Autonomic dysfunction in pediatric patients with headache: migraine versus tension-type headache

Purpose

To examine symptoms indicating central nervous system (CNS) autonomic dysfunction in pediatric patients with migraine and tension-type headache.

Methods

A retrospective chart review assessed six symptoms (i.e. constipation, insomnia, dizziness, blurry vision, abnormal blood pressure, and cold and clammy palms and soles) indicating central nervous system (CNS) autonomic dysfunction in 231 patients, ages 5–18 years, diagnosed with migraine, tension-type headache (TTH), or Idiopathic Scoliosis (IS).

Results

Higher frequencies of “insomnia,” “dizziness,” and “cold and clammy palms and soles” were found for both migraine and TTH patients compared to the IS control group (P < 0.001). Frequencies of all six symptoms were greater in TTH than migraine patients with “cold and clammy palms and soles” reaching significance (P < 0.001).

Conclusions

The need for prospective research investigating autonomic dysfunction in pediatric headache patients is discussed.

     

Psychiatric manifestations as initial presentation for pediatric CNS germ cell tumors,a case series

Background

Central nervous system (CNS) germ cell tumors account for 3 % of all pediatric brain tumors in the USA. Presenting symptoms are typically location based with pineal tumors presenting with obstructive hydrocephalus and suprasellar tumors with hypothalamic/pituitary dysfunction and ophthalmologic abnormalities. Psychiatric manifestations such as psychosis and behavioral changes are atypical presentations of CNS germ cell tumors, with only 11 previously reported cases.

Methods

This is a retrospective case series describing patients with CNS germ cell tumors with an atypical presentation including psychiatric manifestations. Information regarding clinical presentation, treatment course, and outcome were obtained.

Results

We report seven patients who presented with psychiatric symptoms consisting of psychomotor delay as well as behavioral and mood changes. Six of the seven patients were diagnosed ≥6 months after onset of psychiatric symptoms. All of the seven are alive but five continue to have neurologic and psychiatric issues post treatment.

Conclusions

Atypical presentations of CNS germ cell tumors can delay diagnosis and treatment and may be secondary to atypical locations as well as endocrine dysfunction manifesting as psychiatric symptoms. Delayed diagnosis did not appear to affect survival but earlier diagnosis may potentially be associated with better neurologic and psychiatric outcome. Patients who present with these symptoms and atypical neuroimaging should have a thorough evaluation for CNS germ cell tumors including serum and CSF markers. Clinicians should be aware of these less common presentations to aid in prompt diagnosis and treatment.

     

Epilepsy surgery for pediatric low-grade gliomas of the cerebral hemispheres: neurosurgical considerations and outcomes

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.

     

Influence of previous treatments on repeat surgery for recurrent craniopharyngiomas in children

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.

     

Surgical management of supratentorial ependymomas

Introduction

Supratentorial ependymomas are rare neoplasms accounting for just ten to 15 new cases in the UK per year. This article discusses the surgical management of these tumours.

Materials and methods

We present our experience over the past 12 years looking, in particular, at the location, histological grading, postoperative complications, survival and progression-free survival. A literature review of publications discussing the surgical management of ependymoma over the past 10 years is then presented.

Results

The data shows that complete surgical resection confers a significant survival advantage. There appears to be conflicting data with respect to prognosis when comparing supratentorial to infratentorial ependymoma.

Conclusion

The authors suggest complete excision and advocate, where appropriate, the use of pre and intra-operative functional mapping and second-look surgery. The trade off neurological deficit in the pursuit of complete surgical excision in some instances should be considered.

     

Transsphenoidal meningoencephalocele associated with cleft lip and palate: challenges for diagnosis and surgical treatment

Purpose

The aim of this study was to highlight the challenges for early diagnosis and the difficulties observed in surgical treatment of patients with transsphenoidal meningoencephalocele associated with cleft lip and/or palate.

Method

We evaluated six male patients treated over the course of 4 years. Five patients presented encephalic herniation with nonfunctional brain tissue; one of these presented herniation of the pituitary gland and cerebral ventricles.

Results

All the patients received surgical treatment for the cleft lip and/or palate. Only one patient underwent repair of the meningoencephalocele, via nasal endoscopy. There were no postprocedural clinical or surgical complications.

Conclusion

The tendency is to avoid neurosurgery, opting for periodic follow-up with magnetic resonance imaging. In the presence of cleft palate, palatoplasty is essential to protect the meningoencephalocele.

     

The postirradiation incidence of cavernous angioma is higher in patients with childhood pineoblastoma or primitive neuroectodermal tumors than medulloblastoma

Purpose

The purpose of this study is to investigate the incidence of cavernous angioma (CVA) in long-term survivors of childhood embryonal tumors treated by cranial irradiation.

Materials and methods

Between 1990 and 2012, we treated 25 patients (13 males, 12 females) with embryonal tumors (17 medulloblastomas, 5 primitive neuroectodermal tumors (PNET), 3 pineoblastomas) with craniospinal irradiation. Follow-up ranged from 15.5 to 289.9 months, the irradiation dose to the whole neural axis from 18 to 36 Gy, and the total local dose from 49.6 to 60 Gy. All patients underwent follow-up magnetic resonance imaging (MRI) studies at least once a year, and the diagnosis of posttreatment CVA was based solely on MRI findings.

Results

At the time of this writing, 18 were alive and free of the recurrence of the original disease or the development of secondary neoplasms other than CVA; another 2 were alive with medulloblastoma or diffuse astrocytoma. Posttreatment, 14 patients developed CVAs in the course of a median of 56.7 months; 13 of these presented with multiple CVAs. Patients who underwent radiation therapy (RT) at an age younger than 6 years developed multiple CVAs significantly earlier than those treated at a later age (p?=?0.0110). Patients with PNET or pineoblastoma developed Zabramski type 1 and 2 CVA significantly earlier than did medulloblastoma patients (p?=?0.0042).

Conclusion

We attribute the high rate of post-RT CVA in our long-term follow-up study of pediatric patients to the delivery of cranial irradiation for embryonal tumors, especially PNET and pineoblastoma, and recommend the regular, long-term follow-up of patients whose embryonal tumors were treated by cranial irradiation.

     

Onset of bladder and motor symptoms in multiple system atrophy: differences according to phenotype

Purpose

To compare the order of presentation of bladder and motor symptoms between multiple system atrophy phenotypes.

Methods

Medical records were retrospectively reviewed in 144 patients.

Results

Bladder symptoms occurred either before or within 12 months after onset of motor symptoms in significantly more patients with the cerebellar phenotype than the parkinsonian phenotype (80 vs. 53%, p = 0.003); similar results were observed for urinary incontinence (79 vs. 45%, p = 0.001).

Conclusions

Urinary dysfunction is more likely to appear either before or shortly after motor symptoms in the cerebellar phenotype than in the parkinsonian phenotype.

     

Neurosurgical tools to extend tumor resection in pediatric hemispheric low-grade gliomas: iMRI

Introduction

The treatment of low-grade gliomas (LGGs) in pediatric age is still controversial. However, most authors report longer life expectancy in case of completely removed cerebral gliomas. Intraoperative magnetic resonance imaging (iMRI) is increasingly utilized in the surgical management of intra-axial tumor in adults following the demonstration of its effectiveness. In this article, we analyze the management of LGG using iMRI focusing on its impact on resection rate and its limits in the pediatric population.

Methods

We performed review of the literature regarding the treatment of LGG using iMRI focusing on its impact on resection rate and its limits in the pediatric population. Some exemplary cases are also described.

Results

Intraoperative MRI allowed extension of tumor resection after the depiction of residual tumor at the intraoperative imaging control from 21 to 52 % of the cases in the published series. Moreover, the early reoperation rate was significantly lower when compared with the population treated without this tool (0 % vs 7–14 %). Some technical difficulties have been described in literature regarding the use of iMRI in the pediatric population especially for positioning due to the structure of the headrest coil designed for adult patients.

Conclusion

The analysis of the literature and our own experience with iMRI in children indicates significant advantages in the resection of LGG offered by the technique. All these advantages are obtained without elongation of the surgical times or increased risk for complications, namely infection. The main limit for a wider diffusion of iMRI for the pediatric neurosurgical center is the cost required, for acquisition of the system, especially for high-field magnet, and the environmental and organizational changes necessary for its use.

     

Central nervous system and cervical spine abnormalities in Apert syndrome

Purpose

Apert syndrome characterized by acrocephalosyndactyly is a rare autosomal dominant congenital malformation with a prevalence of 1/65,000 births. With an extensive range of phenotypic and developmental manifestations, its management requires a multidisciplinary approach. A variety of craniofacial, central nervous system (CNS), and cervical spine abnormalities have been reported in these patients. This study aimed to determine the incidence of these CNS abnormalities in our case series.

Methods

Retrospective review of Australian Craniofacial Unit (ACFU) database for Apert patients was performed. Data collected that included demographics, place of origin, age at presentation, imaging performed, and images were reviewed and recorded. Where available, developmental data was also recorded.

Results

Ninety-four patients seen and managed at the ACFU had their CNS and cervical spine abnormalities documented. The main CNS abnormalities were prominent convolutional markings (67 %), ventriculomegaly (48 %), crowded foramen magnum (36 %), deficient septum pellucidum (13 %), and corpus callosum agenesis in 11 %. Major C-spine findings were present in 50.8 % of patients and included fusion of posterior elements of C5/C6 (50 %) and C3/4 (27 %). Multilevel fusion was seen in 20 %. Other abnormalities were C1 spina bifida occulta (7 %) and atlanto-axial subluxation (7 %).

Conclusion

Multiple CNS and cervical spine (c-spine) abnormalities are common in Apert syndrome. The significance of these abnormalities remains largely unknown. Further research is needed to better understand the impact of these findings on growth, development, and treatment outcomes.

     

A Self-Regulatory Intervention for Patients with Head and Neck Cancer: Pilot Randomized Trial

Background

Research is yet to investigate whether psychological interventions delivered early after diagnosis can benefit patients with head and neck cancer (HNC).

Purpose

The aim of this study was to investigate the effectiveness of a brief self-regulatory intervention (targeting illness perceptions and coping) at improving HNC patient health-related quality of life (HRQL).

Methods

A pilot randomized controlled trial was conducted, in which 64 patients were assigned to receive three sessions with a health psychologist in addition to standard care or standard care alone. Participants completed questionnaires assessing HRQL, general distress, and illness perceptions at baseline and again 3 and 6 months later.

Results

Compared to the control group, patients who received the intervention had increased treatment control perceptions at 3 months (p = .01), and increased social quality of life at 6 months (p = .01). The intervention was particularly helpful for patients exhibiting distress at baseline.

Conclusion

A brief psychological intervention following HNC diagnosis can improve patient perceptions of treatment and social quality of life over time. Such interventions could be targeted to patients who are distressed in order to confer the greatest benefit.

Trial Registration Number

12614000813684.

     

Pre-operative embolization of a choroid plexus carcinoma: review of the vascular anatomy

Aim

The purpose of this case is to highlight the benefits of preoperative embolization as well as to review the vascular anatomy that needs to be recognized in order to perform pre-operative embolization of choroid plexus tumors.

Method

We achieve this by presenting the case of a 12-month-old female who had symptoms of raised intracranial pressure. MRI demonstrated a large vividly enhancing mass centered within the atria of the right lateral ventricle associated with hydrocephalus in keeping with a choroid plexus tumor.

Result

Enlarged anterior and posterior choroidal arteries supplying the tumor were noted on the MRI scan. Pre-operative embolization was performed with NBCA glue via the anterior and posterior choroidal arteries. Subsequently, total surgical resection was achieved with only 200 cc of blood loss. Pathology confirmed a choroid plexus carcinoma.

Conclusion

Pre-operative embolization can be useful in minimizing blood loss during excision of choroid plexus tumors. It is important to understand the anatomy of the anterior and posterior choroidal arteries to perform embolization of these tumors safely.

     

Preoperative neurocognitive evaluation as a predictor of brain tumor grading in pediatric patients with supratentorial hemispheric tumors

Objective

The objective of the present study was to retrospectively evaluate the relationship between tumor grading and a selective evaluation of neurocognitive and behavioral functions in children with supratentorial hemispheric brain tumors.

Methods

Children admitted with a diagnosis of supratentorial hemispheric tumors involving the cerebral hemispheres or the thalamus at the Pediatric Neurosurgery Unit of the Catholic University of Rome between January 2008 and January 2014 were considered for the present study. Exclusion criteria were represented by age less than 2 years, severe neurological deficits, seizures, and a metastatic disease. A selective neurocognitive and behavioral workout was used for children aged less and more than 5 years.

Results

Global cognitive functions as well as selective neurocognitive and behavioral profiles were found to be significantly worse in children with low-grade tumors, compared with those affected by higher-grades histotypes. Frontal locations for cortical tumors and thalamic lesions were significantly related with worse results, with a clear contribution of dominant vs. nondominant hemisphere involvement and an age higher than 5 years.

Conclusions

Preoperative global and selective neurocognitive evaluation might contribute to the prediction of the tumor aggressiveness. Due to a longer clinical history, more benign tumors more frequently arrive to the diagnosis with a neurocognitive compromise in spite of an apparently mild presence of neurological symptoms and signs.

     

Painful pediatric traumatic neuroma: surgical management and clinical outcomes

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.

     

Clinical Relevance of Steroid Use in Neuro-Oncology

Background

Corticosteroids are commonly used in the management of primary central nervous system (CNS) tumors and CNS metastases to treat cancer- and treatment-related cerebral edema and improve neurologic function. However, they are also associated with significant morbidity and mortality, given their wide range of adverse effects.

Purpose of Review

To review the mechanism of action, pharmacology, and toxicity profile of corticosteroids and to critically appraise the evidence that supports their use in neuro-oncologic practice based on the latest scientific and clinical data.

Recent Findings

Recent data suggest that corticosteroids may negatively impact survival in glioma patients. In addition, corticosteroids should be incorporated as a standard criterion to assess a patient’s clinical and radiographic response to treatment.

Summary

Corticosteroids should be used judiciously in neuro-oncologic patients, given the potential deleterious effects on clinical outcome and patient survival. Anti-angiogenic agents, which lack these adverse effects, may be a reasonable alternative to corticosteroids.

     

Suicidal deaths in elementary school students in Korea

Background

The purpose of this study was to determine the characteristics of childhood suicidal deaths among elementary school students that occurred from 2011 to 2015 in Korea.

Methods

The report form of each suicide case by the teacher in charge to the Education Ministry was reviewed retrospectively.

Results

There were 19 suicidal deaths (12 boys, 7 girls) in elementary school students. The youngest case was a third grader (n = 1). Jumping from heights (n = 12) was the most frequently used method. Most suicides (n = 12) were committed in their homes.

Conclusion

These results highlight the alarming trend of early suicidal deaths and the importance of early suicide prevention strategies, especially in schools.

     

Acute Resective Surgery for the Treatment of Refractory Status Epilepticus

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.

     

Spinal cavernous extradural angiolipoma manifesting as a spontaneous spinal epidural hematoma in a child

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.

     

Awake surgery for hemispheric low-grade gliomas: oncological,functional and methodological differences between pediatric and adult populations

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.