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Background: Incidental intradural tumors of the spine in the pediatric population are rare lesions whose management remains unclear. Surgeons must balance the risks of iatrogenic deficits and complications after surgical resection against the risks from progressive growth of the tumor. Moreover, the natural history of an incidental finding can be difficult to predict. Here we review the literature on incidental intradural tumors of the spine and present considerations for their management. Summary: Growth of the tumor or changes in radiographic features are usually indications for resection. Asymptomatic lesions can be found in patients with genetic syndromes that predispose to tumor formation, such as neurofibromatosis type 1 and 2, schwannomatosis, and Von-Hippel-Lindau syndrome, and careful workup of a genetic cause is warranted in any patient presenting with multiple tumors and/or cutaneous features. Close follow-up is generally favored given the heavy tumor burden; however, some recommend pre-emptive resection to prevent permanent neurological deficits. Incidental intradural tumors can also occur in association with hydrocephalus, significant syringomyelia, and cord compression, and surgical treatment is usually warranted. Tumors may also be discovered as part of the workup for scoliosis, where they are not truly incidental to the scoliosis but rather are contributing to curve deformation. Key Messages: Thorough workup of patients for associated genetic syndromes or comorbidities should be undertaken in pediatric patients with incidental intradural tumors. Further research is needed into the natural history of these incidental lesions. Incidental tumors can often be managed conservatively with close follow-up, with surgical intervention warranted for expanding tumors or new-onset symptoms.

Purpose: Moyamoya disease/syndrome (MMD) is a progressive steno-occlusive disease involving the intracranial arterial circulation. Long term stenosis/hypoperfusion can lead to intellectual disability and cognitive impairment even in the absence of documented infarct. We aim investigate any underlying network abnormalities in MMD patients with no apparent ischemic injury. Materials and Methods: We retrospectively reviewed children with MMD with normal appearing parenchyma on conventional MRI. Control patients were selected from our existing database. Clinical, surgical and demographic data, including degree of stenosis as measured semi-quantitatively on MRA, were collected. Patients with documented infarcts, low quality DTI, and NF1 were excluded from the analysis. Group and correlational connectometry with degree of stenosis were performed. Results: A total of 28 patients were reviewed with 16 patients included in the study. Median (and interquartile range) age was 10.5 (8.5 - 13.5). Between group connectometry analysis identified infratentorial and supratentorial decreased connectivity in patients compared to controls, specifically in: middle cerebellar peduncle, corpus callosum, right parietopontine tract, superior cerebellar peduncle, left inferior fronto-occipital fasciculus. (Figure 1) In addition, we found negative correlation between the degree of stenosis and structural connectivity of multiple white matter pathways. Conclusion: Our findings reveal abnormal structural connectivity in children with MMD in the absence of infarcts on conventional MRI. Connectomics offers a unique opportunity to study the effect of long-term stenosis/hypoperfusion on cerebellar-cerebral networks and provide new insights into the mechanism of the structural plasticity/reorganization in these patients before bypass surgery. Future research is needed to determine longitudinal network changes before and after surgery.

Introduction Pituitary surgery has shifted in recent years from microscopic surgery(MS) to endoscopic endonasal surgery (EES). However, the comparative long-term outcome of these surgical approaches, including the need for subsequent re-operation has never been reported. We present our experience in a high-volume referral center experienced in both endoscopic and microscopic approaches to compare the need for re-operation after initial resection of non-functioning pituitary macroadenomas using these surgical approaches. Methods 684 patients (398 with NF adenomas) underwent trans-sphenoidal pituitary surgery in our institution between 2006 and 2017. Complete follow-up (mean 72 months, minimum two years) was available in 87 newly diagnosed patients with non-functioning pituitary macroadenomas (NFPMA; 48-microscopic and 39-endoscopic). The EES approach has been used almost exclusively since 2012. The need for repeat operation for tumor resection during the follow-up period was assessed as the primary end-point of the study. Extracted data included various demographic and clinical parameters, radiographic findings as well as the extent of resection (EOR). Results The EOR was similar for both groups, with a trend towards better EOR in the EES group. The rate of surgical complications was also similar for both groups. There was a strong trend towards lower need for re-operation in the EES group compared to the MS group (12.8% vs. 29.2%, p = 0.056). In a multivariate analysis, only EOR and Knosp grade were independently associated with the need for re-operation surgery. Conclusion Our data indicate that EES in NFPMA tends to be associated with a lower need for re-operation compared to the MS approach, with a similar rate of EOR and complications.

Intracranial hypertension can be an acute life-threatening event or slowly deteriorating condition, leading to a gradual loss of neurological function. The diagnosis should be taken in a timely fashioned process, which mandates expedite measures to save brain function and sometimes life. An optimal management strategy is selected according to the causative etiology with a core treatment paradigm that can be utilized in various etiologies. Distinct etiologies are intracranial bleeds caused by traumatic brain injury, spontaneous intracranial hemorrhage (e.g., neonatal intraventricular hemorrhage), or the rare pediatric hemorrhagic stroke. The other primary pediatric etiologies for elevated intracranial pressure are intracranial mass (e.g., brain tumor) and hydrocephalus related. Other unique etiologies in the pediatric population are related to congenital diseases, infectious diseases, metabolic or endocrine crisis, and idiopathic intracranial pressure. One of the main goals of treatment is to alleviate the growing pressure and prevent the secondary injury to brain parenchyma due to inadequate blood perfusion and eventually inadequate parenchymal oxygenation and metabolic state. Previous literature discussed essential characteristics of the treatment paradigm derived mainly from pediatric brain traumatic injuries' treatment methodology. Yet, many of these etiologies are not related to trauma; thus, the general treatment methodology must be tailored carefully for each patient. This review focuses on the different possible non-traumatic etiologies that can lead to intracranial hypertension with the relevant modification of each etiology's treatment paradigm based on the current literature.

Objective A paucity of literature is available discussing the associated risk factors, treatment options (including the use of minimally invasive surgery), and outcomes related to lumbar disc herniation (LDH) in children. We have discussed the risk factors for disc disease among pediatric patients and evaluated the efficacy of the minimally invasive approach. Methods A retrospective review of pediatric patients with lumbar disc disease who had undergone microdiscectomy at our institution from 2005 to 2016 was conducted. The preoperative presentation, hospital course, postoperative course, and follow-up data (≥3 years) were reviewed. We evaluated the risk factors for LDH and the surgical outcomes for both groups. Results A total of 52 pediatric patients had undergone 61 lumbar disc surgeries for LDH in our department from 2005 to 2016. Their average age at surgery was 16.65 years. Of the 61 procedures, 48 (78.7%) had been performed via the minimally invasive spine microdiscectomy approach and 13 (21.3%) via the open microdiscectomy approach. The average body mass index for all cases was 29.3 kg/m2. The average interval to diagnosis was 7.9 months. Of the 61 cases, 21 (34.4%) had been required for patients who were competitive athletes. In addition, 15 had been for LDH related to trauma (24.6%). In 46 of the 61 cases, complete resolution of the symptoms had occurred at the 1-year follow-up visit (79.2% of minimally invasive spine microdiscectomy vs 61.5% of open microdiscectomy). Conclusion Risk factors similar to those for adult LDH, such as an elevated body mass index, can be seen in the pediatric population. However, some unique risk factors such as post-traumatic LDH were found in the pediatric age group. Minimally invasive techniques are demonstrably safe and useful in this patient population.

Subependymal giant cell astrocytomas (SEGAs) are the most common intracranial tumors in Tuberous Sclerosis Complex (TSC). Very few cases of solitary SEGA without a diagnosis of TSC have been described. Most of these previously reported solitary SEGAs were located near the caudothalamic groove or in close proximity to the lateral ventricles. Here, we describe a unique case of solitary extraventricular SEGA in a 17-year-old boy who presented with new-onset seizures in the absence of the clinical and genetic diagnosis of TSC. This extraventricular SEGA was involving white matter and cortex of the occipital lobe and was predominantly hypointense on T1 and T2-weighted images with a markedly hypointense signal on susceptibility-weighted images likely secondary to dense internal calcifications. Solitary SEGA can occur in the extraventricular location in patients without TSC and should be included in the differential diagnosis of a densely calcified supratentorial intra-axial tumor in children, especially during the second decade of life.

Background Pediatric isolated penetrating traumatic vertebral artery injuries require urgent multidisciplinary management as they may lead to potentially fatal posterior circulation ischemia. Case Description We present a 19-month-old patient with an isolated penetrating vertebral artery injuries at the V2 segment of the right vertebral artery and report our strategy for her endovascular treatment which involved simultaneous coil embolization and foreign body removal. Conclusion Endovascular treatment can be considered first line treatment of oral penetrating injuries to the vertebral artery, where the penetrating object has an anterior to posterior trajectory.

Background Patients with a lesion within the amygdala and uncus may develop temporal lobe epilepsy despite having functional mesial structures. Resection of functional hippocampus and surrounding structures may lead to unacceptable iatrogenic deficits. To our knowledge, there is limited descriptions of surgical techniques for selectively resecting the amygdala and uncus lesions while preserving the hippocampus in patients with language-dominant temporal lobe pathology. Methods Thirteen patients with language-dominant temporal lobe epilepsy related to amygdala-centric lesions were identified. Patients with sclerosis of the mesial structures or evidence of pathology outside of the amygdala-uncus region were excluded. Neuropsychological evaluation confirmed normal function of the mesial structures ipsilateral to the lesion. All patients were worked up with video-EEG, high-resolution brain MRI, neuro-psychology evaluation, and either Wada or functional MRI testing. Results All patients underwent selective resection of the lesion including amygdala and uncus with preservation of the hippocampus via a transcortical inferior temporal gyrus approach to the mesial temporal lobe. Pathology was compatible with glioneuronal tumors. Post-operative MRI demonstrated complete resection in all patients. Eight of the thirteen patients underwent post-operative neuropsychology evaluations and did not demonstrate any significant decline in tasks of delayed verbal recall or visual memory based on the Rey Auditory Verbal Learning Test (RAVLT). One patient showed a slight decrease in confrontation naming using the Boston Naming Test (BNT). Seizure freedom (Engel class I) was achieved in 12 of 13 patients. Conclusion Selective transcortical amygdala and uncus resection with hippocampus preservation may be a reasonable way to achieve seizure control while sparing functional mesial structures.

OBJECTIVE Colloid cysts (CCs) are rare at all ages, and particularly among children. The current literature on pediatric CC is limited, and often included in mixed adult/pediatric series. The goal of this multinational, multicenter study was to combine forces among centers and investigate the clinical course of pediatric CCs. METHODS A multinational, multicenter retrospective study was performed to attain a large sample size, focusing on CC diagnosis in patients younger than 18 years of age. Collected data included clinical presentation, radiological characteristics, treatment, and outcome. RESULTS One hundred thirty-four children with CCs were included. Patient age at diagnosis ranged from 2.4 to 18 years (mean 12.8 ± 3.4 years, median 13.2 years, interquartile range 10.3–15.4 years; 22% were < 10 years of age). Twenty-two cases (16%) were diagnosed incidentally, including 48% of those younger than 10 years of age. Most of the other patients had symptoms related to increased intracranial pressure and hydrocephalus. The average follow-up duration for the entire group was 49.5 ± 45.8 months. Fifty-nine patients were initially followed, of whom 28 were eventually operated on at a mean of 19 ± 32 months later due to cyst growth, increasing hydrocephalus, and/or new symptoms. There was a clear correlation between larger cysts and symptomatology, acuteness of symptoms, hydrocephalus, and need for surgery. Older age was also associated with the need for surgery. One hundred three children (77%) underwent cyst resection, 60% using a purely endoscopic approach. There was 1 death related to acute hydrocephalus at presentation. Ten percent of operated patients had some form of complication, and 7.7% of operated cases required a shunt at some point during follow-up. Functional outcome was good; however, the need for immediate surgery was associated with educational limitations. Twenty operated cases (20%) experienced a recurrence of their CC at a mean of 38 ± 46 months after the primary surgery. The CC recurrence rate was 24% following endoscopic resection and 15% following open resections (p = 0.28). CONCLUSIONS CCs may present in all pediatric age groups, although most that are symptomatic present after the age of 10 years. Incidentally discovered cysts should be closely followed, as many may grow, leading to hydrocephalus and other new symptoms. Presentation of CC may be acute and may cause life-threatening conditions related to hydrocephalus, necessitating urgent treatment. The outcome of treated children with CCs is favorable.

Background: Surgical management of atlantoaxial instability (AAI) in pediatric patients with Down syndrome is associated with high neurological morbidity. Moreover, Down syndrome cognitive impairment coupled to AAI removes traditional verbal communication to relay evolving symptoms and aid in neurologic examination. It is not clear whether surgical adjuncts can alter clinical outcomes in this vulnerable population. Case Description: Herein, we report the case of a 6-year-old patient with significant developmental delay and severe AAI that was successfully managed by stabilization with guidance of neurophysiologic investigations in the perioperative phase. Conclusion: Perioperative neurophysiologic monitoring is safe, useful, and reliable in pediatric patients with trisomy 21 undergoing cervical traction and occipitocervical instrumented fusion for AAI.

The literature about the association between Chiari malformations (CMs) and scoliosis has been growing over the last three decades; yet, no consensus on the optimal management approach in this patient population has been reached. Spinal anomalies such as isolated syrinxes, isolated CM, and CM with a syrinx are relatively common among patients with presumed idiopathic scoliosis (IS), a rule that also applies to scoliosis among CM patients as well. In CM patients, scoliosis presents with atypical features such as early onset, left apical or kyphotic curvature, and neurological deficits. While spinal X-rays are essential to confirm the diagnosis of scoliosis among CM patients, a magnetic resonance imaging (MRI) is also recommended in IS patients with atypical presentations. Hypotheses attempting to explain the occurrence of scoliosis in CM patients include cerebellar tonsillar compression of the cervicomedullary junction and uneven expansion of a syrinx in the horizontal plane of the spinal cord. Early detection of scoliosis on routine spinal examination and close follow-up on curve stability and progression are essential initial steps in the management of scoliosis, especially in patients with CM, who may require full spine MRI to screen for associated neuro-axial anomalies; bracing and spinal fusion may be subsequently pursued in high-risk patients.

OBJECTIVE Drug-resistant epilepsy (DRE) during the first few months of life is challenging and necessitates aggressive treatment, including surgery. Because the most common causes of DRE in infancy are related to extensive developmental anomalies, surgery often entails extensive tissue resections or disconnection. The literature on "ultra-early" epilepsy surgery is sparse, with limited data concerning efficacy controlling the seizures, and safety. The current study's goal is to review the safety and efficacy of ultra-early epilepsy surgery performed before the age of 3 months. METHODS To achieve a large sample size and external validity, a multinational, multicenter retrospective study was performed, focusing on epilepsy surgery for infants younger than 3 months of age. Collected data included epilepsy characteristics, surgical details, epilepsy outcome, and complications. RESULTS Sixty-four patients underwent 69 surgeries before the age of 3 months. The most common pathologies were cortical dysplasia (28), hemimegalencephaly (17), and tubers (5). The most common procedures were hemispheric surgeries (48 procedures). Two cases were intentionally staged, and one was unexpectedly aborted. Nearly all patients received blood products. There were no perioperative deaths and no major unexpected permanent morbidities. Twenty-five percent of patients undergoing hemispheric surgeries developed hydrocephalus. Excellent epilepsy outcome (International League Against Epilepsy [ILAE] grade I) was achieved in 66% of cases over a median follow-up of 41 months (19-104 interquartile range [IQR]). The number of antiseizure medications was significantly reduced (median 2 drugs, 1-3 IQR, p

Intracranial hypertension can be an acute life-threatening event or slowly deteriorating condition, leading to a gradual loss of neurological function. The diagnosis should be taken in a timely fashioned process, which mandates expedite measures to save brain function and sometimes life. An optimal management strategy is selected according to the causative etiology with a core treatment paradigm that can be utilized in various etiologies. Distinct etiologies are intracranial bleeds caused by traumatic brain injury, spontaneous intracranial hemorrhage (e.g., neonatal intraventricular hemorrhage), or the rare pediatric hemorrhagic stroke. The other primary pediatric etiologies for elevated intracranial pressure are intracranial mass (e.g., brain tumor) and hydrocephalus related. Other unique etiologies in the pediatric population are related to congenital diseases, infectious diseases, metabolic or endocrine crisis, and idiopathic intracranial pressure. One of the main goals of treatment is to alleviate the growing pressure and prevent the secondary injury to brain parenchyma due to inadequate blood perfusion and eventually inadequate parenchymal oxygenation and metabolic state. Previous literature discussed essential characteristics of the treatment paradigm derived mainly from pediatric brain traumatic injuries' treatment methodology. Yet, many of these etiologies are not related to trauma; thus, the general treatment methodology must be tailored carefully for each patient. This review focuses on the different possible non-traumatic etiologies that can lead to intracranial hypertension with the relevant modification of each etiology's treatment paradigm based on the current literature.

OBJECTIVE Pediatric traumatic brain injury (TBI) is the leading cause of death among children and is a significant cause of morbidity. However, the majority of injuries are mild (Glasgow Coma Scale score 13–15) without any need for neurosurgical intervention, and clinically significant neurological decline rarely occurs. Although the question of repeat imaging within the first 24 hours has been discussed in the past, the yield of short-term follow-up imaging has never been thoroughly described. In this paper, the authors focus on the yield of routine repeat imaging for pediatric mild TBI (mTBI) at the first clinic visit following hospital discharge. METHODS The authors conducted a retrospective review of patients with pediatric brain trauma who had been admitted to Johns Hopkins All Children’s Hospital (JHACH). Patients with mTBI were identified, and their presentation, hospital course, and imaging results were reviewed. Those pediatric patients with mTBI who had undergone no procedure during their initial admission (only conservative treatment) were eligible for inclusion in the study. Two distinct groups were identified: patients who underwent repeated imaging at their follow-up clinic visit and those who underwent only clinical evaluation. Each case was assessed on whether the follow-up imaging had changed the follow-up course. RESULTS Between 2010 and 2015, 725 patients with TBI were admitted to JHACH. Of those, 548 patients qualified for analysis (i.e., those with mTBI who received conservative treatment without any procedure and were seen in the clinic for follow-up evaluation within 8 weeks after the trauma). A total of 392 patients had only clinic follow-up, without any diagnostic imaging study conducted as part of their clinic visit, whereas the other 156 patients underwent repeat MRI. Only 1 patient had a symptomatic change and was admitted after undergoing imaging. For 30 patients (19.2%), it was decided after imaging to continue the neurosurgical follow-up, which is a change from the institutional paradigm after mTBI. None of these patients had a change in neurological status, and all had a good functional status. All of these patients had one more follow-up in the clinic with new MRI, and none of them required further follow-up. CONCLUSIONS Children with mTBI are commonly followed up in the ambulatory clinic setting. The authors believe that for children with mTBI, normal clinical examination, and no new symptoms, there is no need for routine ambulatory imaging since the clinical yield of such is relatively low.

Cerebral sinovenous thrombosis (CSVT) represents the second most common type of venous thromboembolism (VTE) in children. Current literature includes limited evidence on risk factors for CSVT, particularly in the pediatric population. We sought to determine risk factors for CSVT in pediatric patients through a single-institutional case-control study. In addition, we evaluated thrombophilias, treatments and outcomes in CSVT among cases.A case-control study was performed at Johns Hopkins All Children's Hospital on patients admitted from March 31, 2006 through April 1, 2018. Cases were identified using diagnostic codes and confirmed based on electronic health record (EHR) and neuroimaging review. Controls were matched in a 2:1 fashion accounting for the month and year of admission.A total of 60 CSVT cases and 120 controls were identified. Median (range) age was 4.8 years (0-21.3 years) for cases and 5.6 years (0-20.0 years) for controls. Factors putatively associated with CSVT in unadjusted analyses were: corticosteroid use, presence of a central venous catheter, mechanical ventilation, systemic infection, head/neck infection, head/neck trauma, and chronic inflammatory disease. In the multivariable model, head/neck infection (OR: 13.8, 95% CI: 4.87-38.7; P 0.01), head/neck trauma (OR: 12.7, 95% CI: 2.88-56.2; P 0.01), and mechanical ventilation (OR: 9.32, 95% CI: 2.35-36.9; P = 0.01) remained independent, statistically-significant risk factors. 61% of patients were subacutely treated with anticoagulants and of those, only two developed relevant bleeding after initiation of therapy.This single-institutional case-control study reveals that head/neck infection, head/neck trauma, and mechanical ventilation are independent risk factors for pediatric CSVT. These findings will be further investigated via a cooperative registry of pediatric hospital-acquired VTE, by which a risk model for pediatric CSVT will be developed and validated, in order to inform future preventive strategies in at-risk pediatric patients.

Diffuse midline glioma, H3 K27M-mutant, formerly known as diffuse intrinsic pontine glioma (DIPG), is a malignant and infiltrative neoplasm of the pons. DIPG mainly affects the pediatric population and is associated with dismal prognosis, where less than 10% of sufferers survive beyond 2 years from diagnosis. The diagnosis was based upon the typical clinical presentation/progression and imaging features, and biopsy was discouraged at some point due to the associated surgical toxicity. The clinical interest and exponential growth in the number of molecular studies during the last decade have lead to the discovery of selective driver mutations. Consequently, biopsy regained popularity due to the importance of obtaining tissue for diagnostic and research purposes, and therapeutic trials are shifting from conventional strategies (radiation and chemotherapy) towards targeting unique molecular mechanisms. In this chapter, we present an overview of the current standards as well as the most important recent and clinically-relevant advances in the diagnosis and management of DIPG.

Surgical management of pontine tumors requires a high-level skill and knowledge set to achieve maximally safe surgery in a deeply eloquent region. Thorough anatomical knowledge of the trajectory towards and the neurovascular structures surrounding the pons as well as the intrapontine architecture allows the surgeon to anticipate encountering the next critical structure that should be cautiously preserved. The anterior pontine surface can be visualized directly through an endoscopic endonasal transclival approach, where the superior ventral pontine entry zone permits relatively safe access to intrapontine lesions, or through the orbito-fronto-zygomatic approach, which exposes the upper anterior pons. The transpetrosal and retrosigmoid approaches are utilized to visualize the anterolateral surface of the pons; safe entry zones through the anterolateral surface include the peritrigeminal, supratrigeminal, and lateral pontine (middle cerebellar peduncle). The dorsal pons is best targeted by a classical median suboccipital telovelar route; the floor of the fourth ventricle allows access to the posterior pontine surface through the suprafacial collicular (suprafacial triangle), infrafacial collicular (infrafacial triangle), superior foveal (lateral sulcus limitans) and medial sulcal (interfacial) safe entry zones. This chapter provides an overview of the anatomy, safe entry zones, and surgical approaches utilized to tackle pontine lesions.

The midbrain acts as a junction between the brain, lower part of the brainstem, and spinal cord. It functions as a relay station for major white matter tracts (e.g., corticospinal tracts) as well as the origin of two cranial nerve nuclei. Tumors that originate in or involve the midbrain pose a significant challenge, and their treatment usually involves several disciplines including neurosurgery and neurooncology. Surgery for midbrain tumors demands a high surgical technique and multidisciplinary approach in the operating room (e.g., neuromonitoring) and outside of it as well. The surgical approach is tailored for each and every case, taking into account the complex anatomy, specific location of the long tracts using advanced imaging, as well as the adjacent neurovascular structures that surround the surgical location. In recent years, there has been a growing interest and understanding of the molecular and genetic parameters of tumors that invade the midbrain, which, with time, will allow us to tailor more specific treatments for these patients.

OBJECTIVEHydrocephalus associated with subdural hygromas is a rare complication after decompression of Chiari malformation type I (CM-I). There is no consensus for management of this complication. The authors present a series of 5 pediatric patients who underwent CM-I decompression with placement of a dural graft complicated by posterior fossa hygromas and hydrocephalus that were successfully managed nonoperatively.METHODSA retrospective review over the last 5 years of patients who presented with hydrocephalus and subdural hygromas following foramen magnum decompression with placement of a dural graft for CM-I was conducted at 2 pediatric institutions. Their preoperative presentation, perioperative hospital course, and postoperative re-presentation are discussed with attention to their treatment regimen and ultimate outcome. In addition to reporting these cases, the authors discuss all similar cases found in their literature review.RESULTSOver the last 5 years, the authors have encountered 194 pediatric cases of CM-I decompression with duraplasty equally distributed at the 2 institutions. Of those cases, 5 pediatric patients with a delayed postoperative complication involving hydrocephalus and subdural hygromas were identified. The 5 patients were managed nonoperatively with acetazolamide and high-dose dexamethasone; dosages of both drugs were adjusted to the age and weight of each patient. All patients were symptom free at follow-up and exhibited resolution of their pathology on imaging. Thirteen similar pediatric cases and 17 adult cases were identified in the literature review. Most reported cases were treated with CSF diversion or reoperation. There were a total of 4 cases previously reported with successful nonoperative management. Of these cases, only 1 case was reported in the pediatric population.CONCLUSIONSDe novo hydrocephalus, in association with subdural hygromas following CM-I decompression, is rare. This presentation suggests that these complications after posterior fossa decompression with duraplasty can be treated with nonoperative medical management, therefore obviating the need for CSF diversion or reoperation.

OBJECTIVEPediatric cerebral venous sinus thrombosis has been previously described in the setting of blunt head trauma; however, the population demographics, risk factors for thrombosis, and the risks and benefits of detection and treatment in this patient population are poorly defined. Furthermore, few reports differentiate between different forms of sinus pathology. A series of pediatric patients with skull fractures who underwent venous imaging and were diagnosed with intrinsic cerebral venous sinus thrombosis or extrinsic sinus compression is presented.METHODSThe medical records of patients at 2 pediatric trauma centers were retrospectively reviewed. Patients who were evaluated for blunt head trauma from January 2003 to December 2013, diagnosed with a skull fracture, and underwent venous imaging were included.RESULTSOf 2224 pediatric patients with skull fractures following blunt trauma, 41 patients (2%) underwent venous imaging. Of these, 8 patients (20%) had intrinsic sinus thrombosis and 14 patients (34%) displayed extrinsic compression of a venous sinus. Three patients with intrinsic sinus thrombosis developed venous infarcts, and 2 of these patients were treated with anticoagulation. One patient with extrinsic sinus compression by a depressed skull fracture underwent surgical elevation of the fracture. All patients with sinus pathology were discharged to home or inpatient rehabilitation. Among patients who underwent follow-up imaging, the sinus pathology had resolved by 6 months postinjury in 80% of patients with intrinsic thrombosis as well as 80% of patients with extrinsic compression. All patients with intrinsic thrombosis or extrinsic compression had a Glasgow Outcome Scale score of 4 or 5 at their last follow-up.CONCLUSIONSIn this series of pediatric trauma patients who underwent venous imaging for suspected thrombosis, the yield of detecting intrinsic thrombosis and/or extrinsic compression of a venous sinus was high. However, few patients developed venous hypertension or infarction and were subsequently treated with anticoagulation or surgical decompression of the sinus. Most had spontaneous resolution and good neurological outcomes without treatment. Therefore, in the setting of pediatric skull fractures after blunt injury, venous imaging is recommended when venous hypertension or infarction is suspected and anticoagulation is being considered. However, there is little indication for pervasive venous imaging after pediatric skull fractures, especially in light of the potential risks of CT venography or MR venography in the pediatric population and the unclear benefits of anticoagulation.

Chronic subdural hematoma (CSDH) tends to be associated with elderly male, non-significant head trauma and anticoagulation therapy. The origin of the hematoma is believed to be a tear in a bridging vein. A CSDH rarely develops acute symptoms, but is rather characterized by progressive neuro-cognitive impairment and/or focal neurological deficits. Head trauma can be identified as the cause of a CSDH in about 60% of cases.We describe an unusual case of a spontaneous non-traumatic of CSDH in a middle aged female patient. This CSDH demonstrated a more therapy resistant behavior and more extensive surgical evacuation was necessary. The final pathology report revealed extramedullary hematopoietic tissue. Keywords: Extra medullary hematopoiesis, Chronic sub-dural hematoma, Spontaneous, Craniectomy

Background Gliomatosis cerebri is a rare diffusely infiltrating malignant glial neoplasm. Presenting symptoms include seizures, neurologic deficits, and frequently symptoms related to increased intracranial pressure (ICP). Surgical intervention, including brain biopsy, may induce worsening of these neurologic symptoms. We reviewed our database to identify prognostic and risk factors for perioperative deterioration specifically associated with elevated ICP. Methods Between 2006 and 2014, 78 patients were treated for gliomatosis cerebri. Ten patients required perioperative emergent treatment for elevated ICP. The clinical course and outcome of these 10 patients (study group) were characterized and compared with the remaining 68 patients. Results The study group patients developed life-threatening symptoms of increased ICP and required urgent decompressive craniectomy ( n = 5 urgent decompressive craniectomy after biopsy, n = 2 urgent decompressive craniectomy on admission) or aggressive medical therapy ( n = 3). Demographic and clinical variables were similar in both groups. In patients with severe symptoms of increased ICP, enhancing tumor volume was significantly greater than in asymptomatic patients. Radiologic evidence of obliteration of the basal cisterns and herniation was more common in symptomatic patients. The proliferation index in the biopsy specimens of tumors was also significantly higher in patients with symptomatic ICP elevation. Conclusions Clinical symptoms and radiologic appearance suggestive of elevated ICP at presentation, volume of contrast enhancement, and high Ki-67 proliferation index may predict the need for aggressive rapid treatment to control ICP in a small but significant subset of patients with GC. Further studies are needed to clarify the biologic basis for the unusual clinical course in these tumors.

Dumbbell schwannoma of the cervical spine is a known entity,1-5 and should be radically resected with the preservation or improvement of neurological function. However, to our knowledge, an operative video of a C1-C2 cervical dumbbell schwannoma with ventral extension and dorsal spinal cord compression has not been reported previously. This tumor resection video performed by the senior author (KIA) includes details of dural opening, and techniques for microsurgical resection and for postoperative closure to avoid cerebrospinal fluid (CSF) leak and pseudomeningocele formation. Fat grafting was performed through a small paraumbilical incision. The patient was prone in MAYFIELD 3-point pin fixation (Integra LifeSciences, Plainsboro Township, New Jersey). Intraoperative neurophysiological electrodes were placed for somatosensory evoked potential (SSEP) and motor evoked potential (MEP) monitoring. Stealth neuronavigation was used to aid in tumor localization. A small suboccipital craniectomy and C1 laminectomy were performed before opening the dura. Using a microsurgical technique, the dura was opened in the form of the letter “Y.” The right-sided dentate ligament was cut to aid in the mobilization of the tumor away from the spinal cord. After dividing the tumor at the dumbbell isthmus, the ventral tumor component was removed, with attention paid to the division of a perforator coming from the vertebral artery. Intraforaminal tumor debulking was performed with a cavitron ultrasonic surgical aspirator (CUSA) and resected. High cervical dumbbell schwannoma should be radically resected while preserving and improving preoperative neurological function. Avoidance of CSF leak and formation of pseudomeningocele should be planned at the beginning, utilizing fascia and fat graft to avoid this feared complication. The patient provided written consent and permission to publish her image.

Chordoma is a rare bony malignancy known to have a high rate of local recurrence after surgery. The best treatment paradigm is still being evaluated. We report our experience and review the literature. We emphasize on the difference between endoscopic and open craniotomy in regard to the anatomical compartment harboring the tumor, the limitations of the approaches and the rate of surgical resection. We retrospectively collected all patients with skull-base chordomas operated on between 2004 and 2014. Detailed radiological description of the compartments being occupied by the tumor and the degree of surgical resection is discussed. Eighteen patients were operated on in our facility for skull-base chordoma. Seventeen endoscopic surgeries were done in 15 patients, and 7 craniotomies were done in 5 patients. The mean age was 48.9 years (±19.8 years). When reviewing the anatomical compartments, we found that the most common were the upper clivus (95.6%) and lower clivus (58.3%), left cavernous sinus (66.7%) and petrous apex (∼60%). Most of the patients had intradural tumor involvement (70.8%). In all craniotomy cases, there was residual tumor in multiple compartments. In the endoscopic cases, the most difficult compartments for total resection were the lower clivus, and lateral extensions to the petrous apex or cavernous sinus. Our experience shows that the endoscopic approach is a good option for midline tumors without significant lateral extension. In cases with very lateral or lower extensions, additional approaches should be added trying to achieve complete resection.

OBJECTIVE The aim of this study was to assess in-hospital (immediate) postoperative pain scores and analgesic consumption (primary goals) and preoperative anxiety and sleep quality (secondary goals) in patients who underwent craniotomy and were treated with pregabalin (PGL). Whenever possible, out-of-hospital pain scores and analgesics usage data were obtained as well. METHODS This prospective, randomized, double-blind and controlled study was conducted in consenting patients who underwent elective craniotomy for brain tumor resection at Tel Aviv Medical Center between 2012 and 2014. Patients received either 150 mg PGL (n = 50) or 500 mg starch (placebo; n = 50) on the evening before surgery, 1.5 hours before surgery, and twice daily for 72 hours following surgery. All patients spent the night before surgery in the hospital, and no other premedication was administered. Opioids and nonsteroidal antiinflammatory drugs were used for pain, which was self-rated by means of a numerical rating scale (score range 0–10). RESULTS Eighty-eight patients completed the study. Data on the American Society of Anesthesiologists class, age, body weight, duration of surgery, and intraoperative drugs were similar for both groups. The pain scores during postoperative Days 0 to 2 were significantly lower in the PGL group than in the placebo group (p < 0.01). Analgesic consumption was also lower in the PGL group, both immediately and 1 month after surgery. There were fewer requests for antiemetics in the PGL group, and the rate of postoperative nausea and vomiting was lower. The preoperative anxiety level and the quality of sleep were significantly better in the PGL group (p < 0.01). There were no PGL-associated major adverse events. CONCLUSIONS Perioperative use of twice-daily 150 mg pregabalin attenuates preoperative anxiety, improves sleep quality, and reduces postoperative pain scores and analgesic usage without increasing the rate of adverse effects. Clinical trial registration no.: NCT01612832 (clinicaltrials.gov)

We present a case of the spinal accessory nerve traversing a fenestrated internal jugular vein. Awareness of this variant may be important in neurosurgical procedures that involve upper cervical exposures.

Intraventricular tumors are rare and comprise a variety of histological entities. They may be found throughout the ventricular system, which is comprised broadly of the lateral ventricles, third ventricle, cerebral aqueduct, and fourth ventricle. Given the widespread and varied topography of the ventricular system, its presence in the supratentorial and infratentorial compartments, as well as its diverse cellular constituency, it is not surprising that tumors arising from this complex are quite heterogeneous in location, clinical consequence, and biological behavior. The diverse nature of intraventricular tumors makes their classification more challenging than the standard intra-axial versus extra-axial categorization applied to many central nervous system lesions.

Compared to their intracranial counterparts, spinal cord tumors are quite rare, accounting for less than 10% of all central nervous system (CNS) lesions. Intramedullary spinal cord tumors (IMSCTs) comprise 20–30% of all spinal cord tumors in adults and 4–10% of all tumors of the spinal cord in children. The most frequently encountered IMSCTs are gliomas. In adults, the most common pathology is ependymoma followed by astrocytoma, comprising 60% and 30% of intramedullary spinal cord tumors, respectively. Children demonstrate the opposite incidence of pathology with astrocytomas being the most common (60%) followed by ependymoma (30%), while embryonal tumors, such as primary neuroectodermal tumors as described by the prior World Health Organization (WHO) classification, represent 4%. There has also been observed a pattern based on the age of the child. Children younger than 2 years of age will more commonly have neuroblastomas, teratomas, or dermoid tumors. Children ages 2–5 years present with astrocytomas, gangliogliomas, and epidermoid tumors. Children 6–10 year of age have a mix of tumor types. Astrocytomas predominate in children >10 years of age. Astrocytomas comprise approximately 90% of all intramedullary tumors in patients >10 years of age and about 60% of adolescent intramedullary neoplasms. By around 30 years of age, ependymomas become slightly more common than astrocytomas and predominate in the middle decades of life. After the sixth decade of life, astrocytomas and ependymomas are encountered with fairly equal frequency. Other IMSCTs include gangliogliomas, lipomas, subependymomas, hemangioblastomas, dermoids, teratomas, central neurocytomas, oligodendrogliomas, and in extremely rare cases, intramedullary metastases. There is a slight predilection for such lesions in males. A propensity for the development of spinal cord astrocytomas exists in children with neurofibromatosis type 1 (NF1). There is an additional propensity for the development of spinal cord ependymomas in children with neurofibromatosis 2 (NF2). In this chapter we are focusing on the most common malignant spinal cord tumors, astrocytomas and ependymomas, their differential diagnosis, diagnostic features, management and disease course.

Historically, spinal cord tumors represent approximately 15% of all primary central nervous system (CNS) tumors, whereas the newest classification of these intramedullary spinal cord tumors (IMSCTs) encompasses only 2–4% of all CNS tumors. The more prevalent intramedullary lesions include astrocytoma and ependymoma, which are thoroughly described elsewhere in this book. Astrocytoma followed by ependymoma are the most common IMSCTs found in children, while in adults ependymomas are more frequent. The less common IMSCTs are hemangioblastoma and spinal cord metastases, which are much more common in the adult population. Exceedingly rare lesions, such as lymphoma, primitive neuroectodermal tumor (PNET), lipoma, and neuroenteric cysts, paraganglioma, ganglioneuroma, and oligodendroglioma—among others—have been documented in both the adult and pediatric literature and will be elaborated on in this chapter. Spinal cord hemangioblastoma, spinal cord metastases, and spinal cord cavernous angioma are thoroughly described elsewhere in this book.

Surgical resection and stereotactic radiosurgery (SRS) are well-established treatment options for selected patients with oligo-brain metastases (BMs). The dynamics of edema resolution with each treatment method have not been well characterized.Of 389 patients treated for BMs between 2012 and 2014, this study retrospectively identified 107 patients (150 metastases) who underwent either surgery or SRS as a single treatment method for BMs. The two groups of patients were matched for clinical parameters. Volumetric assessments of the tumor and associated edema were performed before treatment and then 2-3 months after treatment.In this study, 76 surgical cases were compared with 74 cases treated with SRS. The volume of the tumor and surrounding edema was significantly greater in the surgery group than in the SRS group. However, resolution of edema was significantly more rapid in the surgical group (p 0.0001), accompanied by faster weaning from steroids. After a matching process based on the propensity of a patient to receive SRS, a subgroup cohort was analyzed (mean maximal diameter: 21 mm in the surgical group vs 20.8 mm in the SRS group; p = 0.9). At diagnosis, edema volume, but not tumor volume, was significantly greater in the surgical group. The resolution of edema 2-3 months after treatment was better in the surgical group than in the SRS group (89.6% vs 71.1% of baseline, respectively; p = 0.09), although this difference did not reach the level of significance.Resolution of tumor-associated edema in BMs suitable for either surgery or SRS was significantly faster after surgical resection than after SRS. Accordingly, when both treatment options are suitable, surgery appears to induce faster resolution of the edema.

Introduction Radiation necrosis (RN) and pseudoprogression are known as postradiation treatment effects and may simulate tumor progression. The disease course of glioblastoma patients who had developed RN and the impact of resecting RN on survival have not been evaluated. This study examines the clinical course of patients considered candidates for repeat surgery for a recurring brain mass proven to be RN and compared these with patients who had true tumor recurrence at surgery. Methods Of 159 patients with glioblastoma who were reoperated on because of a presumed recurrent tumor requiring repeat surgery, 18 had RN as the major component of the resected mass. The characteristics and outcome of these 18 patients were retrospectively analyzed and compared with patients in whom active and bulky tumor was found during surgery. Results Radiation necrosis occurred significantly earlier than true tumor recurrence. Patients with RN harbored larger lesions and were significantly more symptomatic before the second surgery. Most patients with RN who underwent GTR of the lesion in the second operation experienced faster resolution of the surrounding edema compared with patients who underwent STR or biopsy only. There was no significant difference in survival between the 2 groups. Conclusions These data provide an opportunity to examine the clinical course of a selected group of patients with histologically verified RN. Although RN is associated with more severe neurologic symptoms that improve after surgery, its occurrence or surgical removal carries no survival advantage compared with patients who undergo a repeat operation for true tumor recurrence.

Pediatric posterior fossa tumors are usually ependymoma, pilocytic astrocytoma, or medulloblastoma. Ependymoma appears well-demarcated with heterogeneous enhancement on magnetic resonance imaging (MRI). Full neural axis MRI is indicated to assess for metastatic disease. Management is typically surgical resection of the tumor, with consideration for cerebrospinal fluid diversion if patients present with severe hydrocephalus. Extent of resection of the tumor is the most important factor in predicting recurrence and overall survival, and gross total resection is ideal. Infratentorial ependymomas have 2 molecular subtypes, which has implications for responsiveness to adjuvant therapy and prognosis. Infratentorial ependymomas are biologically different from supratentorial ependymomas. Postoperative radiation improves local control.