Development of an online calculator for the prediction of seizure freedom following pediatric hemispherectomy using the Hemispherectomy Outcome Prediction Scale (HOPS).

OBJECTIVES: Although hemispheric surgeries are among the most effective procedures for drug-resistant epilepsy (DRE) in the pediatric population, there is a large variability in seizure outcomes at the group level. A recently developed HOPS score provides individualized estimation of likelihood of seizure freedom to complement clinical judgement. The objective of this study was to develop a freely accessible online calculator that accurately predicts the probability of seizure freedom for any patient at 1-, 2-, and 5-years post-hemispherectomy. METHODS: Retrospective data of all pediatric patients with DRE and seizure outcome data from the original Hemispherectomy Outcome Prediction Scale (HOPS) study were included. The primary outcome of interest was time-to-seizure recurrence. A multivariate Cox proportional-hazards regression model was developed to predict the likelihood of post-hemispheric surgery seizure freedom at three time points (1-, 2- and 5- years) based on a combination of variables identified by clinical judgment and inferential statistics predictive of the primary outcome. The final model from this study was encoded in a publicly accessible online calculator on the International Network for Epilepsy Surgery and Treatment (iNEST) website (https://hops-calculator.com/). RESULTS: The selected variables for inclusion in the final model included the five original HOPS variables (age at seizure onset, etiologic substrate, seizure semiology, prior non-hemispheric resective surgery, and contralateral fluorodeoxyglucose-positron emission tomography [FDG-PET] hypometabolism) and three additional variables (age at surgery, history of infantile spasms, and magnetic resonance imaging [MRI] lesion). Predictors of shorter time-to-seizure recurrence included younger age at seizure onset, prior resective surgery, generalized seizure semiology, FDG-PET hypometabolism contralateral to the side of surgery, contralateral MRI lesion, non-lesional MRI, non-stroke etiologies, and a history of infantile spasms. The area under the curve (AUC) of the final model was 73.0%. SIGNIFICANCE: Online calculators are useful, cost-free tools that can assist physicians in risk estimation and inform joint decision-making processes with patients and families, potentially leading to greater satisfaction. Although the HOPS data was validated in the original analysis, the authors encourage external validation of this new calculator.

Patient-reported outcome measures more than fifteen years after treatment of sagittal or metopic craniosynostosis: a prospective cohort study.

PURPOSE: To evaluate the long-term anthropometric measurements, cosmetic satisfaction, and other patient-reported outcome measures (PROMs) of patients who underwent surgical treatment or observation only of sagittal or metopic single-suture craniosynostosis (SSC). METHODS: A prospective study was designed for all patients diagnosed with non-syndromic sagittal and metopic craniosynostosis at the British Columbia Children's Hospital, Vancouver, Canada, in the period July 1986 to July 2006. After a minimum of 15 years post-diagnosis, all eligible patients were invited to fill out the Craniofacial Surgery Outcomes Questionnaire (CSO-Q) and to attend a scheduled follow-up appointment for the collection of anthropometric measurements. A descriptive analysis of the cosmetic results was performed. Statistical analyses compared the differences in anthropometric measurements between treated and non-treated patients. RESULTS: Of the 253 eligible patients, 52 participants were willing to share patient data for use in the study. Of those 52 former patients, 36 (69.2%) filled out and returned the CSO-Q and 23 (44.2%) attended the follow-up appointment. The mean follow-up period between surgical treatment and the CSO-Q was 20.2 ± 2.5 years and between surgical treatment and the follow-up appointment was 20.9 ± 2.7 years. In patients with sagittal SSC, the mean cephalic index (CI) was significantly larger in treated than in non-treated patients (74.6 versus 69.1, p = 0.04), while the mean pupillary distance and forehead to back index were significantly smaller (pupillary distance 6.0 cm versus 6.7 cm [p = 0.04] and forehead to back index 19.6 cm versus 21.1 cm [p = 0.03]). Focusing more on the patient reported outcome measures, overall cosmetic satisfaction was found to be high (80.6%) and no differences were found between sagittal and metopic synostosis patients, nor between treated or non-treated craniosynostosis patients. Overall outcomes regarding self-esteem (RSES) and fear of negative evaluation (FNE) were comparable with population based outcomes. CONCLUSION: This is the first prospective study of sagittal and metopic craniosynostosis patients regarding long-term anthropometric outcome and patient reported outcome measures, including patients who were treated surgically and those who received observation only. Although study participation two decades after initial diagnosis was difficult to obtain, our data provide a platform from which one can develop an inclusive and uniform approach to assess patients' subjective cosmetic satisfaction using the CSO-Questionnaire and might be useful in preoperative counseling and psychosocial care for patients and their families.

Global trends and decision-making in the management of arachnoid cysts.

OBJECTIVE: In pediatric patients, middle cranial fossa (MCF) arachnoid cysts are often discovered incidentally on imaging in asymptomatic patients during workup for other indications. This study aims to describe current management gestalt and threshold for surgical intervention by surveying an international cohort of neurosurgeons. METHODS: A web-based survey was circulated via email list of attendants of the 2019 Canadian Pediatric Neurosurgery Study Group (CPNSG) and International Society of Pediatric Neurosurgery (ISPN) mailing list. The survey consisted of 8 clinical scenarios involving patients with MCF arachnoid cysts. Demographic variables of respondents and their decisions regarding management for each scenario were analyzed using R computing software. RESULTS: A total of 107 respondents were included. Cysts in asymptomatic patients (92%), younger age at diagnosis (81%), and presence of a mild learning delay were predominantly managed non-surgically (80.7 ± 9.4%). Patients with cyst enlargement, headaches, new seizures, or hemorrhage were divided between non-surgical (55.8 ± 3.3%) and surgical (44.2 ± 2.9%) management. Patients with contralateral hemiparesis were treated predominantly surgically (67%). For both Galassi I and II, papilledema was favored as the primary indication for surgical intervention in 54% of patients. Those inclined to surgery (n = 17) were more likely to practice and train outside North America compared to those not pro-surgical (adjusted P = 0.092). CONCLUSION: Incidental MCF arachnoid cysts in asymptomatic patients and younger age of diagnosis are predominantly managed non-surgically. Mild learning delay was not considered an indication to intervene. In contrast, radiological progression, hemorrhagic evolution, or non-focal neurological deficits lead to uncertainty in management, while focal neurological deficits and papilledema with MCF cysts were favored to be intervened surgically. Among the provider level factors, only location of training and practice trended towards a pro-surgery approach.

The Effect of Selective Dorsal Rhizotomy on Hip Displacement in Children With Cerebral Palsy: A Long-term Follow-up Study.

BACKGROUND: Hip displacement is common in children with cerebral palsy (CP). Spasticity in the hip adductor muscles, hip flexors, and medial hamstrings has been identified as a possible cause of progressive hip displacement. Selective dorsal rhizotomy (SDR) aims to reduce lower extremity spasticity in children with CP. Here, we investigate the influence of SDR on hip displacement in children with CP at long-term follow-up, a minimum of 5 years post-SDR. METHODS: A retrospective review of children undergoing SDR at a Canadian pediatric hospital was completed. Migration percentage (MP) was measured on pelvis radiographs taken in the 6 months before SDR and minimum 5 years post-SDR or before hip surgery. The number of hips with displacement, defined as MP >30%, and the number of children with at least 1 hip displaced were determined. A linear mixed-effects model was used to assess potential risk factors for poor outcome post-SDR, defined as having MP >40% or surgical intervention for hip displacement. RESULTS: Ninety children [50 males, 40 females, Gross Motor Function Classification System (GMFCS) levels I to V: 1/13/24/43/9] with a mean follow-up of 8.5 years (SD 5.1) were included. The mean age at SDR was 4.9 years (SD 1.5); more than half of children (52%) had hip displacement at the time of SDR. Post-SDR, MP exceeded 30% in 0 (0%) of children at GMFCS level I, 1 (8%) at II, 11 (46%) at III, 31 (72%) at IV, and 7 (78%) at V. A poor outcome was associated with preoperative MP, age, and GMFCS level. CONCLUSIONS: The incidence of hip displacement post-SDR was consistent with population-based studies when evaluated by GMFCS. Our findings suggest that SDR has neither a positive nor negative effect on hip displacement when assessed at least 5 years postintervention. LEVEL OF EVIDENCE: Level IV.

Hemispherectomy Outcome Prediction Scale: Development and validation of a seizure freedom prediction tool.

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.

Comparison of the real-world effectiveness of vertical versus lateral functional hemispherotomy techniques for pediatric drug-resistant epilepsy: A post hoc analysis of the HOPS study.

OBJECTIVE: This study was undertaken to determine whether the vertical parasagittal approach or the lateral peri-insular/peri-Sylvian approach to hemispheric surgery is the superior technique in achieving long-term seizure freedom. METHODS: We conducted a post hoc subgroup analysis of the HOPS (Hemispheric Surgery Outcome Prediction Scale) study, an international, multicenter, retrospective cohort study that identified predictors of seizure freedom through logistic regression modeling. Only patients undergoing vertical parasagittal, lateral peri-insular/peri-Sylvian, or lateral trans-Sylvian hemispherotomy were included in this post hoc analysis. Differences in seizure freedom rates were assessed using a time-to-event method and calculated using the Kaplan-Meier survival method. RESULTS: Data for 672 participants across 23 centers were collected on the specific hemispherotomy approach. Of these, 72 (10.7%) underwent vertical parasagittal hemispherotomy and 600 (89.3%) underwent lateral peri-insular/peri-Sylvian or trans-Sylvian hemispherotomy. Seizure freedom was obtained in 62.4% (95% confidence interval [CI] = 53.5%-70.2%) of the entire cohort at 10-year follow-up. Seizure freedom was 88.8% (95% CI = 78.9%-94.3%) at 1-year follow-up and persisted at 85.5% (95% CI = 74.7%-92.0%) across 5- and 10-year follow-up in the vertical subgroup. In contrast, seizure freedom decreased from 89.2% (95% CI = 86.3%-91.5%) at 1-year to 72.1% (95% CI = 66.9%-76.7%) at 5-year to 57.2% (95% CI = 46.6%-66.4%) at 10-year follow-up for the lateral subgroup. Log-rank test found that vertical hemispherotomy was associated with durable seizure-free progression compared to the lateral approach (p = .01). Patients undergoing the lateral hemispherotomy technique had a shorter time-to-seizure recurrence (hazard ratio = 2.56, 95% CI = 1.08-6.04, p = .03) and increased seizure recurrence odds (odds ratio = 3.67, 95% CI = 1.05-12.86, p = .04) compared to those undergoing the vertical hemispherotomy technique. SIGNIFICANCE: This pilot study demonstrated more durable seizure freedom of the vertical technique compared to lateral hemispherotomy techniques. Further studies, such as prospective expertise-based observational studies or a randomized clinical trial, are required to determine whether a vertical approach to hemispheric surgery provides superior long-term seizure outcomes.

Long-term upper extremity performance in children with cerebral palsy following selective dorsal rhizotomy.

PURPOSE: 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. METHODS: 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). RESULTS: 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. CONCLUSION: Early improvements in upper extremity QUEST total scores are sustained in the long term following SDR.

Immediate replacement of bone flap after craniotomy for empyema in children.

PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Long term outcome of Selective Dorsal Rhizotomy for the management of childhood spasticity-functional improvement and complications.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Safety and efficacy of independent allied healthcare professionals in the assessment and management of plagiocephaly 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.

Systematic review of spinal deformities following multi-level selective dorsal rhizotomy.

PURPOSE: 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. METHODS: 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. RESULTS: 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 < 0.01, and between scoliosis and the number of years to follow-up, p < 0.01. CONCLUSION: Spinal deformity is an important potential complication of SDR with scoliosis being the most common type of deformity. The major risk factor for postoperative deformity is female sex. Deformity was also found to significantly increase with extended follow-up, indicating a slow process that should be carefully monitored.

Cerebellar mutism after posterior fossa tumor resection in children: a multicenter international retrospective study to determine possible modifiable factors.

PURPOSE: 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. METHODS: 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. RESULTS: 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 < 1 to 17 years). The overall incidence of postoperative CM was 23.5% (range 14.7-47.6% for centers with data on ≥ 20 patients). The overall incidence of CMS was 6.5% (range 0-10.3% for centers contributing data on ≥ 20 patients). A multivariate logistic regression on the full data set showed no significant association between pre-resection surgical hydrocephalus treatment, prone position, ultrasonic aspirator use, EVD use, telovelar approach, complete or near total resection, or treating center and either postoperative CM or CMS. CONCLUSIONS: While there was variation in surgical management of midline PF tumors among centers participating in this study, the factors in management that were examined did not predict postoperative CM or CMS.

Outcomes and complications for individual neurosurgeons for the treatment of Chiari I malformation at a children's hospital.

PURPOSE: 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. METHODS: 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. RESULTS: 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%. CONCLUSION: 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.

Craniotomy bone flap fixation: revisiting the use of bone struts.

INTRODUCTION: Bone flap fixation after craniotomy is a standard part of neurosurgical practice. Several techniques and devices exist, though no ideal strategy has been identified. The key aims are to prevent infection and to achieve adequate cosmesis and bony fusion whilst also minimising costs and complications. Ease of use must also be considered. Fixation with sutures and bony struts in the kerf has been described in children and adults and, although the technique achieves many of the ideals of fixation, it does not seem to have been popularised. We report our experience of using the strut technique. METHODS: A retrospective review of our cranial surgery database, operative notes and follow-up records was conducted. 300 applicable craniotomies were carried out in 8 years. Struts were used in 81 cases and comments on the bony contour described in 21 follow-up records. RESULTS: In nineteen, the contour was perfect. In one, there was a small bony depression; and in one, there was a small ridge in the posterior part. No repeat operations were carried out for surgery or cosmesis. CONCLUSIONS: We report our results with a view to reminding the neurosurgical community of the existence of a technique that achieves all the criteria of the ideal fixation strategy.

International survey on the management of Chiari 1 malformation and syringomyelia: evolving worldwide opinions.

INTRODUCTION: 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. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Osteogenic sarcoma of the skull: long-term outcome of a rare tumor.

BACKGROUND: 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. CASE DESCRIPTION: 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.

Trends in incidence and long-term outcomes of myelomeningocele in British Columbia.

PURPOSE: 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). METHODS: 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. RESULTS: 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 < 0.0001)]. For surviving children, the proportion of children attending special classes was significantly different between the earlier and later cohorts [16 vs 46%, respectively (p = 0.0002)], whereas all other outcome measures, including the proportion with hydrocephalus, kyphoscoliosis, Chiari II surgery, bowel and bladder continence, recreation participation, obesity, and ambulation, were not significantly different. CONCLUSIONS: In BC, the incidence of new cases of MMC has decreased between 1971 and 2016, while the probability of survival for these patients has increased. Despite earlier and more universal post-natal repair, long-term outcomes have not improved significantly over time. Future research should focus on developing ways of reducing disability and improving quality of life for MMC patients and their families.

Manifestations of Tuberous Sclerosis Complex: The Experience of a Provincial Clinic.

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.

Filum Section for Urinary Incontinence in Children with Occult Tethered Cord Syndrome: A Randomized, Controlled Pilot Study.

PURPOSE: Occult tethered cord syndrome, in which there is normal neuroanatomic imaging despite clinical and urodynamic evidence of neuropathic bladder behavior, is controversial. Several uncontrolled series describe improvement in bladder function following section of the filum terminale. We performed a pilot randomized, controlled study comparing medical treatment to surgical section of the filum plus medical treatment in children with occult tethered cord syndrome. MATERIALS AND METHODS: Children refractory to standard medical management for 1 year or more with normal conus position on magnetic resonance imaging and abnormal urodynamics were randomized. Exclusion criteria included any neurological conditions, spinal dysraphism, bladder outlet obstruction and an atonic bladder. Patients were assessed at randomization and 1 year later with a standardized urodynamic score, the validated PEMQOL (Pediatric Enuresis Module on Quality of Life™) scale, and a validated bowel and bladder dysfunction score. RESULTS: After 8 years we accrued 21 patients. The bowel and bladder dysfunction score improved in the surgical and medical arms (20% and 24%) and the urodynamic score improved slightly (6% and 4%, respectively). The PEMQOL Child and Family Impact Scales improved modestly in both groups. All differences were nonsignificant. Interim analysis indicated that more than 700 patients in each arm would be required to demonstrate a statistical difference with respect to urodynamic score based on our preliminary data. CONCLUSIONS: There appears to be no objective difference in urological outcome between medical management plus or minus filum section for patients with occult tethered cord syndrome. These data challenge the existence of the concept of occult tethered cord syndrome, in which bowel and bladder dysfunction score is attributed to tethering by the filum despite a normally located conus.