disorders or brain swelling. Autologous bone is still considered the "gold standard". When it is not available, a wide range of alloplastic materials have been proposed. Hydroxyapatite, a ceramic-based derivative, bears a chemical composition very similar to the human natural bone, making this material a valuable alternative to other cranioplasty solutions., Methods: All patients implanted with a custom-made porous hydroxyapatite device at Santobono-Pausilipon Hospital in Naples were retrospectively reviewed. A follow-up CT scan of the skull was performed from 1 up to 48 months postoperatively to document the bone ingrowth as well as the osteointegration process. The bone density was measured as according to the Hounsfield scale at the bone-implant interface., Results: Between 2014 and 2018, 11 patients (7 males, 4 females) underwent cranioplasty with hydroxyapatite ceramic implants (HAP). Patients' age ranged between 3 and 16 years old. Initial aetiology was trauma in most cases. Two subjects were implanted with HAP as primary cranioplasty, 9 as revision surgery following previous cranioplasty failure. Sites of the cranial defect were unilateral fronto-temporo-parietal (N = 8), unilateral frontal (N = 1) and bifrontal (N = 2). Two patients with large bilateral defects received two prostheses. In one of these, the two prostheses were explanted and replaced with two back-up implants (accounting for a total of 15 implants in 11 patients). Osteointegration was measurable for 12 out of 15 implanted devices. The mean percentage was about 51%. There were six asymptomatic prosthesis fractures (40%), all occurring within 6 months from implant. In one case, the bifrontal prostheses were explanted and replaced. This was the only patient who underwent revision surgery., Conclusion: Hydroxyapatite ceramic implants represent a valid alternative to other cranioplasty solutions. Where coaptation occurs correctly, with good osteointegration, implant mechanical resistance increases over time.

Aim: To evaluate the efficacy of using a neuronavigation system for demonstrating the relationship between the basilar artery (BA) and ventricular floor during endoscopic third ventriculostomy (ETV)., Material and Methods: Records of 28 patients (16 females and 12 males) diagnosed with obstructive hydrocephalus who had undergone a neuroendoscopic procedure were retrospectively examined. Patient age ranged from 1 to 76 years (median 24.46 years). The BA was marked with using the neuronavigation system in all cases to visualise its relationship to the floor of the third ventricle in real time., Results: ETV was successfully performed in 28 patients with obstructive hydrocephalus. Of these, 13 (46.4%) patients had a thickened tuber cinereum (TC) membrane and 3 (10.7%) showed lateralization of the BA under the ventricular floor. No contact with the BA or related complications (e.g., major bleeding) was encountered with BA marking by using neuronavigation., Conclusion: Even though thickening of the TC membrane and/or displacement of the BA might be seen otherwise, we describe a new method that combines marking the BA and using neuronavigation to provide greater safety in the area where the ventriculostomy will be performed. This permits clearer orientation for the surgeon which significantly contributes to minimizing surgical morbidity.

Introduction: Post-traumatic hydrocephalus following head injury is a well-known entity. Most cases occur in patients with severe head injuries, often following decompressive craniectomy. On the contrary, acute post-traumatic hydrocephalus, caused by aqueductal obstruction by a blood clot, following mild head injury is uncommon., Clinical Material: Six patients aged between 6 and 15 months presented hydrocephalus secondary to a blood clot in the aqueduct. Because of intracranial hypertension at presentation, 4 patients were urgently treated with external ventricular drains (EVDs). Post-operative course was uneventful. In 2 cases, EVDs were removed without further treatments. In 2 cases, hydrocephalus recurred. These patients were successfully treated with endoscopic third ventriculostomy. The remaining two patients developed symptoms a few days after the trauma. One, that presented hydrocephalus at imaging, was managed with a ventriculo-peritoneal shunt; the other, that presented subdural hygroma, was managed with subduro-peritoneal shunt that was removed later. All patients had complete recovery., Discussion and Conclusion: Hydrocephalus secondary to clot in the aqueduct may rarely be the result of mild head injury in young children. Usually, prompt surgical management warrants a very good outcome. Most children may be treated without a permanent shunt, by using external drains and endoscopic third ventriculostomy.

Background: Malignant spinal cord compression (MSCC) is associated withpoor prognosis and may lead to permanent paralysis, sensory loss, and sphincter dysfunction. Very limited data are available on incidence and etiology of MSCC in pediatric population. We aimed to examine etiology, clinical presentation and treatment of pediatric patient with MSCC admitted to the Santobono-Pausilipon Children's Hospital, Naples, Italy., Methods: Forty-four children under 18 yearsadmitedsince 2007 and assessed for MSCC clinical presentations, evaluation, and treatment.were retrospectively collected from our institutional pediatric oncology and neurosurgery database., Results: The median age at time of MSCC diagnosis was 52 months, with a peak in young (≤3 years) patients. The leading cause of MSCC was extramedullary tumors (63.6%), in particular neuroblastoma (27.2%) followed by Ewing sarcomas (15.9%). Cord compression was the presenting feature of a new malignancy in 33 (75%) patients, and a consequence of metastatic disease progression or relapse in the remaining 11 (25%) patients. Motor deficit was the initial symptoms of spinal compression in all patients, while pain was present in about 60% of patients, followed by sphincteric deficit (43.2%). The primary tumor site was located in the neck in 3 (6.8%) patients, thorax in 16 (36.4%), cervico-thoracic region in 3 (6.8%), thoraco-lumbar region in 8 (18.2%), abdomen in 5 (11.4%), lumbar-sacral region in 7 (15.9%) and thoracic-lumbar-sacral region in 1 (2.3%). The median length of the interval between symptom onset and tumor diagnosis varied widely from 0 to 360 days in the entire population, however this interval was significantly shorter in patients with known neoplasia in comparisonto patients with new diagnosis (at relapse 7 days [interquartile range 3-10] vs at diagnosis 23 days [7-60]). Pre and post-operative spine magnetic resonance imagingwas performed in all cases, and most(95%) patients underwent neurosurgical treatment as first treatment. Severe motor deficit was associated with younger age and severe motor deficit at diagnosis was associated withworst motor outcomes at discharge from neurosurgery. Patients with progression or relapsed disease showed a worst prognosis, while the majority of patients (70.5%) were alive at 5 years after diagnosis., Conclusions: The natural history of MSCC in children is associated to permanent paralysis, sensory loss, and sphincter dysfunction, thus prompt diagnosis and correct management are needed to minimize morbidity. Treatment strategies differed widely among cancer types and study groups in the absence of optimal evidence-based treatment guidelines. When the diagnosis is uncertain, surgery provides an opportunity to biopsy the lesion in addition to treating the mass.

Objective: Moyamoya disease (MMD) is characterized by a progressive spontaneous occlusion of distal internal carotid arteries and its main branches. It is necessary to promptly diagnose and treat this condition, especially in children, because of the high risk of stroke and consequent severe disability. In this study, we examine the role of multiple burr-hole surgery (MBHS) in the treatment of pediatric patients with MMD and quasi-MMD and the role of perfusion magnetic resonance imaging (MRI) in the surgical indication and during follow-up., Methods: We reviewed preoperative, early postoperative, and late postoperative MRI perfusion and digital subtraction angiography images together with clinical and surgical outcomes in 10 children with MMD treated by MBHS., Results: Fourteen MBHS procedures (6 unilateral, 2 bilateral, and 2 single-setting bilateral) were performed in 10 children. Clinical and radiologic follow-up for all patients ranges from 16 months to 7 years. No ischemic events (transient ischemic attacks or stroke) occurred during the follow-up period. Postoperative digital subtraction angiography showed good revascularization around the burr-hole sites in all patients. The functional efficacy of the surgery was confirmed by dynamic susceptibility contrast perfusion MRI studies in 8 patients., Conclusions: Our data underline the good rate of success of MBHS in pediatric MMD and quasi-MMD. This technique results in good collateral revascularization, improved cerebral perfusion and excellent short and long-term symptoms control, with low perioperative risk. Postoperative changes of perfusion parameters and ivy sign MRI finding seem to show a positive correlation and mainly occur in posterior middle cerebral artery territory., (Copyright © 2019. Published by Elsevier Inc.)

We present an unusual association between Noonan syndrome and tetraventricular hydrocephalus, caused by fourth ventricle outlet obstruction, in a 5-year-old boy. Magnetic resonance imaging showed a diverticular enlargement of the left foramen of Luschka, with compression of the facial nerve that resolved following treatment of hydrocephalus by endoscopic third ventriculostomy., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Introduction: Among children with hydrocephalus, neonates with intraventricular hemorrhage (IVH) and posthemorrhagic hydrocephalus (PH) are considered a group with one of the highest complication rates of treatment. Despite continued progress in neonatal care, a standardized and reliable guideline for surgical management is missing for this challenging condition. Thus, further research is warranted to compare common methods of surgical treatment. The introduction of neuroendoscopic lavage has precipitated the establishment of an international registry aimed at elaborating key elements of a standardized surgical treatment., Methods: The registry is designed as a multicenter, international, prospective data collection for neonates aged 41 weeks gestation, with an indication for surgical treatment for IVH with ventricular dilatation and progressive hydrocephalus. The following initial temporizing surgical interventions, each used as standard treatment at participating centers, will be compared: external ventricular drainage (EVD), ventricular access device (VAD), ventricular subgaleal shunt (VSGS), and neuroendoscopic lavage (NEL). Type of surgery, perioperative data including complications and mortality, subsequent shunt surgeries, ventricular size, and neurological outcome will be recorded at 6, 12, 36, and 60 months., Results: An online, password-protected website will be used to collect the prospective data in a synchronized manner. As a prospective registry, data collection will be ongoing, with no prespecified endpoint. A prespecified analysis will take place after a total of 100 patients in the NEL group have been entered. Analyses will be performed for safety (6 months), shunt dependency (12, 24 months), and neurological outcome (60 months)., Conclusion: The design and online platform of the TROPHY registry will enable the collection of prospective data on different surgical procedures for investigation of safety, efficacy, and neurodevelopmental outcome of neonates with IVH and hydrocephalus. The long-term goal is to provide valid data on NEL that is prospective, international, and multicenter. With the comparison of different surgical treatment modalities, we hope to develop better therapy guidelines for this complex neurosurgical condition.

Background: Interhypothalamic adhesions (IHAs) are horizontally oriented parenchymal bands of tissue connecting the medial hypothalamic regions across the third ventricle. They can be assessed with high-resolution magnetic resonance (MR) techniques., Case Description: We report MR and neuroendoscopic features of IHA in a 3-year-old boy without symptoms referable to the hypothalamus. He presented with obstructive hydrocephalus secondary to posterior fossa tumor. An endoscopic third ventriculostomy (ETV) was attempted but not performed because of the presence of a thick IHA, which prevented an approach to the floor of the third ventricle. During the procedure the patient also experienced supraventricular tachycardia. The procedure was aborted, and an external ventricular drainage was left in the ventricles until resolution of hydrocephalus after posterior fossa surgery. To the best of our knowledge, no study has previously described in detail endoscopic images of IHA., Conclusions: MR imaging allows one to preoperatively identify most anatomic anomalies of the ventricular system and the floor of the third ventricle. However, an IHA that may prevent an approach to the floor of the third ventricle due to his thickness may be missed. This can be recognized only on direct vision, underlining the importance of endoscopy in neurosurgery., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Arachnoid cysts are fluid-filled sacs, located between the brain or spinal cord and the arachnoid membrane. Their prevalence in children is between 1% and 3%. Quadrigeminal arachnoid cysts represent 1% to 10% of them and are often associated with hydrocephalus, mostly by an obstructive mechanism, explained by compression of the tectum of the midbrain. When an indication for treatment is retained, 3 surgical options are available: microsurgical excision/fenestration, endoscopic fenestration, and shunt placement. Endoscopic treatment is considered the best compromise of definitive treatment with least surgical morbidity, especially because quadrigeminal cysts are located close to the midline, in intimate relationships with basal cisterns and ventricles. We here present the endoscopic treatment of a prenatally diagnosed quadrigeminal arachnoid cyst type III1 with right lateral extension into the middle cerebral fossa, and associated hydrocephalus, treated at the age of 18 mo.Step-by-step detail of surgical technique is presented in original anatomic conditions. Restoration of better cerebrospinal fluid pathways being the objective of this surgery, ventriculocystic, and cyst-cisternal fenestrations were made. Secondary obstruction of the cyst occurred a few months later, requiring further endoscopic treatment to obtain a larger fenestration that allowed good long-term clinical and radiological outcome. The key point of this video is to compare the 2 procedures, stressing the importance of the dimension of fenestrations, to ensure a long-term patency of both stomas.The patient being a child, both parents gave their consent for publication and signed a form.

Introduction: Intrathecal baclofen (ITB) is an effective treatment for managing primary and secondary dystonia. Intraventricular baclofen (IVB) was first developed to allow treating patients in which the use of ITB was difficult due to anatomic anomalies. After that, several studies indicate that intraventricular administration of baclofen, is more effective than ITB in refractory dystonia., Clinical Material: We report three cases of children with acute dystonic and dysautonomic storm, treated with IVB. The clinical outcome was satisfactory. The response to the treatment continued after the pump disconnection, suggesting that in this kind of cerebral dysregulations, short-term IVB is an effective treatment., Conclusion: Early treatment with IVB may be an effective option in patients with post-anoxic dysautonomic and dystonic storm.

OBJECTIVEHemorrhage (also known as apoplexy) in optic pathway gliomas (OPGs) is rare. Because of the variable presentations and low incidence of OPG hemorrhages, little is known about their clinical course and the best treatment options. The aim of this work was to review risk factors, clinical course, and treatment strategies of optic glioma hemorrhages in the largest possible number of cases.METHODSA total of 34 patients were analyzed. Nine new cases were collected, and 25 were identified in the literature. Data regarding demographics, radiological and histological features, treatment, and outcome were retrospectively reviewed.RESULTSThe majority of patients were younger than 20 years. Only 3 patients were known to have neurofibromatosis. The histopathological diagnosis was pilocytic astrocytoma in the majority of cases. Five patients had intraorbital hemorrhages, whereas 29 patients had intracranial hemorrhage; the majority of intracranial bleeds were treated surgically. Six patients, all with intracranial hemorrhage, died due to recurrent bleeding, hydrocephalus, or surgical complications. No clear risk factors could be identified.CONCLUSIONSIntracerebral OPG hemorrhages have a fatal outcome in 20% of cases. Age, hormonal status, neurofibromatosis involvement, and histopathological diagnosis have been suggested as risk factors for hemorrhage, but this cannot be reliably established from the present series. The goals of surgery should be patient survival and prevention of further neurological and ophthalmological deterioration.

Purpose: The aims of patients' radiological surveillance are to: ascertain relapse; apply second-line therapy; accrue patients in phase 1/2 protocols if second-line therapy is not standardized/curative; and assess/treat iatrogenic effects. To lessen the emotional and socioeconomic burdens for patients and families, we ideally need to establish whether scheduled radiological surveillance gives patients a better outcome than waiting for symptoms and signs to appear., Methods: We analyzed a prospective series of 160 newly-diagnosed and treated pediatric/adolescent patients with intracranial ependymoma, comparing patients with recurrent disease identified on scheduled MRI (the RECPT group; 34 cases) with those showing signs/symptoms of recurrent disease (the SYMPPT group; 16 cases). The median follow-up was 67 months., Results: No significant differences emerged between the two groups in terms of gender, age, tumor grade/site, shunting, residual disease, or type of relapse (local, distant, or concomitant). The time to relapse (median 19 months; range 5-104) and the MRI follow-up intervals did not differ between the SYMPPT and RECPT groups. The presence of signs/symptoms was an unfavorable factor for overall survival (OS) after recurrence (5-year OS: 8% vs. 37%, p = 0.001). On multivariable analysis, an adjusted model confirmed a significantly worse OS in the SYMPPT than in the RECPT patients., Conclusions: Symptomatic relapses carried a significantly worse survival for ependymoma patients than recurrences detected by MRI alone. It would therefore be desirable to identify recurrences before symptoms develop. Radiological follow-up should be retained in ependymoma patient surveillance because there is a chance of salvage treatment for relapses found on MRI.

Background: Diagnosis and treatment of primary lung adenocarcinoma in children remains challenging given its rarity. Here we highlight the clinical history, pathological evaluation, genomic findings, and management of a very young patient with metastatic lung adenocarcinoma., Case Presentation: A 10-year-old white girl presented with brain metastases due to primary pulmonary adenocarcinoma. Next generation sequencing analysis with "Comprehensive Cancer Panel" highlighted the presence of multiple non-targetable mutations in the FLT4, UBR5, ATM, TAF1, and GUCY1A2 genes. She was treated aggressively with chemotherapy, surgery, and radiation therapy for local and distant recurrence. Eventually, therapy with nivolumab was started compassionately, and she died 23 months after diagnosis., Conclusions: Extremely rare cancers in children such as lung adenocarcinoma need accurate and specific diagnosis in order to develop an optimal plan of treatment. It is also necessary to underline that "children are not little adults," thus implying that an adult-type cancer in the pediatric population might have a different etiopathogenesis. Diagnostic confirmation and primary treatment of such rare conditions should be centralized in reference centers, collaborative networks, or both, with multidisciplinary approaches and very specific expertise.

Background: Intracerebral abscess is a rare, but very serious complication of neonatal sepsis. Aggressive medical and surgical strategy is warranted in patients with large abscesses., Case Report: A neonate with bilateral large frontal abscesses, caused by Serratia marcescens, was operated using a neuroendoscope coupled with an ultrasonic aspiration device. To our knowledge, this is the first report of such utilization of this new tool. The left abscess was surgically drained through endoscopic aspiration using ultrasonic aspirator. Clinical condition rapidly improved, and postoperative MRI of the brain revealed an almost complete resolution of the left abscess. A second endoscopic procedure was performed 1 week later, and the right abscess was endoscopically drained with the same technique. The remaining clinical course was uneventful., Discussion and Conclusion: Ultrasonic aspiration is effective in obtaining complete removal of the purulent material and of the dense fibrin layer adherent to the capsule. However, further studies are warranted to determine its real effectiveness, compared with the classic technique. In our opinion, it should be considered an option in more difficult cases, such as abscesses caused by aggressive germs in young or immunocompromised patients, or in case of recurrent lesions, in order to avoid craniotomy and open surgery.

Genetic modifications during development of paediatric groups 3 and 4 medulloblastoma are responsible for their highly metastatic properties and poor patient survival rates. PRUNE1 is highly expressed in metastatic medulloblastoma group 3, which is characterized by TGF-β signalling activation, c-MYC amplification, and OTX2 expression. We describe the process of activation of the PRUNE1 signalling pathway that includes its binding to NME1, TGF-β activation, OTX2 upregulation, SNAIL (SNAI1) upregulation, and PTEN inhibition. The newly identified small molecule pyrimido-pyrimidine derivative AA7.1 enhances PRUNE1 degradation, inhibits this activation network, and augments PTEN expression. Both AA7.1 and a competitive permeable peptide that impairs PRUNE1/NME1 complex formation, impair tumour growth and metastatic dissemination in orthotopic xenograft models with a metastatic medulloblastoma group 3 cell line (D425-Med cells). Using whole exome sequencing technology in metastatic medulloblastoma primary tumour cells, we also define 23 common 'non-synonymous homozygous' deleterious gene variants as part of the protein molecular network of relevance for metastatic processes. This PRUNE1/TGF-β/OTX2/PTEN axis, together with the medulloblastoma-driver mutations, is of relevance for future rational and targeted therapies for metastatic medulloblastoma group 3.10.1093/brain/awy039_video1awy039media15742053534001.

Background: Neurofibromatosis type 1 (NF1) is related to a generally increased prevalence of seizures. The mechanism underlying the increased predisposition to seizures has not been fully elucidated. The aim of the study was to evaluate the role of NF1 in seizures pathogenesis in a cohort of children with NF1 and seizures., Methods: The medical records of 437 children (0-18 years old) with NF1 were reviewed. All children with at least one afebrile seizure were included. Demographic, clinical, neurological, NF1 mutation status, and EEG data were collected along with brain magnetic resonance imaging. Depending on etiology, structural seizures have been identified and were further classified as NF1 related or not., Results: Nineteen patients (4.3%; 13 males) were included. NF1 was inherited in 7 (37.5%), with 3 maternal forms. Ten children with structural seizures were identified. Seven forms were identified someway related to NF1, two of which were associated to 17q11.2 microdeletion and hypoxic-ischemic encephalopathy. Any brain lesion that could explain seizures was found in nine patients, two third of these patients had a familiar history of epilepsy., Conclusions: Our results suggest seizures are more frequent in NF1 children (4.3%) than in general pediatric population (0.3-0.5%) and that are someway related to NF1 in half of patients. Facing seizures in NF1, the clinician should first exclude brain tumors but also other, and rarer NF1-related scenarios, such as hydrocephalous and vasculopathies. Children with non-structural seizures frequently had a family history of epilepsy, raising questions about the pathogenic role of NF1. They should be approached as for the general population.

OBJECTIVE In the past, the outcome of surgical treatment for thalamic tumor was poor. These lesions were often considered inoperable. However, contemporary microsurgical techniques, together with improvements in neuroimaging that enable accurate presurgical planning, allow resection to be accomplished in a safer way. METHODS The medical records, imaging studies, and operative and pathology reports obtained for pediatric patients who were treated for thalamic tumors at the authors' department were reviewed. Neuronavigation and intraoperative monitoring of motor and somatosensory evoked potentials were used. Preoperative tractography, which helped to identify internal capsule fibers, was very important in selecting the surgical strategy. Postoperatively, an MRI study performed within 24 hours was used to assess the extent of tumor resection as partial (≤ 90%), subtotal (> 90%), or gross total (no residual tumor). RESULTS Since 2002, 27 children with thalamic tumors have been treated at the authors' department. There were 9 patients with unilateral thalamic tumors, 16 with thalamopeduncular tumors, and 2 with a bilateral tumor. These last 2 patients underwent endoscopic biopsy and implantation of a ventriculoperitoneal shunt. Thirty-nine tumor debulking procedures were performed in the remaining 25 patients. Different surgical approaches were chosen according to tumor location and displacement of the posterior limb of the internal capsule (as studied on axial T2-weighted MRI) and corticospinal tract (as studied on diffusion tensor imaging with tractography, after it became available). In 12 cases, multiple procedures were performed; in 7 cases, these were done as part of a planned multistage resection. In the remaining 5 cases, the second procedure was necessary because of late recurrence or regrowth of residual tumor. At the end of the surgical phase, of 25 patients, 15 (60%) achieved a gross-total resection, 4 (16%) achieved a subtotal resection, and 6 (24%) achieved a partial resection. Eighteen patients harbored low-grade tumors in our series. In this group, the mean follow-up was 45 months (range 4-132 months). At the end of follow-up, 1 patient was dead, 12 patients were alive with no evidence of disease, 4 patients were alive with stable disease, and 1 was lost to follow-up. All patients were independent in their daily lives. The outcome of high-grade tumors in 9 patients was very poor: 2 patients died immediately after surgery, 6 died of progressive disease, and 1 was alive with residual disease at the time of this report. CONCLUSIONS This institutional review seems to offer further evidence in favor of attempts at radical resection in pediatric patients harboring unilateral thalamic or thalamopeduncular tumors. In low-grade gliomas, radical resection in a single or staged procedure can be curative without complementary treatment. Recurrences or residual regrowth can be safely managed surgically. In high-grade tumors, the role of and opportunity for radical or partial resection remains a matter of debate.

Background: Hydrocephalus in patients with neurofibromatosis (NF) type 1 is usually obstructive and may arise secondary to tumoral or nontumoral causes. Treatment of hydrocephalus in these patients is often challenging owing to combined pathologies and unique anatomic changes. The use of endoscopic third ventriculostomy (ETV) as treatment has rarely been described in this group. We aimed to characterize indications, considerations, and outcome of ETV in patients with NF 1 gathered in a multicenter international cohort., Methods: Five centers participated in this retrospective study. Following institutional review board approval, data and images were collected. Patients of all ages with NF 1 who underwent ETV for treatment of obstructive hydrocephalus were included. Patients who had no postoperative radiologic or clinical follow-up were excluded. ETV failure was defined as recurrent clinical or radiologic signs of hydrocephalus., Results: The study included 42 patients. Common etiologies for hydrocephalus were aqueductal/tectal tumor (31%), aqueductal web (26%), and aqueductal stenosis owing to NF-related changes (14%). Ten patients had a preoperative diagnosis of optic pathway glioma. ETV failures were identified in 6 patients within 1 month, in 3 patients within 9 months, and in 1 patient within 4 years. ETV was successful in 32 patients (76%) with a mean follow up of 59.4 months ± 50.9 (range, 4 months to 15 years)., Conclusions: ETV is a safe treatment for selected patients with NF 1 and obstructive hydrocephalus. Individual anatomic and pathologic aspects should be taken into consideration., (Copyright © 2017 Elsevier Inc. All rights reserved.)

OBJECTIVE Hydrocephalus remains one of the more common pathologies managed in pediatric neurosurgical units. Endoscopic third ventriculostomy (ETV) has an advantage over ventriculoperitoneal shunting as it enables patients to remain device free. Multiple shunt devices with various valve designs exist, with no one valve proven to be superior to another. The aim of this study was to describe the management of hydrocephalus and its long-term outcome. METHODS The authors retrospectively reviewed the medical records of all patients who had been treated for hydrocephalus at the Hôpital Necker-Enfants Malades in the period from 1985 to 1995. RESULTS Nine hundred seventy-five children had been treated for hydrocephalus. The mean follow-up was 11 ± 7.4 years (mean ± standard deviation). The most common cause of hydrocephalus was tumor related (32.3%), followed by malformative (24.5%) and inflammatory (20.9%) causes. Two hundred eighty patients underwent ETV as the first-line treatment. The procedure was effective in controlling hydrocephalus due to posterior fossa tumors and aqueductal stenosis. Six hundred ninety-five children had initial shunt insertion, with the majority receiving an Orbis-Sigma valve (OSV). The overall OSV shunt survival was 70% at 1 year, 58% at 10 years, and 49% at 20 years. The most common cause for mechanical shunt failure was obstruction (50.7%). Overall shunt survival was statistically different between the OSV and the differential-pressure valve (p = 0.009). CONCLUSIONS Endoscopic third ventriculostomy is effective in the management of childhood hydrocephalus. Its success is directly related to the underlying pathology. In the long term, the OSV has significantly higher event-free shunt survival than the classic differential-pressure valve systems.

Moyamoya syndrome (MMS) is the most common cerebral vasculopathy among children with neurofibromatosis type 1 (NF1). In this study, we clinically, radiologically, and genetically examined a cohort that was not previously described, comprising European children with NF1 and MMS. The NF1 genotyping had been registered. This study included 18 children. The mean age was 2.93 ± 3.03 years at the NF1 diagnosis and 7.43 ± 4.27 years at the MMS diagnosis. In seven patients, MMS was diagnosed before or at the same time as NF1. Neuroimaging was performed in 10 patients due to clinical symptoms, including headache (n = 6), cerebral infarction (n = 2), and complex partial seizures (n = 2). The remaining eight children (47%) had MMS diagnosed incidentally. Sixteen children were characterized molecularly. The features of MMS were similar between patients with and without NF1. Additionally, the NF1 phenotype and genotype were similar between children with and without MMS. Interestingly, three children experienced tumors with malignant histology or behavior. The presence of two first cousins in our cohort suggested that there may be potential genetic factors, not linked to NF1, with an additional role respect of NF1 might play a role in MMS pathogenesis. The incidental diagnosis of MMS, and the observation that, among children with NF1, those with MMS were clinically indistinguishable from those without MMS, suggested that it might be worthwhile to add an angiographic sequence to brain MRIs requested for children with NF1. A MMS diagnosis may assist in properly addressing an NF1 diagnosis in very young children who do not fulfill diagnostic criteria., (© 2017 Wiley Periodicals, Inc.)

Melanotic neuroectodermal tumor of infancy is a rare congenital pigmented neoplasm of neural crest origin, locally aggressive, growing rapidly and developing during the 1st year of life. It most commonly arises from the maxilla, cranial vault, and mandible. Occasionally, it exhibits malignant behavior with local lymph nodes involvement. Cases misdiagnosed and left untreated for a long time can present challenges due to the tumor mass and infiltration. In these cases, adjuvant chemotherapy can be extremely helpful before radical excision. Authors of this report describe a 4-year-old boy from a developing country who was referred to their hospital with an ulcerated bulging lesion in the midline/right parietooccipital region, extending to the right laterocervical and parotid regions, resulting in significant craniofacial deformation. Magnetic resonance imaging of the brain revealed a highly enhancing tumor with intracranial and extracranial development extending mainly at the level of the right parietooccipital region, with lytic and hypertrophic alterations of the skull. The patient was managed with neoadjuvant and adjuvant chemotherapy and radically resective surgery on metastatic lymph nodes and the primary tumor of the skull. Scheduled radiotherapy was not performed, according to the parents' wishes. The patient returned to his native country where the lesion recurred, and he ultimately died approximately 10 months after the end of the treatment. The literature indicates that tumor removal alone has been the treatment of choice in most isolated cases, but in cases of highly advanced tumor with involvement of the skull and cervical lymph nodes, it is preferable to proceed with preoperative chemotherapy with the aim of reducing the tumor volume, allowing better technical conditions for complete surgical removal, and decreasing the risk of local recurrence or metastasis.

Objective: Endoscopic third ventriculostomy (ETV) is a safe and less-invasive treatment strategy for patients with obstructive hydrocephalus and provides excellent outcome. Nevertheless, repeat ETV in cases of ETV failure is a controversial issue., Methods: Between 1993 and 1999, 113 patients underwent a total of 126 ETVs at the Department of Neurosurgery, Mainz University Hospital. Obstructive hydrocephalus was the causative pathology in all cases. A very long-term follow-up of up to 16 years could be achieved. All medical reports of patients who received ETV were reviewed and analyzed with focus on ETV failure with following repeat ETV and its initial as well as very long-term success., Results: Thirty-one events of ETV failure occurred during the follow-up period. Thirteen patients underwent repeat ETV: 3 patients during the first 3 months (early repeat ETV), the other 10 patients after 7-78 months (late repeat ETV, mean 33 months). All repeat ETV were performed without complications. Follow-up evaluation after successful repeat ETV ranged from <1 month up to 14 years (mean 7 years). Of the 3 early revisions, 2 failed and 1 other patient died during follow-up whereas only 2 of the late repeat ETV failed. Very long-term success rate of late repeat ETV up to 14 years yielded 80%., Conclusions: Repeat ETV in cases of late ETV failures represents an excellent option for cerebrospinal fluid circulation restoration up to 14 years of follow-up. Repeat ETV in early ETV failure in contrast is not favored by the performing surgeons; and factors of ETV failure should be analyzed very carefully before a decision for repeat ETV is made., (Copyright © 2017 Elsevier Inc. All rights reserved.)

OBJECTIVE Neuroendoscopic removal of intraventricular tumors is difficult and time consuming because of the lack of an effective decompression system that can be used through the working channel of the endoscope. The authors report on the utilization of an endoscopic ultrasonic aspirator in the resection of intraventricular tumors. METHODS Twelve pediatric patients (10 male, 2 female), ages 1-15 years old, underwent surgery via a purely endoscopic approach using a Gaab rigid endoscope and endoscopic ultrasonic aspirator. Two patients presented with intraventricular metastases from high-grade tumors (medulloblastoma, atypical teratoid rhabdoid tumor), 2 with subependymal giant cell astrocytomas (associated with tuberous sclerosis), 2 with low-grade intraparaventricular tumors, 4 with suprasellar tumors (2 craniopharyngiomas and 2 optic pathway gliomas), and 2 with pineal tumors (1 immature teratoma, 1 pineal anlage tumor). Hydrocephalus was present in 5 cases. In all patients, the endoscopic trajectory and ventricular access were guided by electromagnetic neuronavigation. Nine patients underwent surgery via a precoronal bur hole while supine. In 2 cases, surgery was performed through a frontal bur hole at the level of the hairline. One patient underwent surgery via a posterior parietal approach to the trigone while in a lateral position. The endoscopic technique consisted of visualization of the tumor, ventricular washing to dilate the ventricles and to control bleeding, obtaining a tumor specimen with biopsy forceps, and ultrasonic aspiration of the tumor. Bleeding was controlled with irrigation, monopolar coagulation, and a thulium laser. RESULTS In 7 cases, the resection was total or near total (more than 90% of lesion removed). In 5 cases, the resection was partial. Histological evaluation of the collected material (withdrawn using biopsy forceps and aspirated with an ultrasonic aspirator) was diagnostic in all cases. The duration of surgery ranged from 30 to 120 minutes. One case was complicated by subdural hygroma requiring a subduro-peritoneal shunt implant. CONCLUSIONS In this preliminary series, endoscopic ultrasonic aspiration proved to be a safe and reliable method for achieving extensive decompression or complete removal in the management of intra- and/or paraventricular lesions in pediatric patients.

Introduction: Acute basilar artery occlusion (ABAO) is an infrequent but potentially fatal cause of strokes in both adults and children, and it is usually due to vertebral artery dissection (VAD). VAD has been found to be usually a consequence of traumatic vertebral artery injury. ABAO usually presents with symptoms of acute ischemic stroke (AIS) of the posterior circulation or transient ischemic attack (TIA). It may lead to death or long-term disability if not promptly recanalized. Basilar artery recanalization in children can be achieved safely and with excellent clinical outcome using endovascular thrombectomy with the new generation self-expanding and retrievable stents., Clinical Presentation: We report the case of a 23-month old baby that came to the emergency room of our hospital for progressive impairment of consciousness associated with widespread stiffness and plaintive cry, appeared after accidental fall from stroller. An emergency brain CT scan was obtained showing multiple infarction lesions in the brainstem and left cerebellum suggestive of acute stroke in posterior circulation territories. An MR scan with angiography and diffusion-weighted sequences confirmed the multiple infarction lesions and demonstrated poor representation of the flow signal at the V3 segment of the left vertebral artery and absent representation of the flow signal at the distal segment of the basilar artery suggestive of acute thrombotic occlusion. The patient was immediately referred to interventional neuroradiology unit, and digital subtraction angiography showed complete basilar artery occlusion and left vertebral artery dissection at extracranial V2-V3 segment. The patient underwent intra-arterial thrombectomy using stent retrievers and occlusion of the V2-V3 segment of the left vertebral artery. The patient survived and long-term outcome was excellent., Conclusions: To our knowledge, only nine cases of ABAO in children treated with intra-arterial thrombectomy have been previously reported in the literature. In only three cases, the Solitaire stent was applied. Our case is the first case of basilar artery occlusion treated with Solitaire stent, in a child under 24 months. The reports that are available so far indicate that basilar artery recanalization in children can be achieved safely and with excellent clinical outcome using endovascular thrombectomy with the new generation self-expanding and retrievable stents.

Background: Growing demand for transparent and standardized methods for evaluating surgical competence prompted the construction of the Neuro-Endoscopic Ventriculostomy Assessment Tool (NEVAT)., Objective: To provide validity evidence of the NEVAT by reporting on the tool's internal structure and its relationship with surgical expertise during simulation-based training., Methods: The NEVAT was used to assess performance of trainees and faculty at an international neuroendoscopy workshop. All participants performed an endoscopic third ventriculostomy (ETV) on a synthetic simulator. Participants were simultaneously scored by 2 raters using the NEVAT procedural checklist and global rating scale (GRS). Evidence of internal structure was collected by calculating interrater reliability and internal consistency of raters' scores. Evidence of relationships with other variables was collected by comparing the ETV performance of experts, experienced trainees, and novices using Jonckheere's test (evidence of construct validity)., Results: Thirteen experts, 11 experienced trainees, and 10 novices participated. The interrater reliability by the intraclass correlation coefficient for the checklist and GRS was 0.82 and 0.94, respectively. Internal consistency (Cronbach's α) for the checklist and the GRS was 0.74 and 0.97, respectively. Median scores with interquartile range on the checklist and GRS for novices, experienced trainees, and experts were 0.69 (0.58-0.86), 0.85 (0.63-0.89), and 0.85 (0.81-0.91) and 3.1 (2.5-3.8), 3.7 (2.2-4.3) and 4.6 (4.4-4.9), respectively. Jonckheere's test showed that the median checklist and GRS score increased with performer expertise ( P = .04 and .002, respectively)., Conclusion: This study provides validity evidence for the NEVAT to support its use as a standardized method of evaluating neuroendoscopic competence during simulation-based training., (Copyright © 2016 by the Congress of Neurological Surgeons)

Chiari malformation type I (CM-I) and hydrocephalus are often associated with complex craniosynostosis. On the contrary, their simultaneous occurrence in monosutural synostosis is extremely rare. The pathophysiological hypothesis is that they may alter posterior fossa growth and lead to cerebellar tonsil herniation also without skull base primary involvement. Hydrocephalus is multifactorial and may be secondary to fourth ventricle outlet obstruction. The management of these cases is quite complex and not well defined. Cranial vault remodeling should be the only treatment when CM-I is asymptomatic and not related to syringomyelia. Suboccipital decompression should be reserved only in complicated CM-I, usually as a second surgical step following the correction of the supratentorial deformity. In our opinion, the associated hydrocephalus should be treated first in order to normalize intracranial hypertension before opening the cranial sutures. We report the case of a 26-month-old child that presented with sagittal craniosynostosis, hydrocephalus, and CM-I. He was managed by performing endoscopic third ventriculostomy (ETV) first and cranial vault remodeling thereafter. Clinico-radiological outcome was very satisfying. Concerning literature is reviewed; physiopathology and surgical management are discussed.

OBJECTIVE Intraventricular choroid plexus cysts are unusual causes of acute hydrocephalus in children. Radiological diagnosis of intraventricular choroid plexus cysts is difficult because they have very thin walls and fluid contents similar to CSF and can go undetected on routine CT studies. METHODS This study reports the authors' experience with 5 patients affected by intraventricular cysts originating from the choroid plexus. All patients experienced acute presentation with rapid neurological deterioration, sometimes associated with hypothalamic dysfunction, and required urgent surgery. In 2 cases the symptoms were intermittent, with spontaneous remission and sudden clinical deteriorations, reflecting an intermittent obstruction of the CSF pathway. RESULTS Radiological diagnosis was difficult in these cases because a nonenhanced CT scan revealed only triventricular hydrocephalus, with slight lateral ventricle asymmetry in all cases. MRI with driven-equilibrium sequences and CT ventriculography (in 1 case) allowed the authors to accurately diagnose the intraventricular cysts that typically occupied the posterior part of the third ventricle, occluding the aqueduct and at least 1 foramen of Monro. The patients were managed by urgent implantation of an external ventricular drain in 1 case (followed by endoscopic surgery, after completing a diagnostic workup) and by urgent endoscopic surgery in 4 cases. Endoscopic surgery allowed the shrinkage and near-complete removal of the cysts in all cases. Use of neuronavigation and a laser were indispensable. All procedures were uneventful, resulting in restoration of normal neurological conditions. Long-term follow-up (> 2 years) was available for 2 patients, and no complications or recurrences occurred. CONCLUSIONS This case series emphasizes the necessity of an accurate and precise identification of the possible causes of triventricular hydrocephalus. Endoscopic surgery can be considered the ideal treatment of choroid plexus cysts in children.

Background: This prospective study stratified patients by surgical resection (complete = NED vs incomplete = ED) and centrally reviewed histology (World Health Organization [WHO] grade II vs III)., Methods: WHO grade II/NED patients received focal radiotherapy (RT) up to 59.4 Gy with 1.8 Gy/day. Grade III/NED received 4 courses of VEC (vincristine, etoposide, cyclophosphamide) after RT. ED patients received 1-4 VEC courses, second-look surgery, and 59.4 Gy followed by an 8-Gy boost in 2 fractions on still measurable residue. NED children aged 1-3 years with grade II tumors could receive 6 VEC courses alone., Results: From January 2002 to December 2014, one hundred sixty consecutive children entered the protocol (median age, 4.9 y; males, 100). Follow-up was a median of 67 months. An infratentorial origin was identified in 110 cases. After surgery, 110 patients were NED, and 84 had grade III disease. Multiple resections were performed in 46/160 children (28.8%). A boost was given to 24/40 ED patients achieving progression-free survival (PFS) and overall survival (OS) rates of 58.1% and 68.7%, respectively, in this poor prognosis subgroup. For the whole series, 5-year PFS and OS rates were 65.4% and 81.1%, with no toxic deaths. On multivariable analysis, NED status and grade II were favorable for OS, and for PFS grade II remained favorable., Conclusions: In a multicenter collaboration, this trial accrued the highest number of patients published so far, and results are comparable to the best single-institution series. The RT boost, when feasible, seemed effective in improving prognosis. Even after multiple procedures, complete resection confirmed its prognostic strength, along with tumor grade. Biological parameters emerging in this series will be the object of future correlatives and reports., (© The Author(s) 2016. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Purpose: Posterior fossa ependymoma comprises two distinct molecular variants termed EPN&#95;PFA and EPN&#95;PFB that have a distinct biology and natural history. The therapeutic value of cytoreductive surgery and radiation therapy for posterior fossa ependymoma after accounting for molecular subgroup is not known., Methods: Four independent nonoverlapping retrospective cohorts of posterior fossa ependymomas (n = 820) were profiled using genome-wide methylation arrays. Risk stratification models were designed based on known clinical and newly described molecular biomarkers identified by multivariable Cox proportional hazards analyses., Results: Molecular subgroup is a powerful independent predictor of outcome even when accounting for age or treatment regimen. Incompletely resected EPN&#95;PFA ependymomas have a dismal prognosis, with a 5-year progression-free survival ranging from 26.1% to 56.8% across all four cohorts. Although first-line (adjuvant) radiation is clearly beneficial for completely resected EPN&#95;PFA, a substantial proportion of patients with EPN&#95;PFB can be cured with surgery alone, and patients with relapsed EPN&#95;PFB can often be treated successfully with delayed external-beam irradiation., Conclusion: The most impactful biomarker for posterior fossa ependymoma is molecular subgroup affiliation, independent of other demographic or treatment variables. However, both EPN&#95;PFA and EPN&#95;PFB still benefit from increased extent of resection, with the survival rates being particularly poor for subtotally resected EPN&#95;PFA, even with adjuvant radiation therapy. Patients with EPN&#95;PFB who undergo gross total resection are at lower risk for relapse and should be considered for inclusion in a randomized clinical trial of observation alone with radiation reserved for those who experience recurrence., (© 2016 by American Society of Clinical Oncology.)

Background: Persistent or recurrent shunt infections can be secondary to predisposing factors, such as isolated compartments, cerebrospinal fluid leaks, or foreign bodies., Case Description: A 5-year-old girl experienced several episodes of shunt infections. After careful reevaluation of all neuroradiologic records of the patient, a foreign body in the left frontal horn of the lateral ventricle was suspected. An endoscopic approach was used to identify and remove a small fragment of bone wax., Conclusions: To our knowledge, this is the first report of intraventricular bone wax causing persistent CSF infection. The diagnosis was difficult because wax resembles air on computed tomography and on magnetic resonance imaging, and so it had been not noticed for months. Only its persistence on several images raised the suspicion of foreign body inside the ventricular system., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Object: Endoscopic third ventriculostomy (ETV) is the procedure of choice in the treatment of obstructive hydrocephalus. The excellent clinical and radiological success rates are well known. Nevertheless, very few papers have addressed the very long term outcomes of the procedure in very large series. The authors present a large case series of 113 patients who underwent 126 ETVs, and they highlight the initial postoperative outcome after 3 months and long-term follow-up with an average of 7 years., Methods: All patients who underwent ETV at the Department of Neurosurgery, Mainz University Hospital, between 1993 and 1999 were evaluated. Obstructive hydrocephalus was the causative pathology in all cases., Results: The initial clinical success rate was 82% and decreased slightly to 78% during long-term follow-up. Long-term success was analyzed using Kaplan-Meier curves. Overall, ETV failed in 31 patients. These patients underwent a second ETV or shunt treatment. A positive impact on long-term success was seen for age older than 6 months, and for obstruction due to cysts or benign aqueductal stenosis. The complication rate was 9% with 5 intraoperative and 5 postoperative events., Conclusions: The high clinical success rate in short-term and long-term follow-up confirms ETV's status as the gold standard for the treatment of obstructive hydrocephalus, especially for distinct pathologies. The patient's age and underlying pathology may influence the outcome. These factors should be considered carefully preoperatively by the surgeon.