Showing papers in "Childs Nervous System in 2014"

Comparison of posterior fossa decompression with or without duraplasty in children with Type I Chiari malformation.

Abstract: Chiari malformation type I (CM1) is a common and often debilitating neurosurgical disease. Whether to treat CM1 patients with a traditional posterior fossa decompression with duraplasty (PFDD) or a less invasive extradural decompression (PFDO) is controversial. The purpose of this study was to compare clinical outcome and syrinx resolution between the two procedures. We retrospectively reviewed the records of 36 patients treated with PFDD and 29 patients with PFDO between 2003 and 2011. We compared baseline demographic, clinical, and radiographic characteristics. The primary clinical outcome was the Chicago Chiari Outcome Scale (CCOS). The primary radiographic outcome was qualitative syrinx improvement or resolution. At baseline, age and sex distributions, radiographic characteristics, and presenting symptoms were similar in patients undergoing PFDD and PFDO. Patients undergoing PFDO had shorter surgical time (1.5 vs. 2.8 h; p < 0.001) and length of hospital stay (2.1 days compared to 3.3 days; p < 0.001). Cerebrospinal fluid-related complications were more common in patients receiving PFDD (7/36) than PFDO (0/29) (p = 0.014). Clinical improvement, defined by the mean CCOS score, was comparable in patients receiving PFDO (14.7) and PFDD (14.6) (p = 0.70). Among patients with postoperative syrinx imaging, 10/13 in the PFDD group improved or resolved, compared to 8/8 in the PFDO group (p = 0.26). Extradural decompression for CM1 produces comparable rates of clinical and radiographic improvement as the more invasive decompression with duraplasty. Given the increased morbidity and resource utilization associated with PFDD, PFDO should be considered an attractive first-line option for most CM1 patients.

Are pediatric brain tumors on the rise in the USA? Significant incidence and survival findings from the SEER database analysis

Abstract: Central nervous system tumors are the second most common form of cancer in children between the ages of 1 and 19 years. We aimed to provide the most recent data on the incidence and survival of these tumors in the USA and to assess the literature. Frequency, rates, and survival sessions were calculated using the November 2008 submission for the US Surveillance Epidemiology and End Results Program. Data were collected and analyzed for children and adolescents aged 1 to 19 years with primary brain tumors. We found that the incidence rate of all pediatric brain tumors has been on a gradual but steady increase from 1973 to 2008 (p < 0.001). The average annual increase was 1.37 %. Our survival analysis of the individual tumors revealed that the 5-year overall survival for children diagnosed between 1974 and 1978 with medulloblastoma was 43.7 %. However, this increased to 62.8 % for children diagnosed between 1999 and 2003. A similar survival trend was also observed when all the other pediatric brain cancer histologies were collectively analyzed (p < 0.001). From our study, we can conclude that contrary to previous reports indicating a plateau in the incidence rates of pediatric brain tumors since the mid-1980s, there has been an increase from 1973 to 2008. Potential causes include environmental carcinogens, but more research is needed to investigate the factors behind this sustained rise in incidence over the years.

The ventricular system of the brain: a comprehensive review of its history, anatomy, histology, embryology, and surgical considerations

Abstract: The cerebral ventricles have been recognized since ancient medical history. Their true function started to be realized more than a thousand years later. Their anatomy and function are extremely important in the neurosurgical panorama. The literature was searched for articles and textbooks of different topics related to the history, anatomy, physiology, histology, embryology and surgical considerations of the brain ventricles. Herein, we summarize the literature about the cerebral ventricular system.

The choroid plexus: a comprehensive review of its history, anatomy, function, histology, embryology, and surgical considerations

Abstract: The role of the choroid plexus in cerebrospinal fluid production has been identified for more than a century. Over the years, more intensive studies of this structure has lead to a better understanding of the functions, including brain immunity, protection, absorption, and many others. Here, we review the macro- and microanatomical structure of the choroid plexus in addition to its function and embryology. The literature was searched for articles and textbooks for data related to the history, anatomy, physiology, histology, embryology, potential functions, and surgical implications of the choroid plexus. All were gathered and summarized comprehensively. We summarize the literature regarding the choroid plexus and its surgical implications.

Pediatric intracranial aneurysms—our experience and review of literature

Abstract: Intracranial aneurysms in children are not as common as in adults and there are many differences in the etiology, demographic variables, aneurysm location, aneurysm morphological characteristics, clinical presentation, and outcome in pediatric and adult intracranial aneurysms. All children (≤18 years) suffering from intracranial aneurysm managed at our center from July 2001 through June 2013 were included in the study, and the details of these patients were retrieved from the computerized database of our hospital. A total of 62 pediatric patients were treated for 74 aneurysms during the study period and constituted 2.3 % of all intracranial aneurysms treated during the same period. The mean age at presentation was 13.5 years. Headache (82 %) was the commonest presenting feature; other symptoms included seizures (21 %), ictal loss of consciousness (27 %), and motor/cranial nerve deficits (22.6 %). Computed tomogram revealed subarachnoid hemorrhage in 58 % of patients. Eighty-two percent of aneurysms were in anterior circulation. Sixty-seven percent of aneurysms were complex aneurysms. Fifty-eight percent of patients underwent surgical intervention while 30 % underwent endovascular procedures. Twenty-one percent of the patients developed vasospasm. There was no postoperative mortality. Favorable outcome was seen in 72 % of the patients. Pediatric intracranial aneurysms are uncommon as compared to in adult patients. Seizures and cranial nerve involvement are seen more often as the presenting features in children. Posterior circulation aneurysms are more common in children, as are the internal carotid artery bifurcation aneurysms. There is high incidence of giant, posttraumatic, and mycotic aneurysms in children.

Pediatric intracranial arachnoid cysts: comparative effectiveness of surgical treatment options

Abstract: A variety of surgical approaches for the treatment of pediatric intracranial arachnoid cysts exist. In an effort to identify the optimal surgical treatment for this disorder, we developed a decision analytic model to evaluate outcomes of four surgical approaches in children. These included open craniotomy for cyst excision, open craniotomy for cyst fenestration, endoscopic cyst fenestration, and cystoperitoneal shunting. Pooled data were used to create evidence tables, from which we calculated incidence, relative risks, and summary outcomes in quality-adjusted life years (QALYs) for the four surgical treatments. Our study incorporated data up to 5 years postsurgery. We analyzed 1,324 cases from 36 case series. There were no significant differences in outcome among the four surgical strategies. The QALYs (maximum of 5) for surgical approaches resulted in a range from 4.79 (for open craniotomy and excision) to 4.92 (for endoscopic fenestration). Overall quality of life is comparable between patients undergoing open craniotomy for cyst excision or fenestration, endoscopic fenestration, and cystoperitoneal shunting up to 5 years after surgery. While each approach offers unique advantages and disadvantages, an individualized treatment strategy should be employed in the setting of surgical outcome equipoise.

Cause and outcome of cerebellar mutism: evidence from a systematic review

Abstract: Cerebellar mutism is a serious neurosurgical complication after posterior fossa surgery, but the cause, incidence and outcome remain incompletely defined. The aim of this paper was to identify and review all reports of this phenomenon to better delineate and improve the evidence base. A systematic search and retrieval of databases was conducted using advanced search techniques. Review/outcomes criteria were developed, and study quality was determined. The retrieval identified 2,281 papers of which 96 were relevant, identifying 650 children with cerebellar mutism. Causative factors, clinical features and outcomes were reported variably; papers focussed on multiple areas, the majority reporting incidence in single or series of case studies with little or no analysis further than description. The complexity and variability of data reporting, likely contributing factors and outcomes make cerebellar mutism difficult to predict in incidence and the degree of impact that may ensue. A clear and accepted universal definition would help improve reporting, as would the application of agreed outcome measures. Clear and consistent reporting of surgical technique remains absent. Recommendations for practice are provided.

Stem cells for brain repair in neonatal hypoxia-ischemia.

Abstract: Neonatal hypoxic-ischemic insults are a significant cause of pediatric encephalopathy, developmental delays, and spastic cerebral palsy. Although the developing brain's plasticity allows for remarkable self-repair, severe disruption of normal myelination and cortical development upon neonatal brain injury are likely to generate life-persisting sensory-motor and cognitive deficits in the growing child. Currently, no treatments are available that can address the long-term consequences. Thus, regenerative medicine appears as a promising avenue to help restore normal developmental processes in affected infants. Stem cell therapy has proven effective in promoting functional recovery in animal models of neonatal hypoxic-ischemic injury and therefore represents a hopeful therapy for this unmet medical condition. Neural stem cells derived from pluripotent stem cells or fetal tissues as well as umbilical cord blood and mesenchymal stem cells have all shown initial success in improving functional outcomes. However, much still remains to be understood about how those stem cells can safely be administered to infants and what their repair mechanisms in the brain are. In this review, we discuss updated research into pathophysiological mechanisms of neonatal brain injury, the types of stem cell therapies currently being tested in this context, and the potential mechanisms through which exogenous stem cells might interact with and influence the developing brain.

Abdominal cerebrospinal fluid pseudocyst: a comparative analysis between children and adults

Abstract: Abdominal cerebrospinal fluid (CSF) pseudocyst is a rare but important complication in patients with ventriculoperitoneal shunt (VPS). In addition to presenting our experience, we performed a comparative analysis between children and adults with this entity. To the author’s knowledge, there are no studies in which this condition has been compared. The PubMed database was searched for all relevant reports published from 1954 to 2012. The differences were statistically compared, especially regarding clinical investigations, etiology of the hydrocephalus, shunt revision, CSF infection, treatment, and recurrence. Chi-square test or Fisher’s exact test was used to find associations among the variables. Compiled from literature, we found 393 cases of abdominal pseudocyst: 295 children, including our cases, and 55 adults, with age not informed in 43 cases. In children, 33 % of the patients have a positive culture on presentation, with higher incidence in children younger than 10 years. In contrast, only 15 % among adults were positive CSF culture. In total, 287 abdominal pseudocyst cases who underwent shunt revision have been reported; 78.4 % of children and 62.2 % of adults. The main occurrence of this complication according to the etiology of hydrocephalus in children was different from adults. The recurrence of pseudocyst occurred in 19.8 and 24.2 % of children and adults, respectively. The differences between children and adults might represent distinct trends on the etiology and treatment of this entity. Hence, additional well-designed cohort studies will be necessary to strengthen our findings.

Outcome of surgery in children with focal cortical dysplasia younger than 5 years explored by stereo-electroencephalography

Abstract: Focal cortical dysplasia (FCD) is the most frequent etiology for drug-resistant epilepsy in young children. Complete removal of the lesion is mandatory to cure the epilepsy. Stereo-EEG (SEEG) is an excellent method to delimitate the zone to be resected in older children and adults. We studied its feasibility in younger children. We retrospectively studied 19 children under 5 years of age who underwent SEEG between January 2009 and December 2012 and were subsequently operated on. FCD was diagnosed in all. We reviewed magnetic resonance imaging (MRI), electrophysiological and clinical data, as well as postoperative seizure outcome. We also included fluoro-deoxyglucose positron emission tomography (FDG-PET) studies, which had been systematically performed before invasive recording in 16 of the 19 children. The mean patient’s age at the time of SEEG was 38.6 months, and the mean age at seizure onset was 8 months. Three patients had normal MRI. No SEEG-associated complications occurred. We were able to delineate the epileptogenic zone in all children, and electrode stimulation localized the motor area when necessary (12 patients). Hypometabolic areas on FDG-PET included the epileptogenic zone in 13 of the 16 children, with a lobar concordance in 9 (56 %) and the same anatomical extent in 6 (38 %). Twelve children subsequently underwent focal or sublobar resection, six had multilobar resection, and one had hemispherotomy. The etiology was FCD type 2 in 15 and FCD type 1 or type 3 in three children. Eighty-four percent of our population have remained seizure-free at a mean follow-up of 29 months (12–48 months). Although children with FCD can successfully undergo resective surgery without invasive EEG, poor seizure semiology at this age inclines to perform SEEG when the dysplastic lesion is ill-defined and/or the electroclinical correlation is unclear. In cases with normal imaging as well as with suspected huge malformations, as was the case in 52 % of our patients, we consider it to be indispensable.

Treatment developments and the unfolding of the quality of life discussion in childhood medulloblastoma: a review

Abstract: To describe how the quality of life (QOL) discussion in childhood medulloblastoma (MB) relates to treatment developments, survival and sequelae from 1920 to 2014. Articles containing “childhood medulloblastoma” and “quality of life” were identified in PubMed. Those containing phrases pertaining to psychological, emotional, behavioral or social adjustment in the title, abstract or keywords were selected. Inclusion of relevant older publications was assured by cross-checking references. 1920–1930s: suction, electro-surgery, kilovolt (KV) irradiation. Survival = months. Focus on operative mortality, symptoms and survival. 1940s: radiotherapy improved. 1950s: chemotherapy and intubation. Survival = years. Opinions oscillated between optimism/awareness of physical sequelae of radiotherapy. 1960s: magnified vision, ventriculo-peritoneal (VP) shunts, megavolt (MV) irradiation. Long-term survival shifted the attention towards neurological problems, disability and carcinogenesis of radiotherapy. 1970s: CT, microscope, bipolar coagulation, shunt filters, neuroanesthesia, chemotherapy trials and staging studies. Operative mortality decreased and many patients (re)entered school; emphasis on neuropsychological sequelae, IQ and academic performance. 1980s: magnetic resonance imaging (MRI), Cavitron ultrasonic aspiration (CUSA), laser surgery, hyper-fractionated radiotherapy (HFRT). Cerebellar mutism, psychological and social issues. 1990s: pediatric neurosurgery, proton beams, stem cell rescue. Reflections on QOL as such. 21st century: molecular genetics. Premature aging, patterns of decline, risk- and resilience factors. QOL is a critical outcome measure. Focus depends on survival and sequelae, determined after years of follow-up. Detailed measurements are limited by time, money and human resources, and self-reporting questionnaires represent a crude measure limited by subjectivity. Therapeutic improvements raise the question of QOL versus cure. QOL is a potential primary research endpoint; multicenter international studies are needed, as are web-based tools that work across cultures.

Integrated PET/MRI for planning navigated biopsies in pediatric brain tumors

Abstract: Introduction An integrated PET/MRI scanner has been used in selected cases of pediatric brain tumor patients to obtain additional metabolic information about lesions for preoperative biopsy planning and navigation.

Rapamycin has a beneficial effect on controlling epilepsy in children with tuberous sclerosis complex: results of 7 children from a cohort of 86

Abstract: Purpose Tuberous sclerosis complex (TSC) is a genetic disorder characterized by the formation of hamartomas in various organ systems. We would like share our experience from 86 patients and the results of rapamycin treatment in seven children with TSC. Methods Eighty-six children with TSC were enrolled into this retrospective study. The clinical features of seven children treated with oral rapamycin were presented in detail. Results The most common complaint of administration was convulsion in 77 children (89.5 %). Hypopigmented skin lesions, adenoma sebaceum, resistant epilepsy, intracardiac mass, renal angiomyolipomas, and West syndrome were detected (n = 83, 96.5 %; n = 47, 54.7 %; n = 36, 41.9 %; n = 27, 31.4 %; n = 18, 20.9 %; and n = 13, 15.1 %, respectively). Subependymal nodules were the most frequent finding in cranial imaging followed by cortical tubers and subependymal giant cell astrocytomas (n = 75, 87.2 %; n = 71, 82.6 %; and n =8 , 9.3 %, respectively). Of the seven patients treated with rapamycin, the lesions of six children with facial adenoma sebaceum showed regression in various degrees. The frequency of convulsions decreased in five patients with resistant epilepsy within the first 6 months of the treatment, and complete control of convulsion for all patients was achieved in the second 6 months. Conclusion This is the first study that showed that rapamycin is an effective agent for controlling epilepsy without any significant sideeffectinchildrenwithTSC. Rapamycin seems to be effective after 6 months of therapy, and we recommend tapering the dosage after successful management of epilepsy.

Congenital subependymal giant cell astrocytomas in patients with tuberous sclerosis complex

Abstract: Purpose Subependymal giant cell astrocytoma (SEGA) is a brain tumor associated with tuberous sclerosis complex (TSC). It usually grows in a second decade of life, but may develop in the first months of life. The aim of this work was to establish the incidence, clinical features, and outcome of congenital SEGA in TSC patients.

Prenatal diagnosis and postnatal outcome of fetal intracranial hemorrhage

Abstract: The aim of this study was to present our experience with six cases of fetal intracranial hemorrhage (ICH) in terms of prenatal diagnostic features, and postnatal outcome. The database of prenatal diagnosis unit was searched for antenatally diagnosed ICH cases. Maternal characteristics, ultrasound (US), and magnetic resonance imaging (MRI) findings, clinical course, and postnatal outcome were noted. We evaluated six consecutive cases of fetal ICH. One case was terminated at 24 weeks, and remaining five cases were delivered between 34 and 38 weeks. Five cases (5/6) had intraventricular, and one (1/6) had intraparenchymal hemorrhage. Hemorrhages were right sided in five cases (5/6), left sided in one case (1/6). Dilated and echogenic ventricular wall were the common US findings. No predisposing factor was detected in four of the cases, and intrauterine growth restriction was an underlying factor in two fetuses. Intrauterine progression of the hydrocephaly, and parenchymal thinning was seen in four cases (4/6). In three of four cases (3/4) with progressive grade 3–4 hemorrhage and hydrocephaly, postnatal outcome were dismal, and one case had mild neurological impairment at three months. In one case which had non-progressive mild ventriculomegaly, the lesion regressed after 4 weeks, and had normal short-term outcome Fetal ICH can be accurately identified and categorized by antenatal sonography, and fetal MRI. Although intrauterine regression or normal short-term postnatal outcome is possible, the outcome is usually poor for fetuses with high grade and/or progressive lesions. Therefore, further studies assessing long-term postnatal outcome are needed

Presentation, pathology, and treatment outcome of brain tumors in 172 consecutive children at CURE Children’s Hospital of Uganda. The predominance of the visible diagnosis and the uncertainties of epidemiology in sub-Saharan Africa

Abstract: This study reviews the first operative series of pediatric brain tumors from Uganda, the largest series from Sub-Saharan Africa, and explores the challenges to progress in pediatric neuro-oncology in the region. This is a retrospective operative series of brain tumors in 172 children at Cure Children’s Hospital of Uganda over 10 years. Demographics, clinical presentation, lesion location, histopathology, operative management, and outcome were investigated. Survival was assessed using Kaplan–Meier method. Log-rank test and p value with Bonferroni correction were used to determine significance of survival differences. There were 103 males (59.9 %) and 69 females (40.1 %; mean age at diagnosis 6.5 years with 29 % < 2 years). The most common histologic types were pilocytic astrocytoma (23.2 %), ependymoma (16.3 %), craniopharyngioma (9.9 %), choroid plexus papilloma (9.3 %), and medulloblastoma (8.1 %). Supratentorial tumors (62.2 %) were more common. Symptomatic hydrocephalus predominated at presentation (66.9 %). In 71 (41.3 %), the presentation was macrocephaly or a visible mass. Estimated 5-year survival was 60 %. The majority of pediatric brain tumors in the region likely go unrecognized. Most that do come to attention have a “visible diagnosis.” Unlike operative series from developed countries, information about the incidence, prevalence, and overall burden of disease for different tumor types cannot be deduced from the various operative series reported from limited resource countries because of the selection bias that is unique to this context. Delayed presentation and poor access to adjuvant therapies were important contributors to the high mortality. The epidemiology of pediatric brain tumors in sub-Saharan Africa is obscure.

Neuroprotective effect of levetiracetam on hypoxic ischemic brain injury in neonatal rats

Abstract: Purpose Hypoxic-ischemic brain injury that occurs in the perinatal period is one of the leading causes of mental retardation, visual and auditory impairment, motor defects, epilepsy, cerebral palsy, and death in neonates. The severity of apoptosis that develops after ischemic hypoxia and reperfusion is an indication of brain injury. Thus, it may be possible to prevent or reduce injury with treatments that can be given before the reperfusion period following hypoxia and ischemia. Levetiracetam is a new-generation antiepileptic drug that has begun to be used in the treatment of epilepsy.

Neurosurgical implications of Pott’s puffy tumor in children and adolescents

Abstract: Pott’s puffy tumor (PPT) is rare and usually seen as a complication of frontal sinusitis resulting in subperiosteal abscess of the frontal bone with underlying osteomyelitis. PPT is potentially severe and can lead to life-threatening intracranial complications. The authors present a series of six pediatric patients diagnosed with PPT and intracranial complications between 1999 and 2012. There were five boys and one girl, and the mean age at diagnosis was 11.8 years. All patients presented with frontal swelling, headache, and fever. Two patients had lowered level of consciousness and severe hemodynamic instability. All patients had epidural abscess, and one also presented subdural empyema. Five patients underwent drainage of all associated abscesses, bone resection, and sinusitis treatment. One was treated conservatively with broad-spectrum antibiotics, and no surgical intervention was required. All patients fully recovered their neurologic status, without further complications.

Case series and descriptive cohort studies in neurosurgery: the confusion and solution

Abstract: Case series (CS) are well-known designs in contemporary use in neurosurgery but are sometimes used in contexts that are incompatible with their true meaning as defined by epidemiologists. This inconsistent, inappropriate and incorrect use, and mislabeling impairs the appropriate indexing and sorting of evidence. Using PubMed, we systematically identified published articles that had “case series” in the “title” in 15 top-ranked neurosurgical journals from January 2008 to December 2012. The abstracts and/or full articles were scanned to identify those with descriptions of the principal method as being “case series” and then classified as “true case series” or “non-case series” by two independent investigators with 100 % inter-rater agreement. Sixty-four articles had the label “case series” in their “titles.” Based on the definition of “case series” and our appraisal of the articles using Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) guidelines, 18 articles (28.13 %) were true case series, while 46 (71.87 %) were mislabeled. Thirty-five articles (54.69 %) mistook retrospective (descriptive) cohorts for CS. CS are descriptive with an outcome-based sampling, while “descriptive cohorts” have an exposure-based sampling of patients, followed over time to assess outcome(s). A comparison group is not a defining feature of a cohort study and distinguishes descriptive from analytic cohorts. A distinction between a case report, case series, and descriptive cohorts is absolutely necessary to enable the appropriate indexing, sorting, and application of evidence. Researchers need better training in methods and terminology, and editors and reviewers should scrutinize more carefully manuscripts claiming to be “case series” studies.

Endoscopic third ventriculostomy with/without choroid plexus cauterization for hydrocephalus due to hemorrhage, infection, Dandy–Walker malformation, and neural tube defect: a meta-analysis

Abstract: Endoscopic third ventriculostomy (ETV) is a viable alternative to CSF shunting in hydrocephalic patients and is used with varying degrees of success dependent on age and etiology. The purpose of this meta-analysis is to analyze data on ETV and ETV/CPC (choroid plexus cauterization) outcomes in hopes of providing a clear understanding of their limitations in patients with hydrocephalus due to hemorrhage, infection, Dandy–Walker malformation, or neural tube disorders. An extensive PubMed search dating back 11 years was performed on primary ETV or ETV/CPC procedures for hydrocephalus due to infection, hemorrhage, neural tube defects, and Dandy–Walker malformation. ETV success was defined as no intraoperative or post-operative complications and no need for revision surgery at follow-up. Ten studies were identified for analysis. The data represent 534 patients undergoing primary ETV and 167 patients undergoing primary ETV/CPC. The ETV group reached a 55 % success rate, while the ETV/CPC group reached a 67 % success rate. Success rates of ETV alone for hydrocephalus due to infection, neural tube defects, and intraventricular hemorrhage reached 54, 55, and 57 %, respectively. 84 % success was found in patients older than 2 years of age and 52 % success in patients less than 2 years of age. ETV is a valid treatment for hydrocephalus of any etiology. There exists a small difference in success rates between infection, hemorrhage, and neural tube disorders, though not enough to discount ETV for these etiologies. Initial data utilizing ETV/CPC are promising, and additional studies will need to be done to verify such results.

Different etiologies of acquired torticollis in childhood

Abstract: Torticollis can be congenital or may be acquired in childhood. Acquired torticollis occurs because of another problem and usually presents in previously normal children. The causes of acquired torticollis include ligamentous, muscular, osseous, ocular, psychiatric, and neurologic disorders. We performed this study to evaluate the underlying causes of torticollis in childhood. Ten children presented with complaints of torticollis between April 2007 and April 2012 were enrolled in this study. The additional findings of physical examination included neck pain, twisted neck, walking disorder, imbalance, and vomiting The identified etiologies of the enrolled children was acute disseminated encephalomyelitis in a 2.5-year-old boy, posterior fossa tumor in a 10-month-old boy, spontaneous spinal epidural hematoma in a 5-year-old hemophiliac boy, cervical osteoblastoma in a 3-year-old boy, arachnoid cyst located at posterior fossa in a 16-month-old boy, aneurysm of the anterior communicating artery in a 6-year-old girl, pontine glioma in a 10-year-old girl, and a psychogenic torticollis in a 7-year-old boy were presented. There is a wide differential diagnosis for a patient with torticollis, not just neurological in etiology which should be considered in any patient with acquired torticollis. Moreover, early diagnosis of etiological disease will reduce mortality and morbidity. Therefore, clinicians managing children with torticollis must be vigilant about underlying neurological complications.

Management of postoperative pseudomeningoceles: an international survey study

Abstract: Pseudomeningoceles are common complications after posterior fossa and intradural spinal surgery and are often asymptomatic. Management guidelines are lacking, and anecdotally, we have encountered radically different suggested treatments varying from observation to immediate surgical intervention. The goal of this study was to determine the prevailing opinions among neurosurgeons on the management of this condition. Neurosurgeons from around the world were invited via an International Society for Pediatric Neurosurgery (ISPN) and Neurosurgery ListServ e-blast to participate in a 33-question survey on the management of pseudomeningoceles, presented as simulated scenarios after posterior fossa and spinal intradural surgery. Two hundred forty-one responses were obtained. Pseudomeningoceles after posterior fossa tumor resection, in the absence of hydrocephalus, were typically managed nonoperatively for 7 to 14 days before re-exploration. Only 0.5 % of the surgeons would offer upfront repair of the pseudomeningocele. In the presence of hydrocephalus, 48 % of the neurosurgeons intervene initially with cerebrospinal fluid (CSF) diversion and would change therapy if the lesion did not resolve in 2 to 4 days. Ninety percent of the surgeons manage spinal pseudomeningoceles nonoperatively for 7–14 days before re-exploration is considered. The most common steps taken to prevent pseudomeningoceles are watertight closure, tissue glues, and duroplasty. The present zeitgeist suggests that, in the absence of hydrocephalus, initial observation is appropriate for cranial and spinal pseudomeningoceles. Operative revision should be reserved for failure of conservative treatment. If hydrocephalus is present, consideration should be made for CSF diversion. This study may serve as a guideline regarding acceptable management.

Cognitive and epilepsy outcomes after epilepsy surgery caused by focal cortical dysplasia in children: early intervention maybe better

Abstract: Focal cortical dysplasia (FCD) is a specific malformation of cortical development harboring intrinsic epileptogenicity, and most of the patients develop drug-resistant epilepsy in early childhood. The detrimental effects of early and frequent seizures on cognitive function in children are significant clinical issues. In this study, we evaluate the effects of early surgical intervention of FCD on epilepsy outcome and cognitive development. From 2006 to 2013, 30 children younger than 18 years old underwent resective surgery for FCDs at Taipei Veterans General Hospital. The mean age at surgery was 10.0 years (range 1.7 to 17.6 years). There were 21 boys and 9 girls. In this retrospective clinical study, seizure outcome, cognitive function, and quality of life were evaluated. To evaluate the effects to outcomes on early interventions, the patients were categorized into four groups according to age of seizure onset, duration of seizure before surgery, and severity of cognitive deficits. Eleven of 22 (50 %) patients demonstrated developmental delay preoperatively. The Engel seizure outcome achievements were class I in 21 (70 %), class II in 2 (7 %), class III in 6 (20 %), and class IV in 1 (3 %) patients. The locations of FCDs resected were in the frontal lobe in 18 cases, temporal lobe in 7, parietal lobe in 2, and in bilobes including frontoparietal lobe in 2 and parieto-occipital lobes in 1. Eight cases that had FCDs involved in the rolandic cortex presented hemiparesis before surgical resection. Motor function in four of them improved after operation. The histopathological types of FCDs were type Ia in 1, type Ib in 7, type IIa in 7, type IIb in 12, and type III in 3 patients. FCDs were completely resected in 20 patients. Eighteen (90 %) of them were seizure free (p < 0.001) with three patients that received more than one surgery to accomplish complete resection. The patients who had early seizure onset, no significant cognitive function deficit, and early surgical intervention with complete resection in less than 2 years of seizure duration showed best outcomes on seizure control, cognitive function, and quality of life. Delay in cognitive development and poor quality of life is common in children treated for FCDs. Early surgical intervention and complete resection of the lesion help for a better seizure control, cognitive function development, and quality of life. FCDs involved eloquent cortex may not prohibit complete resection for better outcomes.

The response and survival of children with recurrent diffuse intrinsic pontine glioma based on phase II study of antineoplastons A10 and AS2-1 in patients with brainstem glioma

Abstract: Background Brainstem gliomas (BSG) are relatively rare tumors of which recurrent pediatric diffuse intrinsic pontine gliomas (RPDIPG) comprise a distinct group. Numerous trials have been conducted on RPDIPG, none of which have resulted in identifying any proven pharmacological treatment benefit. This study included 40 patients diagnosed with different types of BSG, but it was decided to describe first the encouraging results in the most challenging group of RPDIPG.

Indications and selection criteria for invasive monitoring in children with cortical dysplasia.

Abstract: In order to presurgically define the anatomical location of the epileptogenic zone (EZ) and its proximity to possible cortical and subcortical eloquent areas in pediatric patients with medically intractable focal epilepsy, an array of noninvasive tools are available: recorded seizure semiology, scalp electroencephalographic (EEG) recordings (ictal and interictal epileptic patterns), magnetic resonance imaging (MRI), positron emission tomography (PET), ictal single-photon emission computed tomography (SPECT), neuropsychological testing, and/or magnetoencephalography. When the noninvasive tools fail or are insufficient in precisely localizing the EZ and its functional and anatomical interphase with potential eloquent cortical areas, invasive extra-operative monitoring procedures might be needed. In this chapter, we will discuss the main goals of extra-operative invasive evaluation for children with medically intractable epilepsy in whom cortical dysplasia is a possible etiology. We will specifically discuss the possible indications, surgical strategies, results, and morbidity associated with the placement of subdural and stereoelectroencephalography (SEEG) electrodes. The rationale behind the choice of each one of the above techniques will also be discussed.

Clinical features and endovascular treatment of intracranial arteriovenous malformations in pediatric patients

Abstract: The purpose of this study was to characterize clinical features and evaluate the clinical outcome of endovascular embolization treatment intracranial arteriovenous malformations in pediatric patients. A cohort of children (age ≤ 18 years) with arteriovenous malformations (AVMs) from 2000 to 2012 was included. Predictors studied included patient gender, age, and angioarchitectural features, including AVM location, nidus morphology and size, venous drainage, and associated aneurysms. Treatment method, complications and outcomes were recorded. The features of AVMs were evaluated before the treatment. One hundred twenty-seven children (77 males, mean age 13.2 years) were included; 90/127 (70.9 %) children were presented with hemorrhage. AVM size and deep venous drainage were independently associated with hemorrhage; 66/127 patients (52 %) treated with endovascular embolization. Complete obliteration at the end of all endovascular procedures was achieved in 14/66 patients (21.2 %), with an average of 78 % (range, 20–100 %) volume reduction. A mean of 2.9 (range, 1–9) feeding pedicles was embolized per patient. Overall, nine complications occurred in a total of 123 procedures (7.3 %). There was no procedure-related death in this study population. There was no significant difference between patients with and without complications in terms of AVM grade, demographic characteristics, or embolization features. AVM size and deep venous drainage were independently associated with hemorrhage in pediatric patients. Endovascular procedure is feasible and safe for pediatric AVMs, and complete embolization can be achieved in small AVMs, while large AVMs can be adequately reduced in size for additional microsurgery or stereotactic radiosurgery.

Clinical outcome of pediatric choroid plexus tumors: retrospective analysis from a single institute

Abstract: Choroid plexus tumor is a rare brain tumor with variable clinical features according to the histological grade. We reviewed the treatment outcome of 23 children, focusing on the biological behavior of the atypical choroid plexus papilloma (ACPP) and the current therapeutic strategy in choroid plexus carcinoma (CPC). The demographics, clinical features, surgical treatments, adjuvant therapies, and survival were reviewed. The median age at diagnosis was 18 months—55 months for choroid plexus papilloma (CPP), 8 months for ACPP, and 15 months for CPC. Gross total resections were achieved in seven of eight patients with CPP, seven of seven with ACPP, and three of eight with CPC. Seven patients with CPC received chemotherapy. Four patients received high-dose chemotherapy (HDCT) and autologous peripheral blood stem cell transplantation (aPBSCT), and three among them have survived. Four patients with CPC received radiotherapy. One CPP patient and one CPC patient underwent radiosurgery. All CPP and ACPP patients have survived. The overall survival rate of the CPC patients was 62.5 % in the first year and 42.9 % in the second year. The progression-free survival rate of the CPC patients was 50 % in the first year and 0 % in the second year. Seven patients underwent permanent cerebrospinal fluid diversion surgery because of hydrocephalus or subdural effusion. CPP and ACPP were surgically curable. Multi-modal treatments are necessary in the management of CPC with poor prognosis. HDCT and aPBSCT may be important to treat infants for whom radiotherapy is limited. Hydrocephalus and subdural effusion should be resolved with appropriate management.

Transorbital penetrating cerebral injury caused by a wooden stick: surgical nuances for removal of a foreign body lodged in cavernous sinus

Abstract: Penetrating head injury (PHI) is rare in civilian population and is mostly caused by low-velocity modes. A transorbital penetrating intracranial injury is very rare and more severe than traumatic brain injury. We report a rare case of transorbital penetrating cranial injury caused by a wooden stick. The surgical strategy was planned as the wooden stick was lodged in the right cavernous sinus. The wooden stick was successfully removed. Patient made an uneventful recovery. Transorbital penetrating injuries are uncommon form of injury and require a multidisciplinary approach. No attempt should be made to remove the foreign body without the backup of an operating room because of the possibility that the object may be tamponading an injured vessel. A careful planning and a strict adherence to basic perioperative principles can lead to a satisfactory outcome.

Percutaneous endoscopic interlaminar discectomy for pediatric lumbar disc herniation

Abstract: Percutaneous endoscopic interlaminar discectomy (PEID) is a widely used minimally invasive procedure which shows satisfying outcomes in the adult population. However, pediatric lumbar disc herniations (PLDH) occur in growing spines and are less related to degeneration, which makes them different from the adult disc herniations. This study evaluates the clinical outcomes of PEID in treating PLDH. A prospect study was done in the period from June 2010 to December 2012, which included 29 consecutive pediatric patients with a mean age of 16.4 years (range, 13 to 18 years) who underwent PEID for single level lumbar disc herniation. The following measuring tools were used: visual analog scale (VAS) for back and leg pain, Oswestry Disability Index (ODI), and Macnab criteria. There were no severe complications such as dural tear or nerve root damage found in our study. The mean follow-up period was 19.7 months. The VAS score for leg and back pain decreased dramatically at 1 day postoperatively and kept decreasing until the follow-up visit at 3 months postoperatively, when it became stable at a low level. ODI kept improving until the follow-up visit at 6 months postoperatively when it reached a stable low level. Of the patients, 91 % reported no longer having leg pain and 9 % had occasional leg pain at last follow-up. PEID shows a satisfying outcome with a minimal rate of complications. It has the advantages of minimal traumatization and scar formation and is a safe and effective treatment for PLDH.

Feasibility of telemetric ICP-guided valve adjustments for complex shunt therapy

Abstract: Object The advances in shunt valve technology towards modern adjustable differential pressure (DP) valves and adjustable gravitational assisted valves result in an increasing complexity of therapeutical options. Modern telemetric intracranial pressure (ICP) sensors may be helpful in their application for diagnostic purposes in shunt therapy. We present our first experiences on telemetric ICP-guided valve adjustments in cases with the combination of an adjustable DP valve and adjustable gravitational unit.