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This review looks at the advances in the surgical technique, selective dorsal rhizotomy, used for the management of spasticity in children.

Highlights • White matter stiffness is significantly reduced in stable, shunted hydrocephalus patients vs controls. • In patients, age, sex and ventricular volume have significant associations with brain stiffness. • Lower brain stiffness, but not ventricular volume, predicts worse quality of life and depression., Introduction Hydrocephalus that develops early in life is often accompanied by developmental delays, headaches and other neurological deficits, which may be associated with changes in brain shear stiffness. However, noninvasive approaches to measuring stiffness are limited. Magnetic Resonance Elastography (MRE) of the brain is a relatively new noninvasive imaging method that provides quantitative measures of brain tissue stiffness. Herein, we aimed to use MRE to assess brain stiffness in hydrocephalus patients compared to healthy controls, and to assess its associations with ventricular size, as well as demographic, shunt-related and clinical outcome measures. Methods MRE was collected at two imaging sites in 39 hydrocephalus patients and 33 healthy controls, along with demographic, shunt-related, and clinical outcome measures including headache and quality of life indices. Brain stiffness was quantified for whole brain, global white matter (WM), and lobar WM stiffness. Group differences in brain stiffness between patients and controls were compared using two-sample t-tests and multivariable linear regression to adjust for age, sex, and ventricular volume. Among patients, multivariable linear or logistic regression was used to assess which factors (age, sex, ventricular volume, age at first shunt, number of shunt revisions) were associated with brain stiffness and whether brain stiffness predicts clinical outcomes (quality of life, headache and depression). Results Brain stiffness was significantly reduced in patients compared to controls, both unadjusted (p ≤ 0.002) and adjusted (p ≤ 0.03) for covariates. Among hydrocephalic patients, lower stiffness was associated with older age in temporal and parietal WM and whole brain (WB) (beta (SE): −7.6 (2.5), p = 0.004; −9.5 (2.2), p = 0.0002; −3.7 (1.8), p = 0.046), being female in global and frontal WM and WB (beta (SE): −75.6 (25.5), p = 0.01; −66.0 (32.4), p = 0.05; −73.2 (25.3), p = 0.01), larger ventricular volume in global, and occipital WM (beta (SE): −11.5 (3.4), p = 0.002; −18.9 (5.4), p = 0.0014). Lower brain stiffness also predicted worse quality of life and a higher likelihood of depression, controlling for all other factors. Conclusions Brain stiffness is reduced in hydrocephalus patients compared to healthy controls, and is associated with clinically-relevant functional outcome measures. MRE may emerge as a clinically-relevant biomarker to assess the neuropathological effects of hydrocephalus and shunting, and may be useful in evaluating the effects of therapeutic alternatives, or as a supplement, of shunting.

Purpose To create a set of consensus-based and evidence-informed recommendations to provide guidance around the safe dispensing and handling of oral anti-cancer drugs in low-volume settings unique to the community pharmacy setting. Methods A review of published and grey literature (published in non-commercial domains such as national organizations and associations) documents and nine key informant interviews were conducted and a modified Delphi approach was taken to achieve consensus. The final list of 47 candidate recommendations was reviewed by a task force and validated by multi-disciplinary stakeholders. A draft of the statements was circulated broadly within the community pharmacy community in an effort to assess relevance and implementation feasibility. Results The final report included 44 recommendations that addressed 11 key areas germane to the safe handling of oral anti-cancer drugs in community pharmacies. Mean agreement increased from 70% to 95%. Early feedback from community pharmacy leaders during the external review suggests that many of the proposed recommendations can be feasibly implemented within a reasonable timeframe when released with appropriate education and resource materials. Conclusions A modified-Delphi approach supplemented by key informant interviews and a comprehensive external review resulted in a set of evidence-informed, community-driven recommendations for community pharmacies. The recommendations address a gap in existing literature to improve understanding of the risks associated with handling and dispensing oral anti-cancer drugs for both community pharmacy staff and management and offer mitigating strategies to reduce those risks. Incorporating feasibility assessment actions early (through the key informant interviews) and late (through the external review) ensures recommendations are grounded in practicality and support broad and early knowledge translation strategies.

Background There has been a dramatic increase in new drug approvals in oncology, consisting of both small molecule inhibitors and monoclonal antibodies. However, Health Canada approval for many of the new agents was based on single randomized trials consisting of only a few hundred patients. As more patients get treated with these newer agents, there is the potential for new and discrete toxicities. Pharmacists are in an ideal position to identify, monitor, manage, and even preempt future events, given their close proximity to the patient. However, the extent of pharmacists’ involvement in formal patient programs is unknown. To address this knowledge gap, a survey of oncology pharmacists practicing in Atlantic Canada was conducted. Methods A structured mailing strategy was adopted as recommended by Dillman (1978). Standardized data collection forms were electronically mailed to 60 oncology pharmacists. Survey items consisted of respondent demographic information, practice setting, the existence of a formal patient monitoring program managed, and if patients are contacted by telephone following the completion of their anticancer cycle. Results Overall, 31 completed surveys were received, for an overall response rate of 50%. Respondents had a median age of 42 and a median of 18 years’ (range 1 to 25) professional experience as a pharmacist. Only 18 of the 31 (58%) respondents indicated that there was a formal monitoring and call back program managed by pharmacy available at their institution. For those without such programs, the main reasons were due to staffing issues and lack of adequately trained clinical personnel. Overall, 100% of respondents would favor the development of a formal monitoring program in hospitals with a high volume of anticancer drug prescribing. Conclusions Even though the number of new anticancer drugs being introduced into clinical pharmacy practice is increasing, formal patient monitoring and patient call back programs are not universal in Atlantic Canada hospitals.

Brain stem mapping is a neurophysiological method of locating cranial nerve motor nuclei (CMN) on the floor of the fourth ventricle. The motor nuclei of the cranial nerves are usually located in the vicinity of specific anatomical landmarks on the floor of the fourth ventricle. Because a tumor can cause distorting effects on the local anatomy, these landmarks are not evident in most patients. Even in patients without a tumor, specific anatomical landmarks are often not visible. Different points of the surgically exposed floor of the fourth ventricle were electrically stimulated by the surgeon using a handheld probe. Electromyographic responses were recorded with electrodes inserted in the face muscles innervated by cranial motor nerves. This technique was found to be useful for locating CMN before tumor resection and enabled the surgeon to avoid damaging the nuclei when entering the brain stem. Furthermore, intraoperative neurophysiological localization of CMN showed specific patterns of displacement by brain stem tumors. Pontine tumors displaced CMN of nerve VII around the edge of the tumor, and medullary tumors ventrally displaced low CMN. Understanding the patterns of CMN displacement can help in establishing a surgical plan that minimizes the risk of damaging CMN and allows for safer surgery for brain stem tumors.

Objectives A recent study of community pharmacists in Canada reported that they required additional education. We conducted a survey of community pharmacists to evaluate the adequacy of education and training in oral anticancer agents in Japan. Methods Between May and June 2014, community pharmacists were asked to complete a questionnaire by using two different survey strategies, one online and one via postal mail. Results Three hundred community pharmacists responded to an online survey and 283 community pharmacists responded to a mailed survey. Only 6-10% of respondents felt that they had received adequate education in oncology or oral chemotherapy. Although 81% of Japanese pharmacists had attended at least one continuing education event related to oncology in the past 2 years, only 54% felt comfortable dispensing oral anticancer agents and only 40% felt comfortable educating patients about oral chemotherapy. In a multivariate analysis, confidence in educating patients about oral chemotherapy was associated with an understanding of chemotherapy cycles and doses (odds ratio = 4.89, 95% confidence interval [2.53-9.45]) and the number of continuing education events they had attended (odds ratio = 1.67, 95% confidence interval [1.35-2.08]). Conclusions This is the first report to evaluate whether community pharmacists are equipped to ensure the safe use of oral anticancer agents in Japan. The results are similar to those previously reported for Canadian pharmacists, namely a low rate of positive responses for education in oncology and oral chemotherapy, demonstrating a similar need for additional education and training in oral chemotherapy.

Objective: To describe clinicopathological correlation of congenital intracranial immature teratoma. Methods: A retrospective case analysis from a tertiary medical center. Results: We report a case of an intracranial immature teratoma detected prenatally at 35 weeks of gestation. The tumor showed rapid growth, causing acute hydrocephalus requiring subsequent ventriculoperitoneal shunting. Resective surgery was performed within 2 weeks after birth. The infant died at day of life 29. Histological examination revealed an immature teratoma, with high MIB1/Ki-67 proliferation index. Conclusion/Implications: Intracranial immature teratoma with high MIB1/Ki-67 proliferation index may serve as an independent poor prognostic factor.

The main objective of this study was to determine if family history of malignant peripheral nerve sheath tumor (MPNST) increases risk of developing an MPNST in patients with neurofibromatosis-1 (NF-1).Individuals with NF-1 registered with the Children's Tumor Foundation's Neurofibromatosis Registry were emailed an anonymous 15-minute survey with regard to personal and family history of NF-1, MPNST, ages of onset, and symptomatology. Participation was voluntary and information was self-reported.The survey was sent to 4801 registrants, 878 responded. Presence of a family history of MPNST was found to be a risk factor for the development of MPNST; 19.4% of respondents confirming a family history of MPNST developed MPNST compared with 7.5% of respondents with no family history (odds ratio, 2.975; 95% confidence interval, 1.232-7.187; P=0.021). NF-1 patients with a positive family history developed MPNST at a younger age than those with no family history (8.3% vs. 0.5% P=0.003 and 13.9% vs. 2.4% P=0.003, for onset before 10 and 20, respectively). In the MPNST population with a known family history, onset prior to age 10 was significantly more prevalent (42.9% vs. 7% P=0.029).These results suggest a positive family history of MPNST represents a risk factor for the development and early onset of MPNST in individuals with NF-1.

Endoscopic third ventriculostomy (ETV) is a common alternative method for the management of hydrocephalus in selected patients. A retrospective chart review was conducted to review our experience with ETV for patients with a variety of causes for hydrocephalus. A total of 67 children with median age of 10.8 years (range= 8 weeks to 21 years) underwent 76 ETVs from July 1992 to June 2003. Two procedures were abandoned due to anatomical distortion. The medical records, operative reports, and imaging studies, were retrospectively reviewed with regard to outcome, complications and patency rate. Treatment failure was defined as the need to shunt the patient within 4 weeks of performing the ETV. The overall success rate was 83% with a mean follow-up 40.8 months. Nine patients underwent 11 repeat ETVs at an average interval period of 24 months with a patency rate of 90% following the second procedure. Ten of eleven patients who underwent re-ETV were ultimately shunt-independent. The highest success rates were achieved for obstructive hydrocephalus from midbrain/tectal tumor (100%, n = 4), pineal tumor (100%, n = 3), intraventricular tumor or cyst (100%, n = 2), and post-infectious hydrocephalus (100%, n = 1). Lower patency rates were noted in patients with Chiari malformation (0%, n = 1) and posterior fossa tumors (63%, n = 8). Due to the efficacy of ETV, it should be considered as the primary procedure, rather than ventricular peritoneal shunts, in carefully selected children. The reliability rate is dependent upon the age and etiology of hydrocephalus.

Sagittal synostosis affects 1 in 1000 live births and may result in increased intracranial pressure, hindrance of normal neural development, and cosmetic deformity due to scaphocephaly. Historically, several approaches have been utilized for surgical correction and recently, computed tomography (CT)-guided reconstruction procedures are increasingly used. In this report, the authors describe the use of a CT-derived virtual and stereolithographic (3D printed) craniofacial models, which are used to guide intraoperative bone placement, and intraoperative CT guidance for confirmation of bone placement, to ensure the accuracy of surgical correction of scaphocephaly, as demonstrated to parents. Preoperative high-resolution CT imaging was used to construct 3D image sets of the skulls of two infants (a 14-month-old female and a 6-month-old male) with scaphocephaly. These 3D image sets were then used to create a virtual model of the proposed surgical correction for each of the infants’ deformities, which was then printed and made available for use intraoperatively to plan the bone flap, fashion the bone cuts, and optimize graft placement. After the remodeling, adherence to the preoperative plan was assessed by overlaying a CT scan of the remodeled skull with the virtual model. Deviations from the preoperative model were noted. Both patients had excellent postoperative cosmetic correction of head shape and contouring. The mean operative time was 5 h, blood loss was 100 ml, and one child required modification of the subocciput after intraoperative imaging showed a deviation of the reconstruction from the surgical goal as depicted by the preoperative model. The addition of neuro-navigation to stereolithographic modeling ensured the accuracy of the reconstruction for our patients and provided greater confidence to both surgeons and parents. While unisutural cases are presented for clarity, correction was still required for one patient. The cost of the models and the additional CT required must be weighed against the complexity of the procedure and possibly reserved for patients with potentially complicated corrections.

While autologous split calvaria remains the preferred material for use in pediatric cranioplasty, it may be difficult to split the bone neatly into two distinct pieces, especially in infants and young children. In this paper, the authors present a technique in which numerous split pieces of bone can be readily joined together and conformed to the shape of the specific defect using a customized template and 3D trellis-like basket.

Purpose Oral anticancer agents offer significant benefits over parenteral anticancer therapy in terms of patient convenience and reduced intrusiveness. Oral anticancer agents give many cancer patients freedom from numerous hospital visits, allowing them to obtain their medications from their local community pharmacy. However, a major concern with increased use of oral anticancer agents is shift of responsibility in ensuring the proper use of anticancer agents from the hospital/clinical oncology team to the patient/caregiver and other healthcare providers such as the community pharmacists who may not be appropriately trained for this. This study assessed the readiness of community pharmacists across Canada to play this increased role with respect to oral anticancer agents. Methods Using a structured electronic mailing strategy, a standardized survey was mailed to practicing pharmacists in five provinces where community pharmacists were dispensing the majority of oral anticancer agents. In addition to collecting basic demographic and their practice setting, the survey assessed the pharmacists’ knowledge regarding cancer therapy and oral anticancer agents in particular, their education needs and access to resources on oral anticancer agents, the quality of prescriptions for oral anticancer agents received by them in terms of the required elements, their role in patient education, and steps to enhance patient and personal safety. Results There were 352 responses to the survey. Only 13.6% of respondents felt that they had received adequate oncology education at the undergraduate level and approximately 19% had attended a continuing education event related to oncology in the past 2 years. Only 24% of the pharmacists who responded were familiar with the common doses of oral anticancer agents and only 9% felt comfortable educating patients on these medications. Conclusions A substantial portion of community pharmacists in Canada lack a solid understanding of oral anticancer agents and thus are poorly equipped to play a major role in ensuring their appropriate use. More education and training on oral anticancer agents are urgently required.

The primary goal of seamless care is improved patient outcomes and improved standards of care for patients with cancer. The pharmacy service of the Newfoundland Cancer Treatment and Research Foundation conducted a randomized control study that measured clinical and humanistic outcomes of a pharmacist-directed seamless care program in an ambulatory oncology clinic. This article focuses on the intervention group, particularly the identification of drug-related problems (DRPs) and utilization of health care services as well the satisfaction of 3 types of health professionals with the services provided by the pharmacist-directed seamless care program. Overall, the seamless care pharmacist (SCP) identified an average of 3.7 DRPs per intervention patient; the most common DRP reported was a patient not receiving or taking a drug therapy for which there is an indication. The SCP identified more DRPs in patients receiving adjuvant treatment compared to those receiving palliative treatment. On average, family physicians, oncology nurses, and hospital pharmacists were satisfied with the SCP intervention indicating that they agreed the information collected and distributed by the SCP was useful to them. Pharmacist-directed seamless care services in an ambulatory oncology clinic have a significant impact on clinical outcomes and processes of patient care. The presence of a SCP can help identify and resolve DRPs experienced by patients in an outpatient oncology clinic, ensuring that patients are receiving the highest standard of care.

At some point in their lives, patients previously shunted for hydrocephalus may experience chronic, debilitating headaches, despite the fact that their shunts are functioning properly. Previously published reports have suggested that a subset of these patients may be suffering from an iatrogenic craniocerebral disproportion (CCD) and, therefore, may benefit from procedures that expand the available intracranial space. A unified definition of this disorder, however, is lacking. Here, the authors chart the history (including historical terminology) of CCD, review its incidence, describe its signs, symptoms, and associated radiologic findings, and expound upon its pathophysiology. Next, a concise clinical definition of CCD based on the temporal correlation of headaches with the appearance of plateau waves on intracranial pressure (ICP) monitoring is proposed. The authors conclude with a discussion of the various therapeutic strategies employed previously to treat this disorder and present their individualized treatment strategy based upon the simultaneous utilization of ICP monitors and gradual external cranial vault expansion.

A subset of hydrocephalic patients in whom shunts are placed at an early age will develop craniocerebral disproportion (CCD), an iatrogenic mismatch between the fixed intracranial volume and the growing brain. The lack of a reliable, reproducible method to diagnose this condition, however, has hampered attempts to treat it appropriately. For those practitioners who acknowledge the need to create more intracranial space in these patients, the lack of agreed-upon therapeutic end points for cranial vault expansion has limited the use of such techniques and has sometimes led to problems of underexpansion. Here, the authors present a definition of CCD based primarily on the temporal correlation of plateau waves on intracranial pressure (ICP) monitoring and headache exacerbation. The authors describe a technique of exploiting continued ICP monitoring during progressive cranial expansion in which the goal of distraction is the cessation of plateau waves. Previously encountered problems of underexpansion may be mitigated through the simultaneous use of ICP monitors and gradual cranial expansion over time.

OBJECTIVEThe object of this study was to use diffusion tensor imaging (DTI) and tract-based spatial statistics (TBSS) to characterize the long-term effects of hydrocephalus and shunting on white matter integrity and to investigate the relationship of ventricular size and alterations in white matter integrity with headache and quality-of-life outcome measures.METHODSPatients with shunt-treated hydrocephalus and age- and sex-matched healthy controls were recruited into the study and underwent anatomical and DTI imaging on a 3-T MRI scanner. All patients were clinically stable, had undergone CSF shunt placement before 2 years of age, and had a documented history of complaints of headaches. Outcome was scored based on the Headache Disability Inventory and the Hydrocephalus Outcome Questionnaire. Fractional anisotropy (FA) and other DTI-based measures (axial, radial, and mean diffusivity; AD, RD, and MD, respectively) were extracted in the corpus callosum and internal capsule with manual region-of-interest delineation and in other regions with TBSS. Paired t-tests, corrected with a 5% false discovery rate, were used to identify regions with significant differences between patients and controls. Within the patient group, linear regression models were used to investigate the relationship between FA or ventricular volume and outcome, as well as the effect of shunt-related covariates.RESULTSTwenty-one hydrocephalus patients and 21 matched controls completed the study, and their data were used in the final analysis. The authors found significantly lower FA for patients than for controls in 20 of the 48 regions, mostly posterior white matter structures, in periventricular as well as more distal tracts. Of these 20 regions, 17 demonstrated increased RD, while only 5 showed increased MD and 3 showed decreased AD. No areas of increased FA were observed. Higher FA in specific periventricular white matter tracts, tending toward FA in controls, was associated with increased ventricular size, as well as improved clinical outcome.CONCLUSIONSThe study shows that TBSS-based DTI is a sensitive technique for elucidating changes in white matter structures due to hydrocephalus and chronic CSF shunting and provides preliminary evidence that DTI may be a valuable tool for tailoring shunt procedures to monitor ventricular size following shunting and achieve optimal outcome, as well as for guiding the development of alternate therapies for hydrocephalus.

An 11-year-old girl with cerebral palsy, known to you for the past 5 years, presents with 6 months of worsening pain and rigidity of her upper extremities. Her parents find it nearly impossible to dress her in the morning without pain medication and state that although her dose of oral baclofen was increased almost 9 months ago, they have not noticed any improvement. They are intrigued by the prospect of surgical therapies that could relieve their daughter of daily challenges.

On December 5, 1960, 4-month-old Theo Dahl, the only son of best-selling author Roald Dahl (1916–1990), had his skull shattered in a horrific traffic accident. What began as a personal tragedy for the Dahl family would soon evolve into a dogged crusade by Dahl to expand upon preexisting valve technology with the goal of developing a shunt that would not become obstructed. Based upon exclusive access to private archives of the Dahl estate, as well as interviews with those involved, this article tells the intricate tale of one famous father's drive to significantly alter the natural history of pediatric hydrocephalus. Dahl's collaboration with British toymaker Stanley Wade and pioneering pediatric neurosurgeons Joseph Ransohoff, Kenneth Shulman, and Kenneth Till to create the Wade-Dahl-Till (WDT) valve is examined in detail. The ensuing rift between the American and British contingents, the valve's multiple design revisions, and the goal of creating an affordable shunt for children in developing countries are among the issues addressed. The development of the WDT valve marked a significant turning point in the surgical management of pediatric hydrocephalus in general and in shunt valve technology in particular.

Background: Over the past decade, there has been a sharp rise in the approval of orally administered anti-cancer agents for disease control. The increase in the use of oral anti-cancer agents (OAAs) raises concerns that community pharmacists may not have the training to safely dispense these agents and provide effective patient care. In order to identify the needs of community pharmacists with respect to oral anti-cancer therapy, a survey was conducted in the province of Newfoundland and Labrador. Methods: A structured electronic mailing strategy was used. Standardized data collection forms with a cover letter were electronically mailed to 560 practising pharmacists. Survey items included questions related to demographic information, practice setting, current knowledge related to cancer therapy, education needs, access to resources, patient education, patient and pharmacist safety and required elements of an OAA prescription. Results: The response rate was 39%. Only 9.6% of respondents felt that they had received adequate oncology education at the undergraduate level and approximately 31% had attended a continuing education event related to oncology in the past 2 years. Just 17% of respondents stated that they used protective equipment when dispensing OAAs. Only 28% of the pharmacists who responded were familiar with the common doses of OAAs and approximately 25% felt comfortable educating patients on these medications. Conclusions: A substantial portion of community pharmacists in Newfoundland and Labrador do not have a solid understanding of oral anti-cancer therapy. These educational gaps must be addressed to ensure patient safety as well as the safe handling and dispensing of OAAs by community pharmacists.

The use of intraoperative physiological monitoring has become increasingly common over the last decade and it is a useful tool to be employed for the resection of ependymomas of the central nervous system. This manuscript reviews the history of its development and its methodology with a particular emphasis on those aspects of particular importance during surgery on ependymomas.

We, as pediatric neurosurgeons, are increasingly involved in the management of childhood hypertonia or elevation in muscle tone. As with any disease that we are called upon to treat, it is important that we understand the etiology of the hypertonia, its different manifestations and that we employ accepted tools to quantify the impact of our treatments on our pediatric patients. This special edition of Child’s Nervous System is devoted to the various treatment options that can be employed in the management of childhood hypertonia. You will find discussions by some of the world’s leading experts in these treatments and in managing such children.

The traditional treatment for all forms of hydrocephalus has been the implantation of ventricular shunt systems; however, these systems have inherent tendencies toward complications such as malfunction and infection. A significant advance in the treatment of hydrocephalus has been the evolution of endoscopy. The recent technological advances in this field have led to a renewed interest in endoscopic third ventriculostomy as the treatment of choice for obstructive hydrocephalus. Although several different endoscopes are available, the authors favor a rigid one to perform a blunt fenestration of the third ventricle floor. This description of the technique stresses the nuances for successful completion of this procedure.

Object Endoscopic third ventriculostomy (ETV) has become a common alternative for managing hydrocephalus in select patients. Nevertheless, there is still controversy regarding the indications for ETV as the primary procedure, given its variable success rates. The purpose of this study is to review the authors' experience with ETV for a variety of patients. Methods A total of 43 children underwent ETV between July 1992 and June 2003. Their medical records, operative reports, and imaging studies, when available, were retrospectively reviewed with regard to outcome, complications, and patency rate. Treatment failure was defined as the need to place a shunt within 4 weeks of performing ETV in the patient. There were 20 male and 23 female patients with a mean age of 9.6 years (range 8 weeks–21 years). The overall success rate was 69.8%, and the mean follow-up duration was 24.6 months. Six patients underwent eight repeated ETVs at a mean interval of 25 months, with a patency rate of 62.5% after the second procedure. Only two surgeries were aborted for anatomical reasons. The highest success rates (100% in each instance) were achieved for obstructive hydrocephalus resulting from midbrain/tectal tumor (four patients) and pineal tumor (three patients). Conclusions The ETV procedure is an effective management tool for obstructive hydrocephalus in children. It should be considered the primary procedure, rather than ventriculoperitoneal shunts, in carefully selected children. The success rate is dependent on the origin of the hydrocephalus.

The purpose of this study was to assess whether there is an improvement in motor function in persons with cerebral palsy (CP) who have had a reduction of muscle tone by continuous intrathecal baclofen infusion. This was a prospective, open label, non-blinded case series without a control group, conducted at multiple centres. There were 31 subjects, aged 4-29 years. All had a pre-treatment mean lower extremity Ashworth scores ofor= 3 and a significant reduction in tone after a bolus injection of intrathecal baclofen (ITB) and received an implanted pump for continuous delivery of ITB. Motor function was assessed by the Gross Motor Function Measure (GMFM) prior to and 1 year following pump implantation. Significant improvement (p0.05) in mean GMFM scores was seen in subjects8 years (mean change 4.1) and in those from 8-18 years (mean change 3.7) and in subjects with CP Classes 2 and 5 (mean changes 6.2 and 2.9). There was a statistically significant decrease (p0.05) in Ashworth scores in CP classes 2-5. Subjects or their caregivers that completed a survey about perceived changes stated that motor control, positioning and endurance improved.

Object Tectal gliomas are a distinct form of pediatric brainstem tumor that present in patients with symptoms related to increased intracranial pressure due to obstructive hydrocephalus. The natural history of these lesions is often uniquely indolent. Thus, initial surgical therapies are directed at treatment of hydrocephalus, usually with ventricular shunt placement. Recently, third ventriculostomy has been used in patients with tectal gliomas, both as an initial procedure and after shunt failures. In this report the authors review their experience with the treatment of hydrocephalus in patients with tectal gliomas. Methods The authors reviewed 31 consecutive cases of tectal gliomas and compared the success rates of ventricular shunt placement with the success rates of endoscopic third ventriculostomy (ETV). Shunt placement procedures were associated with a significant number of malfunctions, and most patients required shunt revisions. The ETV procedure was attempted both as an initial treatment and after shunt malfunction. Overall, ETV was attempted in 18 patients and was performed successfully in all cases. At the time of follow-up evaluation, 16 patients (89%) were shunt free. Conclusions The authors found that ETV could be performed with good long-term success both as an initial treatment and after shunt failure. Overall, ETV was found to be superior to ventricular shunt placement in the management of hydrocephalus associated with tectal gliomas.

Neurosurgery has come full circle with in the management of childhoodhypertonia and dystonia. Neurosurgeons described some of the first surgeries forthe treatment of hypertonia [1,2]. By the second decade of the twentieth century,a large series of children surgically treated with rhizotomies or surgically sec-tioning nerve roots had been reported [3]. Neurosurgeons then lost interest ascomplications and poor outcomes mounted and other treatments became morewidely used. In the 1960s, however, French neurosurgeons began to re-exploretreating spasticity and other forms of abnormal muscle tone [4]. Interest hasgrown enormously since then, and neurosurgeons are quite actively involved inthe management of such problems. This article describes the types of children inwhom neurosurgical approaches can be helpful, the procedures used, their po-tential complications, and the outcomes that can reasonably be expected.Children with abnormal muscle tone caused by cerebral palsy or other con-ditions typically exhibit complex patterns of involvement with many secondarydeformities. Their clinical histories and examinations vary widely and have littlesimilarity with those of well children or children with single-organ injury ordisease.Forthatreasontheyshouldperiodicallybeassessedbyamultidisciplinarygroup with a specialized interest and experience in treating childhood hypertonia/dystonia. Neurosurgical procedures for the treatment of abnormal muscle tone inchildren are best considered by such multidisciplinary groups with the activeparticipation of a neurosurgeon. A neurosurgeon working in isolation runs therisks of incorrectly employing a procedure and of not appreciating the impact ofthat treatment on the child’s function and success in other treatments such asphysical and occupational therapy. The neurosurgeon will not be involved infollowing such a child and therefore will be unable to appreciate the impact of theprocedure on the child’s subsequent motor development and functional capabili-ties. The neurosurgeon also benefits by consultation with pediatric orthopedists

The neuroendoscopic procedures applied inpatients with isolated compartments, including IUH, LV, IFV, IRF, and multiloculated ventricles were foramen of Monro reconstruction, septostomy, septal wall removal, cyst wall fenestration, fourth ventriculostomy, and endoscopic shunt placement. It was found that the operative goal, creating a state of arrested hydrocephalus, could be achieved by communicating the trapped space to the rest of the ventricular system, opening the ventricular isolation. The associated hydrocephalus could not always be managed endoscopically, however, and shunting of the ventricular system was frequently required, especially in infants. This may be because of the immaturity of the subarachnoid CSF dynamics in infants. What does seem to be logical is to continue to consider managing these conditions with the assistance of the endoscope.

Object. Endoscopically assisted ventricular catheter placement has been reported to reduce shunt failure in uncontrolled series. The authors investigated the efficacy of this procedure in a prospective multicenter randomized trial. Methods. Children younger than 18 years old who were scheduled for their first ventriculoperitoneal (VP) shunt placement were randomized to undergo endoscopic or nonendoscopic insertion of a ventricular catheter. Eligibility and primary outcome (shunt failure) were decided in a blinded fashion. An intention-to-treat analysis was performed. The sample size offered 80% power to detect a 10 to 15% absolute reduction in the 1-year shunt failure rate. The authors studied 393 patients from 16 pediatric neurosurgery centers between May 1996 and November 1999. Median patient age at shunt insertion was 89 days. The baseline characteristics of patients within each group were similar: 54% of patients treated with endoscopy were male and 55% of patients treated without endoscopy were male; 30% of patients treated with and 26% of those without endoscopy had myelomeningocele; a differential pressure valve was used in 51% of patients with and 49% of those treated without endoscopy; a Delta valve was inserted in 38% of patients in each group; and a Sigma valve was placed in 9% of patients treated with and 12% of those treated without endoscopy. Median surgical time lasted 40 minutes in the group treated with and 35 minutes in the group treated without endoscopy. Ventricular catheters, which during surgery were thought to be situated away from the choroid plexus, were demonstrated to be in it on postoperative imaging in 67% of patients who had undergone endoscopic insertion and 61% of those who had undergone nonendoscopic shunt placements. The incidence of shunt failure at 1 year was 42% in the endoscopic insertion group and 34% in the nonendoscopic group. The time to first shunt failure was not different between the two groups (log rank = 2.92, p = 0.09). Conclusions. Endoscopic insertion of the initial VP shunt in children suffering from hydrocephalus did not reduce the incidence of shunt failure.

Summary: Brainstem gliomas have historically been some of the most difficult pediatric cancers to treat. Tumors arising in the brainstem were once uniformly discounted as surgically unapproachable lesions. The advent of magnetic resonance imaging and sophisticated neurophysiologic monitoring techniques has significantly advanced the surgical treatment of these precarious lesions. Brainstem gliomas are now recognized as a heterogenous group of tumors. They have been broadly classified in four categories: diffuse, focal, exophytic, and cervicomedullary. This classification system has provided a framework to predict growth patterns, surgical resectability, and overall prognosis. Although most remain unresectable, there are certain brainstem gliomas that are readily amenable to surgical therapy.

Objective To assess the effectiveness of pudendal afferent mapping as a tool to minimize the risk of postoperative bowel, bladder, and sexual dysfunction in patients undergoing selective posterior rhizotomies in whom the S2 roots are candidates for rhizotomy. Methods One-hundred fourteen children with the diagnosis of cerebral palsy and debilitating spasticity were selected to undergo selective posterior rhizotomies at New York University Medical Center during 1991 through 1995. There were 72 male and 42 female patients with a mean age of 3.8 years. At the time of surgery, none of the patients had clinically relevant bladder dysfunction. Dorsal root action potentials were recorded intraoperatively to map the distribution of pudendal afferent fibers in S1-S3 roots bilaterally before performing the rhizotomies. Results Pudendal afferent mapping was successful in 105 of 114 patients. In the majority of these patients (56%), the distribution was asymmetrical. S1 roots contributed 4%, S2 roots 60.5%, and S3 roots 35.5% of the overall pudendal afferent activity. The pudendal afferent distribution was often confined to a single level in 18% of the patients or even to a single root in 7.6%. Fifty-six percent of the pathologically responding S2 roots during rhizotomy testing were preserved because of the significant afferent activity, as demonstrated during pudendal mapping. None of the 105 patients so mapped developed long-term bowel or bladder complications. Conclusions Pudendal afferent mapping identifies S2 roots that carry a significant number of fibers involved with genital sensation. The preservation of such roots during surgical procedures may be important for sexual function and may also contribute to decreasing postoperative bladder and bowel disturbances.

The screening and detection of recurrent medulloblastoma presents the clinician with significant diagnostic challenges, including the risk of misdiagnosis. The authors present the case of a young girl with a history of a treated standard-risk medulloblastoma that highlights the risk of assuming recurrence has occurred when clinical and/or imaging changes are observed. This girl developed both new clinical deficits and had radiographic evidence of recurrence. She subsequently experienced a complete resolution of symptoms and radiographic findings with steroids alone.

Twenty-four children and young adults who underwent surgery for intrinsic tumors of the medulla were studied, correlating preoperative symptoms to postoperative brain stem dysfunction. 75% harbored benign tumors with a mean survival of 3.6 years, while the remaining 25% with anaplastic tumors had a mean survival of 3.2 years. Six patients required postoperative ventilatory support for an extended period of time, and 9 required feeding gastrostomies. Those patients who experienced frequent symptoms of upper respiratory tract infection, preoperative pneumonia, or an alteration in their voice were at risk of postoperative ventilatory dependency. Those without such symptoms did not experience such a fate. Difficulty in swallowing food or liquids seems to predict the need for postoperative feeding gastrostomies, and a lack of such a history was correlated with no such need 100% of the time. There seem to be preoperative signs and symptoms which predict postoperative brain stem dysfunction for an extended period of time in those undergoing brain stem surgery for tumors intrinsic to the medulla.

The introduction of a second portal during endoscopic procedures is presented. This second portal allows passage of larger instruments while the surgical field is viewed with an endoscope placed through the first portal. This dual portal technique provides an angled view of the operative site, thus avoiding optical obstruction by tissue being pulled toward the endoscope. We report the use of this technique in 6 endoscopic cases.

Background Choroid plexus tumors (CPT) in the pediatric population are usually discovered in symptomatic patients often with symptoms of increased intracranial pressure, with hydrocephalus as the most common presentation, along with seizures, subarachnoid hemorrhage, or focal neurological deficit. Most CPTs are found to be benign choroid plexus papillomas (CPP), whereas a small number are intermediate and malignant choroid plexus carcinomas (CPC). Total surgical resection is the established definitive treatment for symptomatic CPP. Case description We describe a young female who was found to have an incidental CPT during workup for recent head trauma without neurological deficits or hydrocephalus. She underwent a surgical operation to remove the tumor successful, with 1-year follow-up showing no recurrence and normal developmental milestones. Conclusion This rare presentation of an asymptomatic CPT brings attention to the fact that there is no clear evidence for how or when to treat such patients. Because discovery of a CPT in an asymptomatic patient is uncommon, the treatment plan appears to be developed on a case-by-case basis. We hope to generate discussion for establishing an agreed upon treatment approach for CPTs in asymptomatic patients.

WE HAVE IMPROVED upon a brain stem mapping technique that can be used to locate cranial motor nuclei on the floor of the fourth ventricle. This technique helped to intraoperatively locate the facial colliculus and the motor nuclei of cranial nerves IX/X and XII in 14 patients undergoing removal of brain stem tumors. The motor nuclei of these cranial nerves are usually located relative to specific anatomic landmarks on the ventricular floor. These landmarks were not evident in most patients studied because of the distorting effects of the tumor. Different points of the floor of the fourth ventricle were electrically stimulated while electromyographic responses were recorded with electrodes inserted in the orbicularis oculi and orbicularis oris muscles, the lateral posterior pharyngeal wall, and the intrinsic muscles of the tongue. Mapping was performed before and after tumor resection. The technique was found to be useful for locating cranial motor nuclei before tumor resection. It enabled surgeons to avoid damaging the nuclei when entering the brain stem. This technique, however, has certain limitations. Because this is a mapping technique, not a monitoring technique, continuous monitoring during tumor resection was unavailable. Also, the presence of a muscle response after tumor resection did not always indicate preservation of immediate postoperative function (7 days after surgery) because damage to the corticobulbar tract and/or the underlying neural circuitry was not detectable by mapping. Further research is needed to determine the point of stimulation in the functional motor circuitry and the relationship between intraoperative recordings and postoperative function in the long term.

✓ Spinal cord lipomas are rare lesions, accounting for approximately 1% of all spinal cord tumors. True intramedullary spinal cord lipomas are extremely rare and are represented in the literature as scattered, single case reports. The authors present a series of six patients with intramedullary spinal cord lipomas managed at our institution from July, 1985 to July, 1993. The patients' ages ranged from 8 to 45 years. Four patients presented with newly diagnosed tumors and two had undergone previous surgery. Patients usually presented with long histories of disability followed by rapid progression of their symptoms. Most patients were in poor neurological condition on presentation. Presenting symptoms included spinal pain, dysesthetic sensory changes, gait difficulties, weakness, and incontinence. Three patients had cervical tumors, two had cervicothoracic tumors, and one patient had a thoracic tumor. Diagnostic studies, including magnetic resonance imaging, were obtained in all patients. No patient exhibited any form of spinal dysraphism or had a dural defect. All patients underwent decompressive, subtotal resections of 40% to 70% of their lesions. Follow-up times ranged from 12 to 96 months. All patients had resolution of their pain, but they generally showed no neurological improvement. As of their most recent follow-up visit, none of the patients was neurologically normal; three can function independently, although with neurological deficits. The other three patients cannot function independently and have severe neurological deficits. The authors conclude that patients with intramedullary spinal cord lipoma who present with significant neurological compromise have a very poor prognosis with regard to neurological function and generally show no improvement with surgical resection.

Fourth-ventricular shunting is commonly used to treat symptomatic posterior fossa cysts of the Dandy-Walker malformation and trapped fourth ventricle. Although the benefits of this procedure have been widely reported, there is a paucity of data on the pitfalls of posterior fossa shunting in the neurosurgical literature. During the 4-year period from July 1989 to June 1993, we placed fourth-ventricular shunts in 12 patients. Remarkably, 5 patients suffered complications related to posterior fossa catheter placement (42% rate). Three of these patients developed new cranial nerve dysfunction caused by direct injury to the floor of the fourth ventricle, 1 patient suffered an intracystic hemorrhage and acute shunt malfunction, and 1 patient had the catheter tip in the brainstem on postoperative studies without new neurological deficit. We conclude that placement of fourth-ventricular shunts can be fraught with complications which we believe is related to technique. We propose that altering the trajectory of the ventricular catheter from our usual midline technique to a more lateral position will lessen the chances for injury to the floor of the fourth ventricle. In this manner we hope to decrease our incidence of complications for this procedure.

Positioned anatomically between the spinal epidural space and the intramedullary compartment, the spinal subdural space remains the least common area of localized infection in the central nervous system. Infectious processes of the subdural spinal space include subdural spinal empyema, subdural spinal abscess, infected spinal subdural cyst, and infectious spinal subdural cyst. To date, there has been no systematic review of these entities in children, with the cumulative knowledge of the pathophysiologic, microbiologic, and demographic characteristics of these infections relegated solely to few small series and case reports. A series of 11 recent cases culled from the collaboration of international authors are presented. In addition, an exhaustive MEDLINE search and manual review of the international literature was performed, identifying a total of 73 cases of spinal subdural infections in patients under the age of 21. Data of interest include the age, sex, signs, and symptoms at presentation, spinal location of infection, presence of spinal dysraphism, and other comorbidities, offending organism, treatment, outcome, and follow-up. Patients ages ranged from 4 weeks to 20 years (mean, 6.5 years). Males outnumbered females by a ratio of 2:1. Over half (53 %) of spinal subdural infections in children were associated with spinal dysraphism or other congenital abnormalities of the spine. The commonest organism to infect the spinal subdural space in children is mycobacterium tuberculosis and the thoracic spinal region was most commonly infected. The disease is usually treated surgically, although a more expectant approach consisting of antibiotics and observation has also been proposed.