Spinal Cord Diffuse Leptomeningeal Glioneuronal Tumor Presenting without Leptomeningeal Dissemination.

BACKGROUND AND IMPORTANCE: Diffuse leptomeningeal glioneuronal tumor (DLGNT) represents a provisional entity in the 2016 World Health Organization classification of tumors; it is characterized by a widespread leptomeningeal growth and oligodendroglial-like cytology. To this day, 4 pediatric patients have been reported to present with an isolated spinal cord tumor in the absence of leptomeningeal dissemination. Gross total resection (GTR) was achieved in only 1 patient. We present the clinical and technical nuances of this unique type of tumor, as well as the second reported case of GTR in a patient with DLGNT. CLINICAL PRESENTATION: A 4-year-old boy presented to the emergency department after an episode of flaccid paralysis of bilateral lower extremities. MRI showed an intramedullary spinal cord tumor centered at T8. The patient was taken to the operative room, where a laminectomy and tumor resection were performed; cystic and solid tumor components were identified. Pathology report was consistent with DLGNT. After achieving GTR, patient is free of recurrence after a 15-month follow-up. CONCLUSION: No standard treatment for DLGNT has been identified. Current literature report surgery and chemotherapy with variable success rates. DLGNT presenting as an isolated intramedullary tumor is an uncommon condition which progression appears to be halted when treated promptly. Identifying solid and cystic components of this tumor is crucial for achieving GTR.

Outcomes following proton therapy for pediatric ependymoma.

BACKGROUND: Proton therapy can reduce the low and intermediate radiation dose to uninvolved brain tissue in children with intracranial ependymomas, which may improve functional outcomes and reduce second malignancies in survivors. Accordingly, ependymoma has become the most common pediatric tumor treated with proton therapy, yet data on efficacy and toxicity are limited. MATERIAL AND METHODS: Between June 2007 and February 2017, 179 children (≤21 years old) with nonmetastatic grade II/III intracranial ependymoma received proton therapy at our institution. Median age, 3.5 years (range, 0.7-21); 58% were male. Most (66%) tumors were in the posterior fossa and classified as WHO grade III (67%). 27% underwent multiple operations to maximize the extent of resection; ultimately 85% had a gross total or near total tumor resection before radiotherapy. 33% received preradiation chemotherapy. Median radiation dose in children ≤3 years old, 54 Gy(RBE). Most (>90%) children over 3 years old received 59.4 Gy(RBE). Patient and treatment variables were assessed for correlation with disease control. RESULTS: Median follow-up, 3.2 years. 3-year local control, progression-free survival, and overall survival rates were 85%, 76%, and 90%, respectively. First site of progression was local, metastatic, or simultaneous in 14, 17 and 6 patients, respectively. On multivariate analysis, subtotal resection was associated with inferior local control (67% vs. 88%; p ≤ .01) and progression-free survival (59% vs. 79%; p < .05). Male sex was associated with inferior progression-free (67% vs. 87%; p< .05) and overall survival (84% vs. 99%; p < .01). The 3-year CTCAE grade 2 + brainstem toxicity rate was 5.5% (95% CI: 2.9-10.2), including 1 grade 5 toxicity. CONCLUSIONS: This series of proton therapy for pediatric intracranial ependymoma demonstrates disease control comparable to photon series without unexpected toxicity. Subtotal resection and male sex were associated with inferior disease control. Additional follow-up to quantify the expected reductions in late toxicity with proton therapy is ongoing.

Hemispherectomy: historical review and recent technical advances.

The surgical options available for intractable hemispheric epilepsy have evolved since their initial description in the early 20th century. Surgical techniques have advanced, as has the ability to predict good surgical outcomes with noninvasive diagnostics. The authors review the history of hemispherectomy and detail the novel imaging and surgical strategies used to confer seizure freedom.

When in doubt, return to fundamentals (seizure semiology).

This case of drug resistant focal epilepsy highlights several invaluable teaching points. Hypotheses grounded in seizure semiology provide the best framework to ensure accurate interpretation of diagnostic testing. Without a hypothesis, information gathered from tests can be difficult to linearly piece together and can lead to poor patient outcomes. The case also provides a real-world conundrum of discordant test results that were ultimately rectified by re-visiting the initial hypothesis and cross-testing. Perinatal stroke continues to be a common etiology of drug resistant epilepsy. Patients can achieve seizure freedom and good quality of life with appropriate workup and in appropriate cases, surgery.

Tandem pediatric neurosurgery: treatment of synostosis and intractable epilepsy. Illustrative case.

BACKGROUND: Hemispherectomy is a surgical procedure reserved for hemispheric intractable epilepsy. Sagittal craniosynostosis is a congenital disorder treated with open or endoscope-assisted approaches for synostosis correction. These procedures are not commonly performed in the same setting. OBSERVATIONS: In this report, the authors present a 6-month-old female with sagittal craniosynostosis, hemimegalencephaly, and intractable epilepsy who underwent a left hemispherotomy with open sagittal synostosis correction followed by cranial molding orthosis therapy. LESSONS: The report highlights the technical nuances of the procedure, but also discusses the possible genetic disorder responsible for both conditions, megalencephaly-capillary malformation syndrome.

Review of Pediatric Neurologic History and Age-Appropriate Neurologic Examination in the Office.

The neurologic examination of an infant or child can be daunting, as they are unable to verbally communicate or follow directions. It starts with tailoring the pediatric neurologic history and examination to the child's specific age group. A good neurologic history obtained from the patient and parents is key to evaluating a pediatric patient. This article offers pearls on what information to ask the caregivers and patients, and salient aspects of a brief neurologic examination.

Advances in Epilepsy Surgery.

BACKGROUND: A large number of patients have epilepsy that is intractable and adversely affects a child's lifelong experience with addition societal burden that is disabling and expensive. The last two decades have seen a major explosion of new antiseizure medication options. Despite these advances, children with epilepsy continue to have intractable seizures. An option that has been long available but little used is epilepsy surgery to control intractable epilepsy. METHODS: This article is a review of the literature as well as published opinions. RESULTS: Epilepsy surgery in pediatrics is an underused modality to effectively treat children with epilepsy. Adverse effects of medication should be weighed against risks of surgery as well as risks of nonefficacy. CONCLUSIONS: We discuss an approach to selecting the appropriate pediatric patient for consideration, a detailed evaluation including necessary evaluation, and the creation of an algorithm to approach patients with both generalized and focal epilepsy. We then discuss surgical options available including outcome data. New modalities are also addressed including high-frequency ultrasound and co-registration techniques including magnetic resonance imaging-guided laser therapy.

Contemporary management of pediatric open skull fractures: a multicenter pediatric trauma center study.

OBJECTIVE: The authors sought to evaluate the contemporary management of pediatric open skull fractures and assess the impact of variations in antibiotic and operative management on the incidence of infectious complications. METHODS: The records of children who presented from 2009 to 2017 to 6 pediatric trauma centers with an open calvarial skull fracture were reviewed. Data collected included mechanism and anatomical site of injury; presence and depth of fracture depression; antibiotic choice, route, and duration; operative management; and infectious complications. RESULTS: Of the fractures among the 138 patients included in the study, 48.6% were frontal and 80.4% were depressed; 58.7% of patients underwent fragment elevation. The average duration of intravenous antibiotics was 4.6 (range 0-21) days. Only 53 patients (38.4%) received a single intravenous antibiotic for fewer than 4 days. and 56 (40.6%) received oral antibiotics for an average of 7.3 (range 1-20) days. Wounds were managed exclusively in the emergency department in 28.3% of patients. Two children had infectious complications, including a late-presenting hardware infection and a superficial wound infection. There were no cases of meningitis or intracranial abscess. Neither antibiotic spectrum or duration nor bedside irrigation was associated with the development of infection. CONCLUSIONS: The incidence of infectious complications in this population of children with open skull fractures was low and was not associated with the antibiotic strategy or site of wound care. Most minimally contaminated open skull fractures are probably best managed with a short duration of a single antibiotic, and emergency department closure is appropriate unless there is significant contamination or fragment elevation is necessary.

Prioritizing Pediatricians' Neurosurgical Education: Results From a National Survey of Primary Care Pediatricians.

Introduction. We surveyed nonretired American Academy of Pediatrics-member US pediatricians regarding common neurosurgical conditions, identifying specific areas of focus in education. Methods. Data were acquired via self-administered electronic questionnaire. Results. Of 505 total respondents, 56% reported neurology was not a required residency rotation, and 86% had diagnosed craniosynostosis, plagiocephaly, or macrocephaly. Craniosynostosis can mostly be diagnosed by physical examination alone, but almost 50% reported relying on skull X-rays. Fifty-four percent reported diagnosing ocular surface disease (OSD; with 15% to 40% not screening an infant despite well-established cutaneous markers). Seventy-four screened OSD in a patient with sacral dimple. Ninety-seven percent reported treating concussion, but nearly 25% did not manage these patients alone. Two out of 3 patients indicated head injury as most important for continuing education. Conclusion. Improved education for craniosynostosis, OSD, head injury, and concussion management are important for earlier diagnosis, management, and referral of some disorders, while decreasing resource utilization in others. These results should be used when considering pediatrician educational programs.

Leopard syndrome and Chiari type I malformation: a case report and review of the literature.

INTRODUCTION: The authors present a 31-year-old man with a Chiari type I malformation (CM-1) occurring in conjunction with LEOPARD syndrome. He presented with severe dysphagia requiring placement of a percutaneous endoscopic gastrostomy (PEG) tube. Evaluation included magnetic resonance imaging (MRI) of the brain and cervical spine that revealed CM-1 with an extensive cervical syrinx. The patient underwent a suboccipital craniectomy with C1 laminectomy and duraplasty. His symptoms quickly resolved and his PEG tube was removed. DISCUSSION: The occurrence of a CM-1 with LEOPARD syndrome has only been reported once, whereas CM-1 and Noonan syndrome have been linked in several cases. The similarity between LEOPARD and Noonan syndromes has been reported and many propose they represent 2 entities along a spectrum. CONCLUSION: In light of this spectrum, we propose that CM-1 should be considered in all patients presenting with LEOPARD-Noonan syndrome.

Outcomes Following Proton Therapy for Pediatric Low-Grade Glioma.

PURPOSE: Dosimetric studies show that proton therapy can reduce the low/intermediate radiation dose to uninvolved tissue in children with low-grade glioma (LGG). For this reason, LGG is the fourth most common pediatric tumor treated with proton therapy, yet clinical outcome data on efficacy and toxicity are limited. METHODS AND MATERIALS: We reviewed the medical records of 174 children (≤21 years old) with nonmetastatic LGG enrolled on a prospective protocol and treated with proton therapy between 2007 and 2017. We assessed clinical outcomes and toxicity and analyzed patient, tumor, and treatment-related variables. RESULTS: The median age was 10.2 years (range, 2-21). Fifty-eight percent of tumors were World Health Organization grade 1 and 30% were grade 2; 12% were diagnosed on imaging characteristics alone. The most common histology was pilocytic astrocytoma (47%). The most common tumor subsites were diencephalon/optic pathway (52%), caudal brainstem (16%), and cerebellum (13%). Forty-two percent received chemotherapy before radiation therapy. The median follow-up was 4.4 years. The 5-year actuarial rates of local control, progression-free survival, and overall survival were 85% (95% confidence interval [CI], 78%-90%), 84% (95% CI, 77%-89%), and 92% (95% CI, 85%-95%), respectively. On univariate analysis, brainstem/spinal cord tumor location (62% vs 90% elsewhere) and dose <54 GyRBE (67% vs 91% for 54 GyRBE) were associated with inferior local control (P < .01 for both). Twenty-two patients (12.6%) experienced acute nausea or vomiting requiring ondansetron; 2 patients (1.1%) required corticosteroids. Serious toxicities (4% of patients) included brainstem necrosis requiring corticosteroids (n = 2), symptomatic vasculopathy (n = 2), radiation retinopathy (n = 1), epilepsy (n = 1), and death from radiation-induced high-grade glioma (n = 1). Thirty-nine patients (22%) developed new-onset central hormone deficiency. Pseudoprogression was observed in 32.1%. CONCLUSIONS: Compared with modern photon series, proton therapy reduces the radiation dose to developing brain tissue, diminishing acute toxicities without compromising disease control.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on the Management of Patients With Myelomeningocele: Whether Persistent Ventriculomegaly Adversely Impacts Neurocognitive Development.

BACKGROUND: Myelomeningocele (MM) is the most common congenital anomaly to affect the nervous system and affects 1500-2000 newborn infants per year in the United States. It is accompanied by symptomatic hydrocephalus in approximately 70%-80% of patients. Different treatment strategies for hydrocephalus characteristically result in different effects on the size of the ventricles. OBJECTIVE: The objective of this systematic review was to determine whether persistent ventricular enlargement adversely impacts neurocognitive development in patients with MM. METHODS: The PubMed National Library of Medicine Medline database and Embase were queried using MeSH headings and keywords relevant to neurocognitive or intellectual development and ventricular size or morphology. Abstracts were reviewed by the authors to identify which studies met strict inclusion criteria. An evidence table was constructed that summarized the included studies and reflected the quality of evidence (Classes I-III) that each represented. A recommendation was made that is based on the quality of the evidence. RESULTS: An initial abstract review utilizing strict inclusion/exclusion criteria yielded 48 studies, 9 of which underwent full-text review. There is limited and conflicting Class III evidence from 2 studies. CONCLUSION: Currently, there is insufficient data to conclude that ventricular size and morphology impact neurocognitive development.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-5.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on the Incidence of Tethered Cord Syndrome in Infants With Myelomeningocele With Prenatal Versus Postnatal Repair.

BACKGROUND: The incidence of spina bifida (SB) is higher in the developing world as compared to the United States because of folic acid deficiency during pregnancy. Advances in technology have made prenatal repair of myelomeningocele (MM) possible. OBJECTIVE: The objective of this guideline was to determine if there is a difference in the rate of development of tethered cord syndrome (TCS) in infants who had prenatal closure compared to infants who had MM repair after birth. METHODS: The Guidelines Task Force developed search terms and strategies to search PubMed and Embase for the relevant literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used. Full text articles were reviewed and, when appropriate, included as evidence. RESULTS: A total of 261 abstracts were reviewed. Fifty-four full-text articles were selected for further analysis. Three studies met inclusion criteria. CONCLUSION: There was Class II evidence from 1 study and Class III evidence from another 2 studies demonstrating that TCS develops in infants with prenatal MM closure at an equal or higher rate than with postnatal closure. There was an increased risk of development of inclusion cysts in infants who underwent in utero closure. Continued surveillance for TCS and/or the development of inclusion cysts in children with prenatal and postnatal closure of MM is indicated (Level II). Differences between prenatal and postnatal repair with respect to the development of TCS and/or inclusion cysts should be considered alongside other relevant maternal and fetal outcomes when deciding upon a preferred method for MM closure.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-6.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Pediatric Myelomeningocele: Executive Summary.

BACKGROUND: The incidence of spina bifida (SB) in the developing world is higher than in the United States because of malnutrition and folic acid deficiency during pregnancy. Advances in technology have made prenatal repair of myelomeningocele (MM) possible. OBJECTIVE: The objective of the guidelines are, (1) To create clinical recommendations for best practices, based on a systematic review and analysis of available literature, (2) to obtain multi-disciplinary endorsement of these guidelines from relevant organizations, and (3) to disseminate the educational content to physicians to improve the care of infants with MM. METHODS: The Guidelines Task Force developed search terms and strategies used to search PubMed and Embase for literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used to screen abstracts and to develop a list of relevant articles for full-text review. RESULTS: Guidelines authors aimed to systematically review the literature and make evidence based recommendations about the timing of closure after birth, hydrocephalus, the impact of prenatal closure, and the effect of prenatal closure on ambulation ability and tethered spinal cord. Evidence concerning persistent ventriculomegaly and cognitive impairment was also evaluated. Hundreds of abstracts were identified and reviewed for each of the 5 topics. A total of 14 studies met stringent inclusion criteria. CONCLUSION: Based on a comprehensive systematic review, a total of 5 clinical practice recommendations were developed, with 1 Level I, 2 Level II and 2 Level III recommendations.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-1.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on the Incidence of Shunt-Dependent Hydrocephalus in Infants With Myelomeningocele After Prenatal Versus Postnatal Repair.

BACKGROUND: Myelomeningocele (MM) is a condition that is responsible for considerable morbidity in the pediatric population. A significant proportion of the morbidity related to MM is attributable to hydrocephalus and the surgical management thereof. Postnatal repair remains the most common form of treatment; however, increased rates of prenatal diagnosis, advances in fetal surgery, and a hypothesis that neural injury continues in utero until the MM defect is repaired have led to the development and evaluation of prenatal surgery as a means to improve outcomes in afflicted infants. OBJECTIVE: The objective of this guideline is to systematically evaluate the literature to determine whether there is a difference in the proportion of patients who develop shunt-dependent hydrocephalus in infants who underwent prenatal MM repair compared to infants who had postnatal repair. METHODS: The Guidelines Task Force developed search terms and strategies used to search PubMed and Embase for relevant literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used to screen abstracts and to develop a list of relevant articles for full-text review. Full-text articles were then reviewed, and when appropriate, included as evidence. RESULTS: A total of 87 abstracts were identified and reviewed by 3 independent reviewers. Thirty-nine full-text articles were selected for analysis. Three studies met selection criteria and were included in the evidence table. CONCLUSION: Class I evidence from 1 study and class III evidence from 2 studies suggest that, in comparison to postnatal repair, prenatal surgery for MM reduces the risk of developing shunt-dependent hydrocephalus. Therefore, prenatal repair of MM is recommended for those fetuses who meet specific criteria for prenatal surgery to reduce the risk of developing shunt-dependent hydrocephalus (level I). Differences between prenatal and postnatal repair with respect to the requirement for permanent cerebrospinal fluid diversion should be considered alongside other relevant maternal and fetal factors when deciding upon a preferred method of MM closure. The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-2.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on the Management of Patients With Myelomeningocele: Whether Prenatal or Postnatal Closure Affects Future Ambulatory Status.

BACKGROUND: Myelomeningocele (MM) is an open neural tube defect treated by pediatric neurosurgeons with prenatal or postnatal closure. OBJECTIVE: The objective of this systematic review was to answer the question: What is the evidence for the effectiveness of prenatal vs postnatal closure of MM regarding short and long-term ambulatory status? Treatment recommendations were provided based on the available evidence. METHODS: The National Library of Medicine PubMed database and Embase were queried using MeSH headings and keywords relevant to ambulatory status after prenatal or postnatal closure of MM. Abstracts were reviewed to identify which studies met the inclusion criteria. An evidence table was assembled summarizing the studies and the quality of evidence (Classes I-III). Based on the quality of the literature, a recommendation was rendered (Level I, II, or III). RESULTS: One randomized controlled trial (Class II) and 3 retrospective cohort studies (Class III) were included as evidence. Initial ambulatory status depended on anatomic level of the neural tube defect. In the short term, prenatal closure may improve ambulatory status compared to postnatal closure. Spinal cord tethering or dermoid inclusion cyst has been associated with neurologic deterioration in infants closed in utero and after birth. Ambulation may cease in both groups over time. No long-term studies evaluated whether there is a difference in the ability to ambulate upon reaching adulthood. CONCLUSION: Prenatal closure of MM may improve ambulatory status in the short term (Level II). Spinal cord tethering in both groups caused deterioration in the ability to walk. Evaluation and treatment of spinal cord tethering may help maintain ambulatory status (Level III). No studies evaluate whether prenatal or postnatal repair provides improved ability to ambulate upon reaching adulthood.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-3.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on Closure of Myelomeningocele Within 48 Hours to Decrease Infection Risk.

BACKGROUND: Appropriate timing for closure of myelomeningocele (MM) varies in the literature. Older studies present 48 h as the timeframe after which infection complication rates rise. OBJECTIVE: The objective of this guideline is to determine if closing the MM within 48 h decreases the risk of wound infection or ventriculitis. METHODS: The Guidelines Task Force developed search terms and strategies used to search PubMed and Embase for relevant literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used to screen abstracts and to develop a list of relevant articles for full-text review. Full text articles were then reviewed and when appropriate, included in the evidentiary table. The class of evidence was evaluated, discussed, and assigned to each study that met inclusion criteria. RESULTS: A total of 148 abstracts were identified and reviewed. A total of 31 articles were selected for full text analysis. Only 4 of these studies met inclusion criteria. CONCLUSION: There is insufficient evidence that operating within 48 h decreases risk of wound infection or ventriculitis in 1 Class III study. There is 1 Class III study that provides evidence of global increase in postoperative infection after 48 h, but is not specific to wound infection or ventriculitis. There is 1 Class III study that provides evidence if surgery is going to be delayed greater than 48 h, antibiotics should be given.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-4.

Choroid plexus adenoma in a child: expanding the clinical and pathological spectrum.

Primary choroid plexus tumors encompass a variety of tumors, with choroid plexus papilloma and carcinoma being the most common. Also in the differential diagnosis is the rare benign choroid plexus adenoma. As these tumors are infrequently described, the histological profile continues to evolve. The authors present a case with unusual characteristics that will broaden the pathological spectrum for choroid plexus adenomas.

Three-dimensional printing and neuroendovascular simulation for the treatment of a pediatric intracranial aneurysm: case report.

The use of simulators has been described in a variety of fields as a training tool to gain technical skills through repeating and rehearsing procedures in a safe environment. In cerebrovascular surgery, simulation of skull base approaches has been used for decades. The use of simulation in neurointervention to acquire and enhance skills before treating a patient is a newer concept, but its utilization has been limited due to the lack of good models and deficient haptics. The advent of 3D printing technology and the development of new training models has changed this landscape. The prevalence of aneurysms in the pediatric population is much lower than in adults, and concepts and tools sometimes have to be adapted from one population to another. Neuroendovascular rehearsal is a valid strategy for the treatment of complex aneurysms, especially for the pediatric population. The authors present the case of an 8-year-old boy with a fusiform intracranial aneurysm and documented progressive growth, who was successfully treated after the authors rehearsed the placement of a flow diverter using a patient-specific 3D-printed replicator system model.