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1.

Purpose

Seizure freedom following resection of focal cortical dysplasia (FCD) correlates with complete resection of the dysplastic cortical tissue. However, difficulty with intraoperative identification of the lesion may limit the ability to achieve the surgical objective of complete extirpation of these lesions. Intraoperative magnetic resonance imaging (iMRI) may aid in FCD resections. The objective of this study is to compare rates of postoperative seizure freedom, completeness of resection, and need for reoperation in patients undergoing iMRI-assisted FCD resection versus conventional surgical techniques.

Methods

We retrospectively reviewed the medical records of pediatric subjects who underwent surgical resection of FCD at Children’s National Medical Center between March 2005 and April 2015.

Results

At the time of the last postoperative follow-up, 11 of the 12 patients (92 %) in the iMRI resection group were seizure free (Engel Class I), compared to 14 of the 42 patients (33 %) in the control resection group (p = 0.0005). All 12 of the iMRI patients (100 %) achieved complete resection, compared to 24 of 42 patients (57 %) in the control group (p = 0.01). One (8 %) patient from the iMRI-assisted resection group has required reoperation, compared to 17 (40 %) patients in the control resection group.

Conclusion

Our results suggest that the utilization of iMRI during surgery for resection of FCD results in improved postoperative seizure freedom, completeness of lesion resection, and reduction in the need for reoperation.
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2.
3.

Introduction

Although meningiomas are frequently diagnosed in adults, it is a rare (intracranial) tumor in the pediatric population, with an incidence of 0.06/100,000. The pathology and treatment of meningiomas in adulthood has been a topic of increasing investigation. So far, the treatment of pediatric meningiomas has been extrapolated from these results. The question remains, however, whether translation of adult meningioma data into the childhood population is legitimate.

Methods

We present the case of a 3-year-old girl diagnosed with an intraventricular malignant meningioma and type 2 neurofibromatosis. She was operated on multiple times to achieve complete resection and received adjuvant chemotherapy. Since, she has been stable with no neurological sequelae and/or recurrence of the meningioma.

Conclusion

Pediatric meningiomas are rare tumors and differ from their adult counterparts in various aspects. We believe that gross total resection of meningioma in the pediatric population, when possible, is the treatment of choice. In the event of a subtotal resection, repeat resection is recommended. Any adjuvant treatment with chemotherapy or radiation therapy should be carefully considered during multidisciplinary meetings.
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4.

Purpose

Pediatric low-grade gliomas (LGGs) are the most frequent solid tumor in childhood. Based on an increasing number of literature reports, maximal safe resection is recommended as the first line of treatment whenever possible. However, distinguishing tumor tissue from the surrounding normal brain is often challenging with infiltrating neoplasms, even with the assistance of intraoperative, microscopic and conventional neuronavigation systems. Therefore, any technique that enhances the detection and visualization of LGGs intraoperatively is certainly desirable.

Methods

In this paper, we reviewed the role of intraoperative conventional ultrasound and contrast-enhanced ultrasound (CEUS) as a tool for extending tumor resection in LGGs. Moreover, our experience with this technology is reported and discussed.

Results

Both B-mode and CEUS are helpful in highlighting LGGs, detecting tumor margins and providing additional information such as vascularization, thus improving the safety of a more radical resection.

Conclusions

Although the full potentialities of the method are yet to be explored, intraoperative ultrasound is a promising tool in oncologic surgery and LGG surgery.
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5.
6.

Purpose

Craniopharyngioma is a common pediatric brain tumor, with a high rate of recurrence after primary treatment. This retrospective study investigated the effect of various primary treatments on surgical strategies and outcomes for recurrent craniopharyngiomas.

Methods

The study population comprised 35 children (mean age 8.77 years, range 1–16 years) with recurrent craniopharyngioma re-operated from January 1990 to January 2009. The recurrent craniopharyngiomas were excised whenever possible. For analysis, the patients were divided into four groups according to the primary treatment: radical tumor resection (A), incomplete tumor resection (B), radiotherapy + incomplete tumor resection (C), and Ommaya reservoir placement + incomplete tumor resection (D).

Results

Group B had a significantly shorter recurrence-free interval than groups A, C, or D. Outcomes were significantly different among the four groups. The hypothalamic status scores of groups A (2.38 ± 0.27) and C (2.28 ± 0.42) were significantly higher than that of group B (1.64 ± 0.20). There were no statistical differences between any two other groups.

Conclusions

In children, the primary treatments for craniopharyngioma should be considered when choosing the surgical strategy for recurrence. Radiotherapy before repeated surgery can result in a worse functional outcome and hypothalamic-pituitary function.
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7.

Introduction

Advanced magnetic resonance imaging (MRI) techniques is an umbrella term that includes diffusion (DWI) and diffusion tensor (DTI), perfusion (PWI), spectroscopy (MRS), and functional (fMRI) imaging. These advanced modalities have improved the imaging of brain tumors and provided valuable additional information for treatment planning. Despite abundant literature on advanced MRI techniques in adult brain tumors, few reports exist for pediatric brain ones, potentially because of technical challenges.

Review of the literature

The authors review techniques and clinical applications of DWI, PWI, MRS, and fMRI, in the setting of pediatric hemispheric low-grade gliomas.

Personal experience

The authors propose their personal experience to highlight benefits and limits of advanced MR imaging in diagnosis, grading, and presurgical planning of pediatric hemispheric low-grade gliomas.

Discussion

Advanced techniques should be used as complementary tools to conventional MRI, and in theory, the combined use of the three techniques should ensure achieving the best results in the diagnosis of hemispheric low-grade glioma and in presurgical planning to maximize tumor resection and preserve brain function.

Future perspectives

In the setting of pediatric neurooncology, these techniques can be used to distinguish low-grade from high-grade tumor. However, these methods have to be applied on a large scale to understand their real potential and clinical relapse, and further technical development is required to reduce the excessive scan times and other technical limitations.
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8.

Introduction

Pediatric low-grade gliomas (PLGG) are the most common primary central nervous system tumor in children. Patients in whom gross total resection can be achieved have an excellent overall (OS) and event-free survival (EFS) and do not require adjuvant therapy. However, children with unresectable tumors often experience multiple progressions and require additional treatment.

Conventional chemotherapy

Radiotherapy results in long-term tumor control, but it is associated with significant toxicity, making chemotherapy the preferred therapeutic option. Several chemotherapy combinations have been found to be successful in PLGG, but 5-year EFS has been below 60 % with most of them.

Molecular-based treatment

Recent molecular advances have led to a better understanding of the molecular pathways involved in the biology of LGG, allowing the development of promising tumor-specific, molecularly targeted therapies.
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9.

Introduction

Brain mapping through a direct cortical and subcortical electrical stimulation during an awake craniotomy has gained an increasing popularity as a powerful tool to prevent neurological deficit while increasing extent of resection of hemispheric diffuse low-grade gliomas in adults. However, few case reports or very limited series of awake surgery in children are currently available in the literature.

Methods

In this paper, we review the oncological and functional differences between pediatric and adult populations, and the methodological specificities that may limit the use of awake mapping in pediatric low-grade glioma surgery.

Results

This could be explained by the fact that pediatric low-grade gliomas have a different epidemiology and biologic behavior in comparison to adults, with pilocytic astrocytomas (WHO grade I glioma) as the most frequent histotype, and with WHO grade II gliomas less prone to anaplastic transformation than their adult counterparts. In addition, aside from the issue of poor collaboration of younger children under 10 years of age, some anatomical and functional peculiarities of children developing brain (cortical and subcortical myelination, maturation of neural networks and of specialized cortical areas) can influence direct electrical stimulation methodology and sensitivity, limiting its use in children.

Conclusions

Therefore, even though awake procedure with cortical and axonal stimulation mapping can be adapted in a specific subgroup of children with a diffuse glioma from the age of 10 years, only few pediatric patients are nonetheless candidates for awake brain surgery.
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10.

Purpose

Painful neuromas can limit function and decrease quality of life. Although management of traumatic neuromas in adults is well represented in the literature, traumatic neuromas are seen less frequently in children and adolescents, and their management is underrepresented in the literature. We present a sample of our clinical experience with painful pediatric neuromas and describe the surgical management and clinical outcomes of these cases.

Methods

A retrospective case review was conducted on patients treated at our pediatric tertiary care center. The same surgeon was responsible for management and follow-up of all patients.

Results

The sample of five patients was 60 % female and had a mean age of 12.2 (3–16) years. Each case was managed using a different surgical technique. All the patients had acceptable outcomes with a mean post-operative pain score of 0.4 (0–2) out of 10 and no residual functional outcomes.

Conclusions

It is important for clinicians to recognize that pediatric patients develop painful neuromas following nerve trauma and to understand the neurophysiologic basis for their management. Our report demonstrates that many of the techniques that we use for neuroma repair in adults are applicable in the pediatric population.
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11.

Introduction

Despite being the second most frequent tumor in children, pediatric central nervous system (CNS) tumors are rare, and the published European epidemiological data is limited. Our goal is to present the first surgical series of pediatric CNS tumors in Portugal and to review other similar worldwide series.

Methods

Retrospective review of all patients younger than 19 years old, operated to a CNS tumor in the Neurosurgery Department at Hospital de Santa Maria (Lisbon, Portugal) between January 2004 and December 2014. Demographic data, tumor location, clinical data, histopathology, and surgical treatment were analyzed and compared to surgical series of pediatric CNS tumors published in PubMed indexed journals over the last 20 years.

Results

We performed 253 surgeries in 215 patients, with a male:female ratio of 1.2:1 and a mean age of 9.2 years old. Primary brain tumors accounted for 95 % of all tumors and had more often a supratentorial location. Tumors of neuroepithelial tissue, particularly astrocytic tumors, embryonal tumors, neuronal and mixed neuronal-glial tumors, and oligodendrogliomas accounted for 81 % of cases. A gross-total resection was achieved in most cases. There was no mortality, and the overall morbidity was low.

Conclusions

The demography, topography, and clinical presentation of the tumors and the surgical results of this series are comparable to other European ones. We found a higher incidence of neuronal and mixed neuronal-glial tumors and oligodendrogliomas and a slight lower incidence of ependymomas. Our results should encourage further national multi-institutional studies to better characterize these tumors in the pediatric population.
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12.

Background

Diet-related environmental and policy interventions are being advocated at a population level because individual change is more likely to be facilitated and sustained if the environment within which choices are made supports healthful food options.

Purpose

This study aims to review research that examines factors having an influence on food choices in social environments, physical environments, and macroenvironments.

Methods

A snowball strategy was used to identify relevant peer-reviewed studies and reviews, with a focus on research completed in the US and published within the past 10 years.

Results

Research has identified a number of environmental factors associated with dietary intake; however, the majority of completed studies have methodological limitations which limit their credibility to guide interventions and policy changes.

Conclusions

Future research will need to emphasize multilevel investigations, examine how associations vary across population subgroups, develop a standard set of measures for assessing food environments and policies, and improve dietary assessment methodology.
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13.

Introduction

Angiolipomas are benign tumors usually manifested as subcutaneous nodules. Rarely they may form spinal extradural masses. These spinal cavernous angiolipomas have mostly been described in adults.

Diagnosis

To our knowledge, spinal cavernous angiolipomas have been reported in nine pediatric patients.

Clinical presentation

In this paper, we present a 1-year-old child presenting with a spontaneous spinal epidural hematoma from a spinal cavernous angiolipoma and highlight the importance of conducting a histopathological analysis of spontaneous hematomas in previously healthy children.
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14.

Introduction

Primary melanocytic lesions of the central nervous system originate from leptomeningeal melanocytes, with a spectrum ranging from well-differentiated benign meningeal melanocytomas to malignant melanomas. Atypical melanocytomas are borderline tumors, which have clinical and pathological characteristics between benign melanocytomas and malignant melanomas. Melanocytomas are rare in children and infrequently arise from the cavernous sinus. Approximately five patients with such an origin site have been reported.

Materials and methods

We report a 15-year-old girl with an atypical melanocytoma arising from the cavernous sinus. She underwent partial resection of the tumor and postoperative gamma knife surgery (GKS). She is stable 39 months after surgery.

Conclusion

We discuss the first pediatric case with an intracranial atypical melanocytoma arising from the cavernous sinus.
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15.

Purpose of Review

To review the clinical practice guideline landscape for depression screening in pediatric primary care and to identify current gaps in knowledge.

Recent Findings

Various organizations have recommendations that support screening for depression in pediatric primary care, although some differ based on the age of the child. To date, guidelines have been made based on indirect evidence of efficacy. For example, indirect evidence shows that several screening tools exist for use in primary care, and various primary care-administered or referred treatments for childhood depression have some evidence of efficacy (particularly among adolescents). In addition to determining the applicability of this evidence to younger children, more research is needed on the direct net benefits of screening and to identify factors that facilitate its effective implementation.

Summary

Indirect evidence supports the benefits of screening for depression in pediatric primary care; most organizations that publish screening guidelines recommend its use.
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16.

Introduction

Pediatric low-grade tumors are found in roughly 1–3 % of patients with childhood epilepsy; seizures associated with these tumors are often medically refractory and often present a significant morbidity, greater than the presence of the tumor itself.

Discussion

The unique morbidity of the seizures often requires an epilepsy surgical approach over a standard oncologic resection to achieve a reduction in morbidity for the child. Multiple quality-of-life studies have shown that unless a patient is seizure-free, they remain disabled throughout their life; the best way to achieve this in our patient population is with a multidisciplinary team approach with treatment goals focusing primarily on the epilepsy.

Conclusion

In those patients treated with gross total resection, roughly 80 % will have an Engel class I outcome and 90 % will achieve some reduction in seizure frequency with a significant improvement in quality of life.
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17.

Purpose of Review

Sport-related concussion (SRC) and mild traumatic brain injury (mTBI) have been thrust into the national spotlight, with youth athletes bearing the burden of this public health problem. The current review aims to provide a practical summary of pediatric SRC, including key terminology, return to play/school, and risk factors for post-concussion syndrome (PCS).

Recent Findings

While the majority of youth athletes recover within 2 to 4 weeks, approximately 10% of athletes experience a protracted recovery with symptoms lasting months, impacting social, scholastic, and sporting activities.

Summary

In the pediatric population, the strongest predictors of PCS are initial symptom burden and prior concussion, with mixed results behind the factors of gender, headaches, and learning disability. The role of psychiatric, family history, sports, and socioeconomic factors remain in their infancy.
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18.

Background

To identify the role of acute surgical intervention in the treatment of refractory status epilepticus (RSE).

Methods

Retrospective review of consecutive patients who underwent epilepsy surgery from 2006 to 2015 was done to identify cases where acute surgical intervention was employed for the treatment of RSE. In addition, the adult and pediatric RSE literature was reviewed for reports of surgical treatment of RSE.

Results

Nine patients, aged 20–68 years, with various etiologies were identified to have undergone acute surgical resection for the treatment of RSE, aided by electrocorticography. Patients required aggressive medical therapy with antiepileptic drugs and intravenous anesthetic drugs for 10–54 days and underwent extensive neurodiagnostic testing prior to resective surgery. Eight out of nine patients survived and five patients were seizure-free at the last follow-up. The literature revealed 13 adult and 48 pediatric cases where adequate historical detail was available for review and comparison.

Conclusions

We present the largest cohort of consecutive adult patients who underwent resective surgery in the setting of RSE. We also reveal that surgery can be efficacious in aborting status and in some can lead to long-term seizure freedom. Acute surgical intervention is a viable option in prolonged RSE and proper evaluation for such intervention should be conducted, although the timing and type of surgical intervention remain poorly defined.
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19.

Purpose

Giant cell tumors (GCT) are benign primary bone tumors, locally aggressive, affecting in long bones in young adults during the third decade. It is rare to experience this lesion in skeletally immature patients. GCT are related to a risk of local recurrence and malignant transformation.

Method

We report a rare case of a giant cell tumor of the thoracic spine in a skeletally immature girl presenting with a painful right scoliosis.

Results

MRI, CT scan, and bone scintigraphy were discordant and the percutaneous biopsy non-contributive.

Conclusion

A marginal “en bloc” resection was performed and revealed the GCT. Based on a literature review, the diagnosis and the surgical management of this case are discussed.
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20.

Purpose of Review

We review and summarize the key issues affecting general health and quality of life (QOL) of pediatric long-term survivors of brain tumors.

Recent Findings

Long-term survivors of brain tumors are at risk of considerable late morbidity and mortality. Lengthening survival in brain tumors has highlighted the deep impact of tumor and its treatment on the physical, psychological, functional, and social health and QOL of these survivors. Evolution in tumor therapy including surgery, radiotherapy, and systemic therapies, etc., has the potential to mitigate this impact to some extent.

Summary

Sensitization of health staff, policy makers, and the primary designers of clinical trials towards integration of QOL end points while measuring survival in brain tumor patients is the need of the hour. New developments in tumor therapeutics must not only provide quantitative gain but also improve the quality of survival in these long-term survivors. While majority of the issues presented pertain to survivorship in pediatric brain tumor population, similar challenges are likely to exist in young adults surviving brain tumors as well.
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