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Objective To develop and validate a model to predict seizure freedom in children undergoing cerebral hemispheric surgery for the treatment of drug-resistant epilepsy. Methods We analyzed 1267 hemispheric surgeries performed in pediatric participants across 32 centers and 12 countries to identify predictors of seizure freedom at 3 months after surgery. A multivariate logistic regression model was developed based on 70% of the dataset (training set) and validated on 30% of the dataset (validation set). Missing data were handled using multiple imputation techniques. Results Overall, 817 of 1237 (66%) hemispheric surgeries led to seizure freedom (median follow-up = 24 months), and 1050 of 1237 (85%) were seizure-free at 12 months after surgery. A simple regression model containing age at seizure onset, presence of generalized seizure semiology, presence of contralateral 18-fluoro-2-deoxyglucose-positron emission tomography hypometabolism, etiologic substrate, and previous nonhemispheric resective surgery is predictive of seizure freedom (area under the curve = .72). A Hemispheric Surgery Outcome Prediction Scale (HOPS) score was devised that can be used to predict seizure freedom. Significance Children most likely to benefit from hemispheric surgery can be selected and counseled through the implementation of a scale derived from a multiple regression model. Importantly, children who are unlikely to experience seizure control can be spared from the complications and deficits associated with this surgery. The HOPS score is likely to help physicians in clinical decision-making.

Optimal management of the bone flap after craniotomy for intracranial infection has not been well defined in the pediatric population. This study reviewed the outcomes of a single Canadian center where immediate replacement of the bone flap was standard practice. This is a retrospective study of all patients who underwent craniotomies for evacuation of epidural or subdural empyema at a single center from 1982 to 2018. Patients were identified using the prospective surgical database maintained by the Division of Pediatric Neurosurgery at BC Children’s Hospital. Primary outcome was treatment failure, defined as reoperation at the site of initial surgery for removal of an infected bone flap or repeat drainage of empyema under the replaced bone flap. Secondary outcome was any reoperation for recurrent infection at any site. Twenty-four patients met the inclusion criteria with a minimum of 3-month follow-up from the index intervention. Treatment failure occurred in four patients (17%), all of whom required repeat surgery for further drainage of pus underlying the bone flap. Mean time to repeat surgery was 13 days. Any reoperation for recurrent infection at any site occurred in three patients. Seven out of 24 patients required a second surgery to evacuate empyema (29.2%). Age, sex, epidural or subdural location, osteomyelitis, and bone flap wash were not associated with the primary outcome of treatment failure. Immediate replacement of the bone flap in the surgical management of pediatric subdural or epidural empyema is reasonable. Replacing the flap at the time of first surgery avoids the morbidity and costs of a subsequent reconstructive operation.

Selective dorsal rhizotomy (SDR) for the management of lower extremity spasticity is a surgical technique that has existed since the 1900s. While much evidence supports its efficaciousness in reducing tone in the short term, limited information exists detailing the long-term outcome and evolution over time of patients undergoing SDR. All publications with 10 years or more of outcome data on patients undergoing SDR were identified from Medline and Embase databases using the search term “Rhizotmy.” Only publications that were in English included patients with cerebral palsy under the age of 21 and discussed SDR for lower extremity spasticity were included. Case reports, reviews without primary data, or publications not accessible online were excluded from the review. A total of 2128 publications were initially identified, of which 19 papers describing 1054 patients fit inclusion and exclusion criteria. GMFCS in most patients improved or remained stable over time after surgery, although durability of improvement and final outcome was dependent on initial functional status. Tone was noted to also substantially improve although a small proportion required additional oral or injectable pharmacologic agents and an even smaller proportion required baclofen pumps. Upper extremity function was also noted to improve substantially after SDR. A significant number of patients were independent for activities of daily living and were engaged in secondary education or gainful employment. A significant proportion of patients still required orthopedic surgery to the lower extremities after SDR, although the incidence of spinal deformity was not observed to be substantially elevated over that of natural history. SDR offers substantial improvements to a number of domains beyond pure tone reduction. These changes are durable over time, although patient selection is crucial in identifying those patients that will have the most benefit. Long-term follow-up is in important in this population given the potential need for further interventions that still exist in many patients.

Background The incidence of positional plagiocephaly has increased significantly over the last two decades, which has caused a service delivery challenge for pediatric neurosurgeons. As a potential solution to the long waitlists for abnormal head shape, a plagiocephaly clinic was established at BC Children's Hospital (BCCH) in Vancouver, Canada. This clinic was supervised by an occupational therapist who had been trained by a neurosurgeon to independently assess and manage patients with a referring diagnosis of positional plagiocephaly. Objectives To determine the efficiency of the BCCH Plagiocephaly Clinic in the management of positional plagiocephaly patients and to investigate the clinic's ability to appropriately identify and refer patients with craniosynostosis to pediatric neurosurgeons for further assessment. Methods A retrospective chart review was conducted to identify patients who were assessed and managed at the BCCH Plagiocephaly Clinic between 2008 and 2014. Data on patient demographics, head shape measurements, and treatment recommendations were collected, and the BC Children's neurosurgical database was cross-referenced to identify craniosynostosis cases missed by the Plagiocephaly Clinic. A descriptive analysis of the clinic's average wait times, severity of the patients' plagiocephaly, and recommended interventions was conducted. In addition, the sensitivity and specificity of the clinic's ability to appropriately refer craniosynostosis patients to pediatric neurosurgery were calculated. Results Of 1752 patients seen in the BC Children's Plagiocephaly Clinic between 2008 and 2014, 66% of patients received counseling about repositioning, 34% were referred for head banding, 19% were referred to physiotherapy for torticollis, and 1.4% were referred to the BC Children's Pediatric Neurosurgery Clinic for suspicion of craniosynostosis. The mean time from referral to first assessment by the Plagiocephaly Clinic was 41 days, and time from referral by the plagiocephaly clinic to diagnosis of craniosynostosis by a pediatric neurosurgeon was 8 days. Pediatric neurosurgeons requested imaging for 6 of the referred patients (25% ). The sensitivity and specificity of the plagiocephaly clinic for referral of craniosynostosis patients to the Pediatric Neurosurgery Clinic were 100 and 99%, respectively. Conclusion The BC Children's Plagiocephaly Clinic is efficient and safe for the initial evaluation and treatment of patients with positional plagiocephaly. The clinic's model decreases wait times, appropriately manages patients with positional plagiocephaly, screens for craniosynostosis with high sensitivity and specificity, and takes pressure off outpatient neurosurgical clinics. This model for assessment of plagiocephaly could be considered in other medical centers.

The aim of this study was to evaluate the outcomes and complications for individual surgeons at British Columbia Children’s Hospital for the treatment of Chiari I Malformation (CMI) in children. This was a retrospective review of patients with CMI who had surgery from 1986 to 2015. We assessed the Chicago Chiari Outcome Scores (CCOS) and complication rates by surgeon. Seventy patients, 38 males and 32 females, underwent posterior fossa decompression including 14 extradural and 56 intradural approaches. Syringomyelia was present in 74.3%. Most syringomyelia improved with no difference between intradural and extradural surgeries. After initial surgery, 13 patients (18.6%) had complications including 2/14 (14.3%) of extradural and 11/56 (19.6%) of intradural surgeries. Two patients required surgical intervention for complications whereas 11 had transient complications. The complication rate by surgeon ranged from 11 to 20% for extradural (2 surgeons only) and 10.5 to 40% for intradural surgeries (4 surgeons). The CCOS ranged from 12 to 15 for extradural and 6 to 16 for intradural. The CCOS ranges for surgeons 1 and 2 were 12–15 and 13–15 respectively for extradural. The CCOS ranges for surgeons 1, 2, 3, and 4 were 12–16, 6–15, 12–16, and 12–16 respectively for intradural. Thirteen patients had a second surgery for CMI. The final CCOS was good in 86% and moderate in 14%. There was variability in surgeries performed at BCCH by different surgeons, with variations in CCOS and complication rates. This information is important during decision making, consent process, and for quality improvement.

In children with spastic cerebral palsy, selective dorsal rhizotomy (SDR) is conducted to improve lower limb spasticity. Improvements in upper extremity function have also been noted in early follow-up. The purpose of this study was to determine if upper extremity improvements are sustained in the long term. A retrospective review of prospectively collected data on children who underwent SDR was conducted. Quality of Upper Extremities Skill Test (QUEST) scores for dissociated movement, grasp and total scores were compared using repeated measures ANOVA for individual patients at three time points: preoperatively, early post-operatively (≤ 2 years) and late post-operatively (9+ years). Out of 200+ patients having SDR, 32 had QUEST assessment at all three time points. Significant improvements in QUEST dissociated movement (F = 3.665, p = 0.045), grasp (F = 7.995, p = 0.001) and total scores (F = 9.471, p = 0.001) were found. Pairwise comparisons were significant from pre-operative to early post-operative times for all QUEST scores (p = 0.001, 0.003, 0.001), and this was maintained at late post-operative assessment for grasp and total scores (p = 0.02, p = 0.02). There was no significant change in scores between early and late post-operative assessment time points. Early improvements in upper extremity QUEST total scores are sustained in the long term following SDR.

A preliminary survey of pediatric neurosurgeons working at different centers around the world suggested differences in clinical practice resulting in variation in the risk of pediatric cerebellar mutism (CM) and cerebellar mutism syndrome (CMS) after posterior fossa (PF) tumor resection. The purposes of this study were (1) to determine the incidence and severity of CM and CMS after midline PF tumor resection in children treated at these centers and (2) to identify potentially modifiable factors related to surgical management (rather than tumor biology) that correlate with the incidence of CM/CMS. Attending pediatric neurosurgeons at British Columbia’s Children’s Hospital (BCCH) and neurosurgeons who completed a pediatric neurosurgery fellowship at BCCH were invited to provide data from the center where they currently practiced. Children aged from birth to less than 18 years who underwent initial midline PF tumor resection within a contemporary, center-selected 2-year period were included. Data was obtained by retrospective chart and imaging review. Modifiable surgical factors that were assessed included pre-resection surgical hydrocephalus treatment, surgical positioning, ultrasonic aspirator use, intraoperative external ventricular drain (EVD) use, surgical access route to the tumor, and extent of resection. CM was defined as decreased or absent speech output postoperatively and CMS as CM plus new or worsened irritability. There were 263 patients from 11 centers in 6 countries (Canada, Germany, the Netherlands, India, Indonesia, and the USA). Median age at surgery was 6 years (range

In 2003, pediatric neurosurgeons were surveyed under the auspices of the education committee of the International Society for Pediatric Neurosurgery (ISPN) to determine prevailing opinions regarding the management of Chiari I malformation (C1M) with and without associated syringomyelia. In the ensuing years, there has been further information from multiple C1M studies, with regards to indications, success rates of different surgical interventions, and complications. The purpose of this study was to re-evaluate current opinions and practices in pediatric C1M. Pediatric neurosurgeons worldwide were surveyed, using an e-mail list provided by the ISPN communication committee chairperson. Respondents were given scenarios similar to the 2003 C1M survey in order to determine opinions regarding whether to surgically intervene, and if so, with which operations. Of 300 surveys electronically distributed, 122 responses were received (40.6% response rate)—an improvement over the 30.8% response rate in 2003. Pediatric neurosurgeons from 34 different countries responded. There was broad consensus that non-operative management is appropriate in asymptomatic C1M (> 90%) as well as asymptomatic C1M with a small syrinx (> 65%). With a large syrinx, a majority (almost 80%) recommended surgical intervention. Scoliotic patients with CIM were generally offered surgery only when there was a large syrinx. There has been a shift in the surgical management over the past decade, with a bone-only decompression now being offered more commonly. There remains, however, great variability in the operation offered. This survey, with a relatively strong response rate, and with broad geographic representation, summarizes current worldwide expert opinion regarding management of pediatric C1M. Asymptomatic C1M and C1M with a small syrinx are generally managed non-operatively. When an operation is indicated, there has been a shift towards less invasive surgical approaches.

Myelomeningocele is typically a disabling condition that results in neurologic, orthopedic, and urologic morbidity. The aim of this study was to examine the trends over time in both incidence and outcomes of myelomeningocele (MMC) in British Columbia (BC). A retrospective chart review was performed of all children with MMC followed in the British Columbia Children’s Hospital (BCCH) Spinal Cord Clinic between 1971 and 2016. The incidence of new MMC cases and the long-term outcomes of MMC were compared between two 10-year cohorts. The first cohort comprised children born with MMC between 1971 and 1981, and the second cohort comprised children born with MMC between 1996 and 2006. A total of 309 children with MMC were followed in the BCCH Spinal Cord Clinic between 1971 and 2016. There were 101 and 46 children with MMC in the two-time cohorts, respectively. Between the earlier and later cohorts, there was a significant difference in the following: MMC incidence [2.5/10,000 births vs 1.1/10,000 births, respectively (p = 0.0002)], mortality [18 vs 0% (p = 0.0009)], and the proportion of cases repaired in under 48 h [56 vs 98% (p

Background: A subset of pediatric epilepsy patients suffer from epilepsy syndromes related to multi-lobar or hemispheric epileptogenic networks and diseases. Most of these patients are refractory to anti-seizure drug treatment, resulting in persistent disabling seizures, neurocognitive delay and impaired quality of life. In well-selected patients, hemispheric surgery is associated with excellent seizure freedom rates and relatively low complication rates. Nevertheless, 30% of patients develop seizure recurrence after cerebral hemispheric surgery. The purpose of this study was to develop and validate a model to predict seizure freedom in children undergoing cerebral hemispheric surgery for the treatment of drug-resistant epilepsy. Methods: We analyzed 1237 hemispheric surgeries performed in pediatric patients across 31 centers and 12 countries to identify predictors of seizure freedom at 12 months after surgery. A multivariate logistic regression model was developed based on the entire dataset and its performance was evaluated based on cross-validation. Missing data was handled using multiple imputation techniques. Findings: Overall, 817/1237 (66%) hemispheric surgeries led to seizure freedom (median follow-up of 24 months), and 1050/1237 (85%) were seizure free at 12 months after surgery. The regression model containing age at seizure onset, presence of generalized seizure semiology, presence of contralateral 18-fluoro-2-deoxyglucose–positron emission tomography hypometabolism, etiologic substrate and previous non-hemispheric resective surgery were predictive of seizure freedom (AUC 0·72). A practical Hemispheric Surgery Outcome Prediction Scale (HOPS) score was devised based on these results that can be used to predict seizure freedom. Interpretation: Children most likely to benefit from hemispheric surgery can be selected and counselled through the implementation of a scale derived from a multiple regression model. Importantly, children who are unlikely to experience seizure control can be spared from the complications and deficits associated with this surgery. The HOPS score is likely to help physicians in clinical decision-making. Funding Statement: The authors stated: "None." Declaration of Interests: None of the authors has any conflict of interest to disclose. None of the authors have financial relationships relevant to this article. Gary W Mathern MD is partly supported by the Davies/Crandall Endowed Chair for epilepsy Research at UCLA. Dr. Phillip Pearl receives research support from the NIH, NSF, and Boston Healthcare Associates; royalty payments from Elsevier, Springer Pubs, and UpToDate; has served as a consultant for Agilis Biotherapeutics and GLG Health Care Council; and is Associate Editor for the Journal of Child Neurology and on the editorial boards of Annals Neurology, Epilepsia, Future Neurology, Music and Medicine, and Neurology. Dr. Jeffrey Ojemann is a shareholder of Therma Neurosciences, a grant recipient of the NIH/NSF, and serves on the advisory board for the Epilepsy Foundation of the Northwest. The views expressed in this article are not the official positions of any authors’ affiliated institutions. Ethics Approval Statement: The organizing center received IRB approval for this study and each contributing center obtained research ethics approval locally.

Cerebral palsy is a common neurological disorder that involves spasticity of the extremities and can lead to lifelong disability. Selective dorsal rhizotomy (SDR) can improve spasticity and quality of life in these patients, but it may be associated with the development of spinal deformity. Risk factors for spinal deformity after SDR have not yet been systematically examined. Medline, Embase, and Web of Science databases were queried for clinical studies reporting incidence of new or worsening spinal deformity, including scoliosis, after SDR. Variables that represent possible risk factors for deformity were correlated with reported incidence of deformity. Twenty-two articles for a total of 1485 patients met the inclusion criteria for this study. Deformity occurs among all patients with a weighted mean incidence of 28.0%. Scoliosis appears to be the most common deformity occurring with a weighted mean incidence of 31.6%. There is substantial heterogeneity between studies, limiting our analysis. Significant positive correlation was found between percent of patients that developed any type of deformity and the ratio of female to male patients, p = 0.02. Significant positive correlation was also found between percent of patients that develop scoliosis and the ratio of female to male patients, p

Osteogenic sarcoma of the skull is uncommon and long-term outcome is not well defined. We review the literature and present a pediatric case of calvarial osteogenic sarcoma with good long-term oncological and cosmetic outcome and excellent quality of life. This case presented major surgical challenges, which are detailed. A 6-year-old boy presented with a painless 5 cm × 5 cm lump over the vertex region. He was neurologically normal. Imaging showed an extensive bony lesion with intradural extension. After incisional biopsy showed probable low grade osteosarcoma, a complete en bloc resection with margins was attempted via a concentric craniotomy around the lesion after embolization to reduce blood loss. Invasion of the brain by the tumor precluded the complete en bloc resection, but gross total resection was achieved. The final pathology was consistent with a low-grade osteosarcoma and adjuvant chemotherapy was provided. Follow-up for 8 years has shown no recurrence with good cosmetic and functional outcome.

OBJECTIVEAt British Columbia Children’s Hospital (BCCH), pediatric patients with nonsyndromic craniosynostosis are admitted directly to a standard surgical ward after craniosynostosis surgery. This study’s purpose was to investigate the safety of direct ward admission and to examine the rate at which patients were transferred to the intensive care unit (ICU), the cause for the transfer, and any patient characteristics that indicate higher risk for ICU care.METHODSThe authors retrospectively reviewed medical records of pediatric patients who underwent single-suture or nonsyndromic craniosynostosis repair from 2011 to 2016 at BCCH. Destination of admission from the operating room (i.e., ward or ICU) and transfer to the ICU from the ward were evaluated. Patient characteristics and operative factors were recorded and analyzed.RESULTSOne hundred fourteen patients underwent surgery for single-suture or nonsyndromic craniosynostosis. Eighty surgeries were open procedures (cranial vault reconstruction, frontoorbital advancement, extended-strip craniectomy) and 34 were minimally invasive endoscope-assisted craniectomy (EAC). Sutures affected were sagittal in 66 cases (32 open, 34 EAC), coronal in 20 (15 unilateral, 5 bilateral), metopic in 23, and multisuture in 5. Only 5 patients who underwent open procedures (6%) were initially admitted to the ICU from the operating room; the reasons for direct admission were as follows: the suggestion of preoperative elevated intracranial pressure, pain control, older-age patients with large reconstruction sites, or a significant medical comorbidity. Overall, of the 107 patients admitted directly to the ward (75 who underwent an open surgery, 32 who underwent an EAC), none required ICU transfer.CONCLUSIONSOverall, the findings of this study suggest that patients with nonsyndromic craniosynostosis can be managed safely on the ward and do not require postoperative ICU admission. This could potentially increase cost savings and ICU resource utilization.

Thalamic gliomas are rare. The natural history is unpredictable, and the optimal management of these tumors in children is poorly defined. The aim was to identify outcomes, prognostic factors, and response to various modalities of treatment in a relatively large population of pediatric thalamic tumors from many centers within a fairly homogeneous health care system. We performed a Canadian multicenter retrospective review of pediatric thalamic tumors presenting during the MRI era (1989–2012). Radiology and pathology were reviewed by central independent reviewers. Paraffin shavings for RNA extraction were taken and tested for fusion events involving KIAA1549:BRAF. Tumors were classified as unilateral or bithalamic based on their origin on imaging. Univariate and multivariate analyses on factors influencing survival were performed. Seventy-two thalamic tumors were identified from 11 institutions. Females represented 53 % of the study population, and the mean age at presentation was 8.9 years. Sixty-two tumors were unilateral and 10 bithalamic. Unilateral tumors had a greater propensity to grow inferiorly towards the brainstem. These tumors were predominantly low grade in comparison to bithalamic tumors which were high-grade astrocytomas. The 5-year overall survival was 61 ± 13 % for unithalamic tumors compared to 37 ± 32 % for bithalamic tumors (p = 0.097). Multivariate analysis indicated tumor grade as the only significant prognostic factor for unithalamic tumors. Six unilateral tumors, all low grade, were BRAF fusion positive. Unilateral and bilateral thalamic tumors behave differently. Surgical resection is an appropriate treatment option in unilateral tumors, most of which are low grade, but outcome is not related to extent of resection (EOR). Bilateral thalamic tumors have a poorer prognosis, but the occasional patient does remarkably well. The efficacy of chemotherapy and radiotherapy has not been clearly demonstrated. Novel therapeutic approaches are required to improve the prognosis for malignant unilateral thalamic tumors and bilateral thalamic tumors.

Intraoperative ultrasound (iUS) is a valuable tool—inexpensive, adds minimal surgical time, and involves minimal risk. The diagnostic predictive value of iUS is not fully characterized in Pediatric Neurosurgery. Our objective is to determine if surgeon-completed iUS has good concordance with post-operative MRI in estimating extent of surgical resection (EOR) of pediatric brain tumors. We reviewed charts of all pediatric brain tumor resections (single institution 2006–2013). Those with iUS and postoperative imaging (

A formal pediatric neurosurgical service in British Columbia (BC) started with the opening in 1982 of British Columbia’s Children’s Hospital (BCCH) as a full service pediatric tertiary care center serving the population of BC and the Yukon (Fig. 1). There had been a Children’s Hospital in Vancouver prior to this at a different location. That previous Children’s Hospital opened in 1933 on West 59th Avenue as a Crippled Children’s Hospital and in 1947 the facility was renamed Children’s Hospital. Neurosurgeons participated at this Children’s Hospital only in the outpatient Spina Bifida clinic. Prior to the opening of the new BCCH in 1982, inpatient pediatric neurosurgical care, including operations, was provided in a wing of the Vancouver General Hospital (VGA), called the Health Centre for Children. VGH was the major tertiary care center serving adults and children in BC and was affiliated with the University of British Columbia (UBC). In 1964, Children’s Hospital and the Health Centre for Children agreed to develop a joint facility and finally, construction began in 1977 at 28th Avenue and Oak Street, the current site of BCCH. The new BCCH opened to its first patients in June 1982. In the years preceding the opening of BCCH, there was a well-established neurosurgical service and neurosurgical residency program at VGH, and this was chaired by Dr. Gordon Thompson. Children and infants, including pediatric neurosurgical patients, were operated on in the general operating room facilities of VGH, with anesthesia provided by general anesthesiologists. Intravenous access in the infants was a significant problem and often cutdowns had to perform to obtain adequate intravenous access. The adult patients typically received the highest priority in the neurosurgical operating room and elective pediatric cases tended to be left until late in the day. All neurosurgeons would do the occasional pediatric operation, especially for patients encountered while on call. However, for many years, the majority of the pediatric neurosurgical operations were performed by Dr. Peter Moyes, a technically-gifted, Mayo Clinic-trained neurosurgeon, but pediatric cases still accounted for a minority of his practice. In the late 1970s, Dr. Moyes was forced to slow down and eventually retire because of illness. The pediatric neurosurgical cases at VGH were referred more and more to Dr. Felix Durity, a graduate of the UBC Neurosurgical residency program in Vancouver, who had joined the neurosurgical group at VGH in 1975. In 1979, Paul Steinbok, who had also trained in the neurosurgical program at VGH, returned from a neurooncology fellowship with Drs. Steve Mahaley and Darryl Bigner in North Carolina and joined the VGH group. Gradually, the pediatric component of his neurosurgical practice increased. By the time that Peter Moyes retired, most of the pediatric neurosurgical cases at BCCH were being managed by Drs. Felix Durity and Paul Steinbok. In 1982, with the opening of BCCH, a pediatric neurosurgical service was required at the new hospital, which was located 2 km from VGH. Comprehensive pediatric * Paul Steinbok psteinbok@cw.bc.ca

Bobble-head doll syndrome (BHDS) is a rare pediatric movement disorder presenting with involuntary 2- to 3-Hz head movements. Common signs and symptoms also found on presentation include macrocephaly, ataxia, developmental delay, optic disc pallor or atrophy, hyperreflexia, tremor, obesity, endocrinopathy, visual disturbance or impairment, headache, and vomiting, among others. The syndrome is associated with suprasellar cysts, third ventricular cysts, or aqueductal obstruction, along with a few other less common conditions. The cause of involuntary head motions is not understood. Treatment is surgical. The authors present 2 cases of BHDS. The first is a 14-year-old boy with BHDS associated with aqueductal obstruction and triventricular hydrocephalus secondary to a tectal tumor. He was successfully treated by endoscopic third ventriculostomy, and all symptoms resolved immediately in the recovery room. This case is unusual in its late age of symptom onset, the primacy of lateral (“no-no”) involuntary head rotations, and the associated tectal tumor. The second case is a 7.5-year-old girl with BHDS associated with a suprasellar cyst. She was successfully treated with an endoscopic fenestration but preexisting endocrinopathy persisted, and the patient was diagnosed with autism spectrum disorder at age 12 years. This second case is more typical of BHDS. A comprehensive and up-to-date review of the literature of BHDS and video documentation of the phenomenon are presented.

The purpose of this study is to evaluate long-term outcomes after selective dorsal rhizotomy (SDR) for children with spastic cerebral palsy. This is a retrospective review of a prospective database of patients who underwent SDR at British Columbia Children’s Hospital. Hip adductor spasticity, hip range of motion (ROM), quadriceps strength, and motor function were assessed pre-operatively, at 6 months to 5 years and more than 10 years postoperatively. Patients were stratified by Gross Motor Function Classification System (GMFCS) level into group 1 (GMFCS II and III) and group 2 (GMFCS IV and V). Forty-four patients, with mean age at SDR of 4.5 years (range 2.9–7.7), were followed for a mean 14.4 years. Spasticity (Modified Ashworth Scale) decreased 1.5 (p

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.

Cerebrospinal fluid (CSF) leakage is a complication of intradural spinal surgery and is associated with poor wound healing and infection. The incidence of CSF leak is reported at ∼16 % in adults, but little information is available in children. The aim of this study is to determine the CSF leak rate and predisposing factors after intradural pediatric spinal surgeries. This study was a retrospective chart review of 638 intradural spinal operations at BC Children’s Hospital. CSF leak was defined as pseudomeningocele or CSF leak through incision. Primary operations to untether lipomyelomeningoceles, myelomeningocele/meningocele closure, and Chiari decompressions were excluded. CSF leaks occurred in 7.1 %, with 3 % having overt CSF leaks through skin (OCSF leak). CSF leaks, specifically OCSF leaks, were associated with postoperative wound infection (P = 0.0016). Sixteen of 45 cases of CSF leak required reoperation. The type of dural suture used, site of operation, or use of fibrin glue did not affect CSF leak rates. Previous spinal surgery (P

Desmoplastic infantile astrocytoma (DIA) and ganglioglioma (DIG) are uncommon and generally benign intracranial tumors that typically affect infants. Unusual cases bearing aggressive clinical and malignant histological features have been described in the literature. We report a patient who was diagnosed at the age of 6 weeks with a DIA that relapsed 3 months postoperatively despite gross total resection (GTR). Pathologic examination revealed several mitoses, not only in the primitive neuroectodermal tumor (PNET)-like areas, but also unexpectedly in the predominating spindle cell component. The Ki67 proliferative index within the spindled component was 25%. The tumor continued to grow after initial relapse despite 2 courses of intensive chemotherapy (including vincristine, carboplatin, and temozolomide), but the recurrence was eventually controlled with oral etoposide. Currently, the patient is stable at 9 months after her initial diagnosis. This case illustrates that rare cases of DIA may display malignant histologic features in the typically benign and predominating spindle cell component and behave in an aggressive clinical manner despite GTR. As such, we recommend early and extended clinical follow-up of all DIA and DIG cases.

Background: Tuberous sclerosis complex (TSC) is a neurocutaneous disorder with a wide spectrum of manifestations. Recent consensus recommendations stress the importance of multidisciplinary management of children with TSC. The objective of this study was to examine the manifestations of TSC at a large referral centre to determine the care needs of this population. Methods: A retrospective, systematic chart review was performed of children with TSC managed at British Columbia Children’s Hospital. Patients were identified through epilepsy and clinical neurophysiology databases. Results: The study population comprised 81 patients, born between 1987 and 2014, who were a median of 10 years (range, 0.2-23.2) at most recent follow-up. Epilepsy occurred in 91% of patients, including 32% with a history of infantile spasms. Nineteen patients underwent epilepsy surgery, nine (47%) of whom were seizure-free at most recent follow-up. Overall, 61% of epilepsy patients had been seizure-free for at least 1 year at the time of last follow-up. Neuropsychiatric disorders were diagnosed in 49% of children, with autism (25%), attention deficit hyperactivity order (19%) and anxiety (16%) being the most common. Cardiac rhabdomyomata occurred in 35% of children and renal angiomyolipomas were seen in 43%. A total of 91% had skin manifestations. Conclusion: This study outlines the multisystem manifestations of TSC, observed through a large pediatric referral center. Epilepsy and neuropsychiatric disorders are the major source of morbidity in this age group and provide many challenges to the treating clinician. Because a subset of the study population is still quite young, the prevalence of neuropsychiatric disorders is likely underestimated.

OBJECTIVE Focal cortical dysplasia (FCD) Type II is divided into 2 subgroups based on the absence (IIA) or presence (IIB) of balloon cells. In particular, extratemporal FCD Type IIA and IIB is not completely understood in terms of clinical, imaging, biological, and neuropathological differences. The aim of the authors was to analyze distinctions between these 2 formal entities and address clinical, MRI, and immunohistochemical features of extratemporal epilepsies in children. METHODS Cases formerly classified as Palmini FCD Type II nontemporal epilepsies were identified through the prospectively maintained epilepsy database at the British Columbia Children's Hospital in Vancouver, Canada. Clinical data, including age of seizure onset, age at surgery, seizure type(s) and frequency, affected brain region(s), intraoperative electrocorticographic findings, and outcome defined by Engel's classification were obtained for each patient. Preoperative and postoperative MRI results were reevaluated. H & E–stained tissue sections were reevaluated by using the 2011 International League Against Epilepsy classification system and additional immunostaining for standard cellular markers (neuronal nuclei, neurofilament, glial fibrillary acidic protein, CD68). Two additional established markers of pathology in epilepsy resection, namely, CD34 and α-B crystallin, were applied. RESULTS Seven nontemporal FCD Type IIA and 7 Type B cases were included. Patients with FCD Type IIA presented with an earlier age of epilepsy onset and slightly better Engel outcome. Radiology distinguished FCD Types IIA and IIB, in that Type IIB presented more frequently with characteristic cortical alterations. Nonphosphorylated neurofilament protein staining confirmed dysplastic cells in dyslaminated areas. The white-gray matter junction was focally blurred in patients with FCD Type IIB. α-B crystallin highlighted glial cells in the white matter and subpial layer with either of the 2 FCD Type II subtypes and balloon cells in patients with FCD Type IIB. α-B crystallin positivity proved to be a valuable tool for confirming the histological diagnosis of FCD Type IIB in specimens with rare balloon cells or difficult section orientation. Distinct nonendothelial cellular CD34 staining was found exclusively in tissue from patients with MRI-positive FCD Type IIB. CONCLUSIONS Extratemporal FCD Types IIA and IIB in the pediatric age group exhibited imaging and immunohistochemical characteristics; cellular immunoreactivity to CD34 emerged as an especially potential surrogate marker for lesional FCD Type IIB, providing additional evidence that FCD Types IIA and IIB might differ in their etiology and biology. Although the sample number in this study was small, the results further support the theory that postoperative outcome—defined by Engel's classification—is multifactorial and determined by not only histology but also the extent of the initial lesion, its location in eloquent areas, intraoperative electrocorticographic findings, and achieved resection grade.

Paediatric brain tumours arising in the thalamus present significant diagnostic and therapeutic challenges to physicians due to their sensitive midline location. As such, genetic analysis for biomarkers to aid in the diagnosis, prognosis and treatment of these tumours is needed. Here, we identified 64 thalamic gliomas with clinical follow-up and characterized targeted genomic alterations using newly optimized droplet digital and NanoString-based assays. The median age at diagnosis was 9.25 years (range, 0.63–17.55) and median survival was 6.43 (range, 0.01–27.63) years. Our cohort contained 42 and 22 tumours reviewed as low and high grade gliomas, respectively. Five (12 %) low grade and 11 (50 %) high grade gliomas were positive for the H3F3A/HIST1H3B K27M (H3K27M) mutation. Kaplan-Meier survival analysis revealed significantly worse overall survival for patients harbouring the H3K27M mutation versus H3F3A/HIST1H3B wild type (H3WT) samples (log-rank p

EPN-37. PAEDIATRIC SPINAL EPENDYMOMA IN CANADA: A MULTICENTRE RETROSPECTIVE STUDY Bjorn Baadjes1, Sylvia Cheng1, Paul Steinbok1, Jim Potts1, Karen Goddard2, Tamir Ailon3, Uri Tabori4, Anne-Sophie Carret5, Daniel McNeely6, David Eisenstat7, Beverly Wilson7, Donna Johnston8, Shayna Zelcer9, Katrin Scheinemann10, Christopher Dunham1, and Juliette Hukin1; British Columbia Children’s Hospital, Vancouver, BC, Canada; British Columbia Cancer Agency, Vancouver, BC, Canada; Vancouver Hospital and Health Sciences Centre, Vancouver, BC, Canada; The Hospital for Sick Children, Toronto, ON, Canada; Sainte-Justine Hospital, Montreal, QC, Canada; IWK Health Centre, Halifax, NS, Canada; Stollery Children’s Hospital, Edmonton, AB, Canada; Children’s Hospital of Eastern Ontario, Ottawa, ON, Canada; Children’s Hospital of Western Ontario, London, ON, Canada; The University Hospital Munster, Munster, NRW, Germany BACKGROUND: Ependymomas in children are predominantly located intracranially, with spinal cord ependymomas being rare. Our objective was to determine long-term outcomes of our Canadian paediatric cohort with spinal ependymoma. METHODS: This retrospective multicentre study analysed diagnosis, treatment and outcome of spinal ependymoma patients treated at 9 paediatric oncology centres in Canada between 1986 and 2006. RESULTS: Thirty-six children (20 male), median age 11.9 years (interquartile range 9.1 to 14.4 years), were identified. Length of follow-up was 72.8 months (34.6 to 108.4 months). Thirty-one patients (86%) had WHO grade I disease (all myxopapillary ependymoma located in cauda equina), while 5 (14%) had WHO grade 2 disease. Twenty-fiveof 36 patients (69%) underwent gross total resection (GTR) of tumour, with 11/36 (31%) patients having incomplete resections. Six patients received focal adjuvant radiation therapy. Twelve of 36 (33%) relapsed, with a median time to relapse of 27 months. Six of 25 patients (24%) with GTR relapsed, while 6/11 (55%) patients with incomplete resectionrelapsed.Amongrelapsedpatients, 9underwent further surgery (4GTR,4 incomplete resection, 1 unknown) and 8 had adjuvant radiation therapy. There was no statistical difference in WHO grade and whether adjuvant radiation therapy was administered at diagnosis between relapse and non-relapse patients. Seventeen patients (47%) were disease-free, 18 (50%) were alive with disease, and tumour status on one patient was unknown. One patient died of the disease. CONCLUSIONS: Spinal ependymoma in children is rare, but overall survival outcomes are excellent despite some children suffering recurrence. Neuro-Oncology 18:iii30–iii39, 2016. doi:10.1093/neuonc/now070.36 #The Author(s) 2016. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

The authors describe the case of a late preterm infant girl who presented prenatally with a low lumbar neural tube defect and features of Chiari malformation type II (CM-II). At birth, she exhibited stridor and underwent surgical repair of a lumbosacral myelomeningocele on Day 2 of life. The prognosis was deemed to be poor, and hence a “Chiari decompression” procedure was not undertaken. The patient was subsequently extubated and died on Day 10. Postmortem findings included a rarely described but characteristic granulomatous meningitic reaction to vernix caseosa, which presumably entered the subarachnoid space and spinal cord syrinx antenatally via the open neural tube defect. The significance of congenital stridor in the context of CM-II and in particular the role of vernix caseosa granulomatous meningitis are examined. The antenatal repair of myelomeningoceles, as championed by some, may prevent this ominous meningitic complication.

Stridor, associated with vocal cord paralysis, in neonates with myelomeningocele (MMC) is a recognized symptom related to Chiari II malformation (CM). In most children, stridor appears after birth. Control of hydrocephalus, if present, and urgent decompression of the CM are recommended for treatment of these patients. Such management typically improves symptoms. Occasionally, stridor is present at birth and may be secondary, in part, to maldevelopment or prenatal ischemia of the brain stem, rather than treatable compression. There is minimal literature describing the outcome after Chiari decompression in this population. The purpose of this study was to review the outcomes of neonates with MMC and stridor at birth and compare it to MMC patients who develop stridor later. We hypothesized that unlike stridor which develops after birth, stridor at birth predicts a dismal outcome, despite aggressive surgical treatment. Retrospective review of newborns with MMC and CM was performed in our institution from 1975 to 2010. Patients with stridor at birth and those who developed stridor later in infancy were identified. Outcomes were analyzed. Autopsy findings were reviewed when available. Six patients with MMC who presented with stridor at birth were identified. Five of these patients had decompression of CM and treatment of hydrocephalus, if present, within the first 2 weeks of life. All patients died: three within 1 month and the oldest at 62 months. In the three patients with autopsies, vernix caseosa meningitis was present. Eight patients presented with stridor later in infancy. CM decompression was performed in seven of them. One patient out of the seven with late onset of stridor died at 13 months after CM surgery. The mortality rate after CM decompression was worse in patients with stridor at birth than those presenting later with stridor (chi-square p = 0.015). In newborns with MMC, stridor at birth may predict dismal outcome despite CM decompression. Unlike the situation in neonates who develop stridor after birth, the outcome in those presenting with stridor at birth does not seem to be impacted by decompression of the CM. Nonoperative management may be an option to offer in this population. Additionally, vernix caseosa meningitis may contribute to the severe irreversible brain stem dysfunction in these newborns.

Object In general, patients who present with low Glasgow Coma Scale (GCS) scores and/or fixed and dilated pupils are not expected to do well following arteriovenous malformation (AVM) hemorrhage. However, there is a sense among neurosurgeons that pediatric patients may make a better recovery than adults following such an event. There have been few studies focusing on the outcome of pediatric patients with poor neurological status following AVM hemorrhage. The purpose of this study was to characterize functional outcome in pediatric patients with severe disability after AVM hemorrhage. Methods This was a retrospective analysis of clinical presentation and outcome in 15 patients seen at the authors' pediatric hospital presenting with low GCS scores (defined as GCS ≤ 8) following AVM hemorrhage. Results Initial GCS scores ranged from 3 to 6, and 11 of 14 patients had fixed pupils on clinical examination (data were not available in 1 patient). Eight of 15 patients suffered primarily a lobar hemorrhage, 3 suffered primarily infratentorial bleeding, 2 suffered primarily hemorrhages of the basal ganglia, and 2 suffered intraventricular hemorrhage. The overall mortality rate was 20% (3 of 15 patients). The clinical outcome of survivors was defined by the Pediatric Cerebral Performance Category (PCPC) and Pediatric Overall Performance Category (POPC) scores at follow-up. One year after AVM hemorrhage, 7 (58%) of the 12 surviving patients showed normal or mild disability (PCPC Score 1 or 2), whereas 5 (42%) of 12 patients had moderate or severe disability (PCPC Score 3 or 4). No patients were in a coma or vegetative state, and 11 (92%) of the 12 patients were functioning independently (POPC Score 1, 2, or 3) 1 year after AVM hemorrhage. All patients were functionally independent by last follow-up, with 8 patients (67%) in the normal or mild disability PCPC category, and 4 in the moderate category (PCPC Score 3). All 12 survivors made a meaningful recovery and went on to live independent lives. Conclusions Pediatric patients suffering AVM hemorrhage have a good outcome and are able to function independently, despite a poor neurological state initially.

Object The criteria for identifying patients in whom endoscopic third ventriculostomy (ETV) provides control of hydrocephalus remain in evolution. In particular, it is not clear when ETV would be effective if intraventricular obstruction is not found preoperatively. The authors postulated that 1) displacement of the third ventricle floor inferiorly into the interpeduncular cistern and displacement of the lamina terminalis anteriorly into the lamina terminalis cistern could predict clinical success of ETV, and 2) improvement in these displacements would correlate with the success of ETV. Methods Magnetic resonance imaging in 38 consecutive patients treated between 2004 and 2010 was reviewed to assess displacement of the lamina terminalis and third ventricular floor prior to and following ETV. Displacements of the floor and lamina terminalis were judged qualitatively and quantitatively, using a newly created index, the Third Ventricular Morphology Index (TVMI). The association between the aforementioned morphological features and clinical success of ETV was analyzed. Results Ninety-six percent of patients in whom the authors preoperatively observed displacement of the lamina terminalis and the third ventricular floor were successfully treated with ETV. Displacements of the third ventricular floor and lamina terminalis, as judged qualitatively, correlated with the clinical success of ETV. The TVMI correlated with the qualitative assessments of displacement. Postoperative decrease in the TVMI occurred in the majority of successfully treated patients. Changes in third ventricular morphology preceded changes in other measures of third and lateral ventricular volume following ETV. Conclusions Assessment of third ventricular floor and lamina terminalis morphology is useful in predicting clinical success of ETV and in the follow-up in treated patients. The TVMI provided a quantitative assessment of the third ventricular morphology, which may be useful in equivocal cases and in research studies.

Object Microfibrillar collagen hemostat (MCH; trade name Avitene) is a partially water-insoluble acid salt of purified bovine corium collagen. This agent has been widely used to control hemorrhage at surgery, and especially during pediatric neurosurgeries at the authors' institution. Despite its effectiveness, rare case reports detailing adverse inflammatory reactions to MCH have been documented. Based primarily on MR imaging, postoperative reactions have most commonly elicited clinical differential diagnoses of tumor recurrence or abscess. According to the literature, MCH induces a very characteristic mixed inflammatory response that is rich in eosinophils; in light of these observations, many authors have suggested an allergy-based pathogenesis. Methods The authors retrospectively reviewed 3 pediatric neurosurgical cases treated at their institution, wherein a common histomorphological inflammatory reaction to MCH was elicited at the site of prior craniotomy. Results Case 1 is that of a 10-year-old girl whose diagnosis was a right temporal lobe ganglioglioma, classified as WHO Grade I. Case 2 is that of a 9-year-old boy whose diagnosis was a left parietal lobe anaplastic ependymoma, classified as WHO Grade III. Finally, Case 3 is that of a 15-year-old girl whose diagnosis was focal cortical dysplasia Type IIA affecting the left occipital lobe. Each patient presented with new or recurrent seizures 5–6 weeks after the initial resection. The postsurgical reactions incited by MCH mimicked the radiological appearance of either an abscess (Cases 2 and 3) or recurrent tumor (Case 1). Histologically, the mixed inflammatory infiltrate was typified by the presence of MCH-centric necrotizing granulomas that were surrounded by a palisade of macrophages and often several eosinophils. Conclusions The findings are in keeping with previous case reports describing the clinicopathological features of adverse reactions occurring due to MCH. Based on the authors' observations, the possibility of an idiopathic inflammatory reaction to MCH should be considered when either seizures, a typical radiological appearance (that is, consistent with tumor recurrence or abscess formation), or both arise shortly after initial surgery. A conservative treatment approach to this type of inflammatory lesion appears to be the most appropriate management strategy.

Ten year survival rates for patients with craniopharyngioma vary from 24 to 100%. A review of the database of all children diagnosed with craniopharyngioma in British Columbia (BC) revealed that several patients died >10 years after diagnosis. This retrospective study investigates the causes and timing of deaths and reports the overall survival in this population based group of patients. A chart review was conducted on all patients aged

To describe the novel finding of epileptic spasms arising from a cingulate lesion.We describe two children presenting with epileptic spasms who had lesions of the cingulate gyrus on MRI.In both patients, the lesions involved the midcingulate cortex. Seizures were characterized by prominent motor manifestations and resembled spasms. Both patients had a complete resolution of seizures after resection of their cingulate lesions.Cingulate seizures can present with a variety of behaviors including complex motor manifestations, vocalizations, and autonomic changes. Lesions in the midcingulate cortex can also produce epileptic spasms.

Summary Objective: To determine the clinical characteristics, surgical challenges, and outcome in children younger than 3 years of age undergoing epilepsy surgery in Canada. Methods: Retrospective data on patients younger than age 3 years who underwent epilepsy surgery at multiple centers across Canada from January 1987 to September 2005 were collected and analyzed. Results: There were 116 patients from eight centers. Seizure onset was in the first year of life in 82%, and mean age at first surgery was 15.8 months (1–35 months). Second surgeries were done in 27 patients, and a third surgery in 6. Etiologies were malformations of cortical development (57), tumor (22), Sturge-Weber syndrome (19), infarct (8), and other (10). Surgeries comprised 40 hemispheric operations, 33 cortical resections, 35 lesionectomies, 7 temporal lobectomies, and one callosotomy. There was one surgical mortality. The most common surgical complications (151 operations in 116 patients) were infection (17) and aseptic meningitis in 13. Of 107 patients with seizure outcome assessed more than one year postoperatively, 72 (67.3%) were seizure free (Engel I), 15(14%) had >90% improvement (Engel II), 12 had >50% improvement (Engel III), and 8 did not benefit from surgery (Engel IV). Development improved in 55.3% after surgery. Conclusion: Epilepsy surgery in children younger than 3 years of age is relatively safe and is effective in controlling seizures. Very young age is not a contraindication to surgery in children with refractory epilepsy, and early surgery may impact development positively.

The perceived need for electrophysiological guidance (EPG) during selective dorsal rhizotomy (SDR) has limited the frequency with which SDR is performed. The need for EPG during SDR has been questioned. At our institution, of200 children with SDR for spastic cerebral palsy, 22 children underwent SDR without EPG using clinical guidance (no EPG group). Electrophysiological stimulation was used to distinguish dorsal from ventral roots. The remainder had SDR with EPG. The purpose of this study was to compare outcomes between the groups having SDR with and without EPG.The 22 patients in the no EPG group were matched with 22 controls in whom EPG was used, with respect to Gross Motor Function Classification System score (GMFCS) and age. The 12-month outcomes with respect to motor function score, hip adductor spasticity (Ashworth), hip abduction range of motion (ROM), quadriceps power [Medical Research Council (MRC)], WeeFIM, Quality of Upper Extremities Skills Test (QUEST), and incidence of complications were compared.There were no statistically significant differences preoperatively with respect to GMFCS, age, gross motor function, Ashworth or MRC scores, joint ROM, WeeFIM, or QUEST. At 1 year after SDR, there were no differences between the groups in the incidence of complications or outcome measures. Percentage of dorsal roots cut was similar, but the duration of surgery was significantly shorter in the no EPG group.There was no advantage of doing SDR with EPG compared to no EPG. SDR can reasonably be done in centers where EPG is not available, but electrophysiological stimulation to distinguish dorsal from ventral roots may be useful in avoiding complications.

Brain tumors are one of the most common types of solid neoplasm in children. As life expectancy of these patients has increased with new and improved therapies, the morbidities associated with the treatments and the tumor itself have become more important.One of the most common morbidities is growth hormone deficiency, and since recombinant growth hormone (GH) became available, its use has increased exponentially. There is concern that in the population of children with brain tumors, GH treatment might increase the risk of tumor recurrence or progression or the appearance of a second neoplasm. In the light of this ongoing concern, the current literature has been reviewed to provide an update on the risk of tumor recurrence, tumor progression, or new intracranial tumor formation when GH is used to treat GH deficiency in children, who have had or have intracranial tumors.On the basis of this review, the authors conclude that the use of GH in patients with brain tumor is safe. GH therapy is not associated with an increased risk of central nervous system tumor progression or recurrence, leukemia (de novo or relapse), or extracranial non-leukemic neoplasms.

A randomized controlled single-blind trial was performed to compare lumbo-sacral selective posterior rhizotomy (SPR) followed by intensive physiotherapy, with intensive physiotherapy alone in improving motor function in children with spastic diplegic cerebral palsy. Fifteen patients were randomly assigned to each treatment modality. Patients in the SPR group had rhizotomy within 1 month, followed by intensive outpatient physiotherapy for 9 months. Patients assigned to physiotherapy alone had identical intensive physiotherapy. There was a statistically significant and clinically important difference in improvement in motor function in favor of the SPR group, with a mean increase in total Gross Motor Function Measure (GMFM) score of 11.3% at 9 months for the SPR group compared with 5.2% for the physiotherapy-only group (P = 0.007). Significant improvements in spasticity (P < 0.001) and range of movement (P < 0.001) were noted in the SPR group compared to the physiotherapy-only group. The results indicate that the improvement in motor function after SPR is more than can be explained by the associated intensive physiotherapy.

A head fixation device with pins is commonly used for immobilization of the patient’s head during craniotomy. The safety of head fixation devices in children has been discussed rarely in the literature. The purpose of this report is to review our experience with complications of head fixation with pins in children undergoing craniotomies and to review the literature on this subject. The database of the Division of Neurosurgery was reviewed to identify children who had cranial complications related to the use of a pin head fixation device. The charts of these patients were reviewed retrospectively. Five of 766 children (0.65%) undergoing craniotomies with pin fixation of the head had depressed skull fractures and/or epidural hematomas from the pin fixation. Age ranged from 2.6 to 7.5 years; all fractures were temporal and occurred during posterior fossa craniotomies. Depressed skull fractures and associated epidural hematomas need to be considered as possible complications of pin fixation of the head for craniotomy in young children.

The significance of beaten copper appearance (BCA) on skull radiographs in children following surgery for isolated sagittal craniosynostosis has not been studied. This study was designed to look for any correlation between BCA and symptoms suggestive of intracranial hypertension in this group of patients.Forty-eight consecutive children, who were operated for isolated sagittal synostosis from 1987 to 2000 and had postoperative skull radiographs, were included. Patients were divided into: (a) BCA group (n = 20), consisting of children who had beaten copper appearance on skull radiographs at last follow up, and (b) Non-BCA group (n = 28), consisting of children who did not have this finding. Records were reviewed to look for symptoms suggestive of intracranial hypertension, such as headache, head banging, and irritability.Median age at surgery was 4.8 months for BCA group and 4 months for the non-BCA group. Follow up ranged from 4 to 156 months with a mean of 36.2 months. Total of 28.6% (n = 6) of the children with follow up radiographs done ator =18 months of age had BCA. The incidence of BCA increased to 83.3% in children with skull radiographs performed after 48 months of age. In 18 (90%) children, the BCA was 'diffuse' with 5 (25%) children having the maximum possible score of 8. In the BCA group, 45% (n = 9) had symptoms compared to 10.7% (n = 3) in the control group (p = 0.0068).This study suggests a significant number of children with BCA on radiographs develop symptoms suggestive of raised ICP following surgical treatment in infancy and prolonged follow up may be warranted in this group of patients.

The purpose of this report is to review the historical development, current operative techniques, selection criteria, outcomes, and complications of selective dorsal rhizotomy (SDR) for treatment of spastic cerebral palsy (CP).This review is based on a review of literature and personal observations.SDR has evolved from the 1960s onwards into a standard neurosurgical procedure for spastic CP. There is much variation in the operative technique among surgeons with respect to the level of exposure, electrophysiological guidance, and extent of rhizotomies. Appropriate selection of patients for SDR requires determination that spasticity, not dystonia, is the major disabling hypertonia and that the lower limbs are maximally involved. Positive outcomes have been well demonstrated in the impairment, functional limitations, and disability dimensions, as per the National Center for Medical Rehabilitation Research Model of Dimensions of the Disabling Process. Complications have been relatively few.SDR is the procedure of choice for treatment of spasticity in spastic diplegic CP and in selected children with spastic quadriplegic CP. Optimal selection and outcomes are achieved using a multidisciplinary approach.

OBJECTIVE: Hypodensities on computed tomographic (CT) brain scans are thought to take at least 6 hours to become apparent after blunt head trauma. This finding, in conjunction with the later evolution of the hypodensities, is used in timing the injury in children with suspected non-accidental brain injury, in whom the history may be inaccurate. The purpose of this study is to report the occurrence of diffuse cerebral parenchymal hypodensities on CT scans performed within 5 hours of a well-defined accidental head injury. METHODS: A retrospective review was performed of five patients admitted to British Columbia Children's Hospital who had accidental head injury and who were identified as having diffuse cerebral hemispheric hypodensities on early CT scans. RESULTS: We present five patients (age range, 4 mo-14 yr) with well-documented accidental head injuries who demonstrated obvious and extensive CT brain scan cerebral hemispheric hypodensity from 60 minutes to 4.5 hours after trauma. All five patients presented with severe head injuries and immediate, unremitting coma, and all five progressed rapidly to brain death within 48 hours. CONCLUSION: It is unusual, but possible, to develop CT hypodensities as early as 1 hour after accidental head injury. In our small series of cerebral hemispheric hypodensity occurring less than 5 hours after trauma, all five patients had a uniformly fatal outcome. These observations may be important medicolegally in the assessment of the timing of head injury when the history of the trauma is not clear, as in children with suspected non-accidentally inflicted injury. It is inappropriate to generalize these findings to patients who are not unconscious immediately after a head injury, who regain consciousness after an injury before deteriorating, or who do not progress rapidly to brain death. Language: en

Summary: Health-related quality of life (HRQOL) is an important outcome measure in clinical research. Given the psychosocial and behavioral difficulties associated with pediatric epilepsy, evaluating HRQOL in this patient population is of particular importance. Though HRQOL has been examined in pediatric patients receiving focal resection or pharmacological (antiepileptic drug; AED) treatment, it has not been assessed in patients receiving hemispherectomy (HE) for intractable epilepsy. The current study evaluated HRQOL in a sample of pediatric HE cases (N = 26) using previously validated questionnaires relative to surgical (N = 30) and nonsurgical (N = 84) comparison groups. Compared with focal resection and nonsurgical patients, parents of children who received HE reported similar levels of HRQOL. In surgical cases, worse HRQOL was correlated with residual seizure frequency. In both surgical and nonsurgical cases, female gender, higher AED load, and lower functional independence predicted worse HRQOL. Interestingly, HE status (i.e., having undergone HE) predicted fewer epilepsy-related limitations. Consistent with previous findings, AED load, in addition to lower functional abilities, appear particularly detrimental to life quality in pediatric epilepsy. HE, however, is not associated with increased risk for poor HRQOL. When considered in light of the multiple, significant risk factors for poor outcome associated with HE, children who undergo the procedure fare surprisingly well.

The survival rate for childhood craniopharyngioma has been improving, with more long-term survivors. Unfortunately it is rare for the patient to be normal, either from the disease itself or from the effects of treatment. Long-term survivors of childhood craniopharyngioma suffer a number of impairments, which include visual loss, endocrinopathy, hypothalamic dysfunction, cerebrovascular problems, neurologic and neurocognitive dysfunction. Pituitary insufficiency is present in almost 100%. Visual and hypothalamic dysfunction is common. There is a high risk of metabolic syndrome and increased risk of cerebrovascular disease, including stroke and Moyamoya syndrome. Cognitive, psychosocial, and emotional problems are prevalent. Finally, there is a higher risk of premature death among survivors of craniopharyngioma, and often this is not from tumor recurrence. It is important to consider craniopharyngioma as a chronic disease. There is no perfect treatment. The treatment has to be tailored to the individual patient to minimize dysfunction caused by tumor and treatments. So "cure" of the tumor does not mean a normal patient. The management of the patient and family needs multidisciplinary evaluation and should involve ophthalmology, endocrinology, neurosurgery, oncology, and psychology. Furthermore, it is also important to address emotional issues and social integration.

Sagittal craniosynostosis, the most common single suture craniosynostosis, is treated by numerous surgical techniques. Minimally invasive endoscopy-assisted procedures with postoperative helmeting are being used with reports of good cosmetic outcomes with decreased morbidity, shortened hospital stay, and less blood loss and transfusion. This procedure uses small skin incisions, which must be properly placed to provide safe access to the posterior sagittal and lambdoid sutures. However, the lambda is often hard to palpate through the skin due to the abnormal head shape. The authors describe their experience with the use of intraoperative, preincision ultrasound localization of the lambda in patients with scaphocephaly undergoing a minimally invasive procedure. This simple technique can also be applied to other operations where proper identification of the cranial sutures is necessary.

The focus of this annual special issue is posterior fossa tumors in children. There are a number of reasons to choose a unique theme for publication in a special issue. The most obvious is when a new field develops (e.g., antenatal neurosurgery) or when new progress or understanding has occurred in a narrow field of interest (e.g., cortical dysplasia in children or treatment of craniopharyngiomas). However, it is also important from time to time to have an up-to-date appraisal on a common topic (e.g., hydrocephalus, craniosynostosis) in which we all have extensive knowledge but in which new trends, sometimes at the limit of our field of competence, make such an update valuable. Posterior fossa tumors are clearly in this latter category. They are part of our daily practice and thousands of them are treated all around the word in any pediatric neurosurgical unit. However, advanced neuroimaging, developments in molecular genetics, the use of intraoperative neurophysiology, and better understanding of longer-term results have changed our management of these common tumors. This special issue covers the different types of posterior fossa tumors, common (medulloblastoma, ependymoma, cerebellar astrocytoma, brain stem tumor) or less common and less discussed (CriNET, PNET, CP angle tumors), and focuses on specific and sometimes controversial surgical aspects of these tumors (biopsy in DIPG, surgical approaches of the brain stem, intraoperative monitoring, treatment of hydrocephalus). The value of neuroimaging of these tumors is also reviewed. Most importantly, we wished to address in detail a number of topics for which our understanding has improved dramatically in the last 10 years and pushed us to completely reconsider the treatment of posterior fossa tumors in children. Firstly, the new biological understanding of the nature of these tumors has led to new classifications, new prognostic factors, and new protocols of treatment, some of which have already been implemented and others that are in development for future management. Secondly, with the increased number of long-term survivors of patients with posterior fossa tumors, it is obvious that the quality of life is a crucial aspect of the management of these tumors. Thirdly, our understanding of the importance of the cerebellum not only in motor function but also in higher cortical function has become better recognized and in this issue, cerebellar mutism and all the long-term neuropsychological problems after posterior fossa tumor surgery and their possible solutions are extensively discussed. We wish to thank all the authors for their tremendous contributions, and we hope that you will have the same interest and pleasure to read this special issue as we have had to edit it.

Our center previously reported low transfusion rates for craniosynostosis surgery by two experienced neurosurgeons using standard intraoperative techniques and acceptance of low hemoglobin levels. This study evaluated whether low rates were maintained over the last 10 years and if a less experienced neurosurgeon, trained in and practicing in the same environment, could achieve similar outcomes. All craniosynostosis operations performed in children between 2004 and 2015 were reviewed retrospectively. Transfusion rates were calculated. Analyses examined the relationship of transfusion to craniosynostosis type, surgical procedure, redo operation, surgeon, and perioperative hemoglobin levels. Two hundred eighteen patients were included: 71 open sagittal, 28 endoscopic-assisted sagittal, 32 unicoronal, 14 bicoronal, 42 metopic, and 31 multisuture. Median age at operation was 9.1 months. Overall transfusion rate was 24 %: 17 % open sagittal, 7 % endoscopic-assisted sagittal, 6 % unicoronal, 21 % bicoronal, 45 % metopic, and 45 % multisuture. The timing of transfusions were 75, 21, and 4 % for intraoperative, postoperative, and both, respectively. Patients not receiving transfusion had a mean lowest hemoglobin of 87 g/l (range 61–111) intraoperatively and 83 g/l (range 58–115) postoperatively. Mean lowest hemoglobin values were significantly lower in those necessitating intraoperative (75 g/l, range 54–102) or postoperative (59 g/l, range 51–71) transfusions. There was no significant difference in transfusion rate between less and more experienced surgeons. There were no cardiovascular complications or mortalities. In craniosynostosis surgery, reproducible, long-term low blood transfusion rates were able to be maintained at a single center by careful intraoperative technique and acceptance of low intraoperative and postoperative hemoglobin levels in hemodynamically stable patients. Furthermore, low rates were also achieved by an inexperienced neurosurgeon in the group. This suggests that these results may be achievable by other neurosurgeons, who follow a similar protocol.

OBJECT Cerebellar cavernous malformations (CCMs) have not been specifically described in the pediatric age group. Authors of this study, after considering the published literature, describe the characteristic clinical, radiological, and surgical features of CCM in children. METHOSDS Patients younger than 18 years of age who were known to have CCM and had undergone surgery between 1992 and 2014 at the authors’ institution were reviewed. Pediatric CCM cases reported in the literature (case reports and cases included in series on CMs in the pediatric age group) were also analyzed for specific features of this entity. RESULTS Four male patients and 1 female patient (2.5–14 years of age) with CCM presented acutely with severe headache followed by cerebellar dysfunction. In all patients, neuroimaging (cranial CT and MRI) demonstrated hemorrhagic cerebellar lesions with heterogeneous T1 and T2 signal intensities and hyperintense blooming on susceptibility-weighted imaging. The lesions reached large sizes exhibiting spherical, cystic, and often “pseudotumoral” morphology. In 3 patients, developmental venous anomalies (DVAs) were found. In 4 of the 5 patients, the CCMs and hematomas were totally removed. All patients had a clinically excellent functional outcome without surgical complication and with complete resolution of their presenting symptoms. CONCLUSIONS Cerebellar CMs occur in all pediatric age groups and display characteristic clinical and imaging features. In children, CCMs reach large sizes and can result in massive hemorrhage, often leading to a possible diagnosis of hemorrhage into a tumor. An associated DVA is quite common. Surgery is a safe and efficient treatment option with excellent outcomes in patients.

OBJECT Current craniosynostosis procedures can result in complications due to absorbable plates and screws or other specialized expensive hardware. The authors propose the cranial orbital buttress (COB) technique of frontoorbital remodeling for metopic and unicoronal synostoses, wherein no plates or screws are used. They hypothesize that, with this technique, aesthetically acceptable outcomes for unicoronal and metopic synostosis can be achieved. In this article, they present this technique and compare the results with current frontoorbital remodeling practices. METHODS The authors conducted a retrospective chart review of cases in which patients with nonsyndromic unicoronal or metopic synostosis underwent cranio-orbital surgery at their institution from 1985 through 2009. Operative parameters, surgical variations, and complications were analyzed. The COB technique uses a 1-piece switch, hemiforeheads, or multiple pieces for forehead remodeling. The supraorbital bar is reconstructed in patients with metopic synostosis using a double wedge or greenstick fracture technique, and in patients with unicoronal synostosis a hinge procedure based on a 1.5-orbital osteotomy is used. The supraorbital bar is advanced and supported in place by bone graft(s) inserted at the lateral aspect(s) of the orbit(s) to form a buttress, with fixation done using absorbable sutures. RESULTS A total of 79 cases met the criteria for inclusion in the study. Twenty-nine patients had metopic synostosis, 3 had combined metopic and sagittal synostoses, and 47 had unicoronal synostosis. The patients’ mean age at surgery was 11.4 ± 10.1 months and the mean operative time was 183.4 ± 41.0 minutes. The mean length of hospital stay was 3.7 ± 1.2 days. The mean blood loss was 150.0 ± 125.6 ml, and 33% of patients required a blood transfusion (mean volume 206.9 ± 102.3 ml). In metopic synostosis, hemiforeheads were used most often (24/29, 83%), and the supraorbital bar was remodeled using a bilateral intracranial orbital osteotomy followed by a double wedge modification (23/29, 79%) or a greenstick fracture (4/29 14%) for milder cases. Forehead remodeling for unicoronal synostosis was by a forehead switch (39/47, 83%) and the supraorbital bar was remodeled using a 1.5-orbital intracranial orbital osteotomy (34/47, 72%) such that the bar was advanced on the abnormal side and hinged at the midline of the normal orbit. Perioperative complications occurred in 19% of cases and included dural tears (16%), inconsequential subdural hematoma (1.3%), and nasal greenstick fracture (1.3%). The total reoperation rate was 7.6% (cranioplasties for irregular contours, 6.3%; scar revision, 1.3%). CONCLUSIONS The COB remodeling technique is simple and efficient, gives acceptable outcomes, and is less resource intensive than previous techniques reported in the literature.

Cerebellar astrocytomas (CA) are one of the most common posterior fossa tumors in children. The vast majority is low grade, and prognosis for long-term survival is excellent.Recent literature about CA was reviewed to provide an up to date overview of the epidemiology, pathology, molecular and cell biology, diagnosis, presentation, management, and long-term outcomes.Surgical resection remains the first-line treatment with complete removal of the tumor the goal. However, even when only subtotal resection has been achieved, there is a significant chance that the tumor will remain stable or will regress spontaneously. Adjuvant chemotherapy is reserved for those tumors that progress despite surgery, and more personalized chemotherapy is being pursued with better understanding of the molecular genetics of this tumor. Radiotherapy has generally not been recommended, but stereotactic radiotherapy and conformal proton beam radiotherapy may be reasonable options in the setting of relapse or progression. In the long term, permanent neurologic deficits, mainly cerebellar dysfunction, are common, but quality of life and cognitive function are generally good.Low-grade CA remains primarily a surgical disease, with excellent survival rates. Care must be taken with surgery and adjuvant treatments to preserve neurologic function to allow for optimal outcomes in the long term.