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Background: Spinal cord injury without radiographic abnormality (SCIWORA) represents a traumatic myelopathy, either transient or permanent, that is not associated with visible vertebral fractures or ligamentous abnormalities on plain radiographs or CT. MRI has become essential in the diagnosis and evaluation of trauma patients and in predicting the long-term neurological outcome. Methods: The medical charts of 578 children with vertebral trauma at the Children's Hospital of Eastern Ontario and the Hospital for Sick Children were retrospectively reviewed. Results: Forty-five patients were identified as having traumatic myelopathy. Three of these patients had SCIWORA. Two sustained thoracic spinal cord injuries as a result of motor vehicle accidents and permanent neurological deficits. The third was involved in a sports-related injury and sustained a cervical spine injury that improved in 48 h. Conclusions: SCIWORA is uncommon. The serious neurological sequelae that can result from a missed diagnosis merit more attention in identifying a spinal lesion in children with traumatic myelopathy. MRI has played a valuable role in this respect and may be even more predictive of outcome than the presenting neurological findings. The SCIWORA acronym can be modified to SCIWONA (spinal cord injury without neuroimaging abnormality) in order to highlight the importance of MRI in the prognosis. Copyright © 2010 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Purpose: To describe and explore the relationship between baseline anxiety, depression and symptom experience in children and youth assessed at a concussion clinic., Design and Methods: A retrospective chart review of concussed children and youth referred to a pediatric teaching hospital concussion clinic over a 15-month period was completed. Kutcher Adolescent Depression Scale (KAD-6), General Anxiety Disorder Scale (GAD-7), and the Post-Concussion Symptom Inventory (PCSI) scores were extracted., Results: A total of 155 patients were included. The most common symptoms (PCSI) at baseline were headache, fatigue, and feelings of head pressure. Symptoms were rated as mild to moderate in intensity. Overall, mean depression and anxiety scores were low. The mean anxiety scores, as measured by the GAD-7 (n = 108), was 7.4 (range, 0-24). The mean depression score, as measured by the KAD-6 (n = 94), was 4.7 (range, 0-18). A statistically significant, moderate positive correlation between PCSI scores with KAD-6 scores for male (r = .64, p < .001) and female (r = .61, p < .001) participants was identified. Similarly, a statistically significant, moderate positive correlation between PCSI scores with GAD-7 scores for male (r = .68, p < .001) and female (r = .60, p < .001) participants was identified., Practice Implications: Concussed children may experience a wide array of symptoms, including emotional challenges such as anxiety and depression. Feelings of anxiety and depression may contribute to overall post concussive symptoms in concussed children. The electronic health record can be leveraged to provide important patient data. Clinicians should systematically assess symptoms at each visit in concussed children and youth so that appropriate interventions can be implemented and monitored., (© 2020 Wiley Periodicals LLC.)

Compared to adults, it has been documented that children are at elevated risk for concussion, repeated concussions, and experience longer recovery times. What is unknown, is whether the developing brain may be injured at differing strain levels. This study examined peak and cumulative brain strain from 20 cases of concussion in both young children and adults using physical reconstructions and finite element modelling of the brain response to impacts. The child group showed lower impact kinematics as well as strain metrics. Results suggest children may suffer concussive injuries with lower brain strains compared to adults.

Objective: Complications following pediatric cranioplasty after craniectomy with either autologous bone flaps or cranial implants are reported to be common, particularly bone flap resorption. However, only sparse data are available regarding cranioplasty strategies, complications, and outcomes. This manuscript describes a Canadian-Dutch multicenter pediatric cohort study with autografts and cranial implant cranioplasties following craniectomies for a variety of indications., Methods: The study included all children (< 18 years) who underwent craniectomy and subsequent cranioplasty surgeries from 2008 to 2014 (with a minimum of 1-year follow-up) at four academic hospitals with a dedicated pediatric neurosurgical service. Data were collected regarding initial diagnosis, age, time interval between craniectomy and cranioplasty, bone flap storage method, type of cranioplasty for initial procedure (and redo if applicable), and the postoperative outcome including surgical site infection, wound breakdowns, bone flap resorption, and inadequate fit/disfigurement., Results: Sixty-four patients (46 males, average age 9.7 ± 5.5 years) were eligible for inclusion, with mean follow-up of 82.3 ± 31.2 months after craniectomy. Forty cranioplasties (62.5%) used autologous bone re-implant, 23 (57.5%) of which showed resorption. On average, resorption was documented at 434 days (range 62-2796 days) after reimplantation. In 20 cases, a revision cranioplasty was needed. In 24 of the post-craniectomy cases (37.5%), a cranial implant was used with one of ten different implant types. Implant loosening prompted a complete revision cranioplasty in 2 cases (8.3%). Cranial implants were associated with low morbidity and lower reoperation dates compared to the autologous cranioplasties., Conclusion: The most prominent finding in this multicenter cohort study was that bone flap resorption in children remains a common and widespread problem following craniectomy. Cranioplasty strategies varied between centers and evolved over time within centers. Cranial implants were associated with low morbidity and low reoperation rates. Still, longer term and prospective multicenter cohort studies are needed to optimize cranioplasty strategies in children after craniectomies.

The identification of clinical phenotypes may help parse the substantial heterogeneity that characterizes children with concussion. This study used latent class analysis (LCA) to identify discernible phenotypes among children with acute concussion and examine the association between phenotypes and persistent post-concussive symptoms (PPCS) at 4 and 12 weeks post-injury. We conducted LCA of variables representing pre-injury history, clinical presentation, and parent symptom ratings, derived from a prospective cohort, observational study that recruited participants from August 2013 until June 2015 at nine pediatric emergency departments within the Pediatric Emergency Research Canada network. This substudy included 2323 children from the original cohort ages 8.00-17.99 years who had data for at least 80% of all variables included in each LCA. Concussion was defined according to Zurich consensus statement diagnostic criteria. The primary outcome was PPCS at 4 and 12 weeks after enrollment. Participants were 39.5% female and had a mean age of 12.8 years (standard deviation = 2.6). Follow-up was completed by 1980 (85%) at 4 weeks and 1744 (75%) at 12 weeks. LCA identified four groups with discrete pre-injury histories, four groups with discrete clinical presentations, and seven groups with discrete profiles of acute symptoms. Clinical phenotypes based on the profile of group membership across the three LCAs varied significantly in their predicted probability of PPCS at 4 and 12 weeks. The results indicate that children with concussion can be grouped into distinct clinical phenotypes, based on pre-injury history, clinical presentation, and acute symptoms, with markedly different risks of PPCS. With further validation, clinical phenotypes may provide a useful heuristic for clinical assessment and management.

Infantile myofibroma is a rare benign mesenchymal tumor that presents as solitary or multiple lesions (myofibromatosis) in the skin, soft tissue, bone, or internal organs. It most commonly affects the head and neck of infants and young children, but it can also affect adults. Intracranial involvement is reported to be extremely rare, and its clinical picture has been poorly characterized. Recently, it has been demonstrated that germline and somatic mutations in the platelet-derived growth factor receptor beta (PDGFRB) are associated with familial infantile myofibromatosis. We report a case of infantile myofibromatosis with predominant posterior fossa extradural involvement in a 14-year-old adolescent girl with a confirmed mutation in the PDGFRB gene.

Second impact syndrome (SIS) is associated with malignant brain swelling and usually occurs in young athletes with one or more prior, recent concussions. SIS is rare and some dispute its existence. We report a case of SIS in Rowan Stringer, age 17, a rugby player who sustained a fatal brain injury despite prompt medical therapy including decompression surgery. The cause of the massive brain swelling was initially unknown. An inquest revealed Rowan's text messages to friends describing symptoms from two prior, recent rugby brain injuries, likely concussions, within 5 days of the fatal blow and confirming the diagnosis of SIS.

Radiation-associated vascular changes most commonly present in the form of stenosis, thrombosis and occlusion. However, development of intracranial aneurysms secondary to radiation is far less common and often manifests with rupture. These aneurysms are difficult to treat and associated with high morbidity and mortality when ruptured compared with saccular aneurysms unrelated to radiation treatment. Both surgical and endovascular options are available for treatment of these aneurysms. We present a young patient with a radiation-induced intracranial pseudoaneurysm arising from the lenticulostriate branch of the left middle cerebral artery (MCA); this developed 1 year 4 months after 59.4 Gy of focused radiation to the suprasellar pilomyxoid astrocytoma. The patient successfully underwent endovascular glue embolization of the aneurysm and occlusion of the lenticulostriate artery after unsuccessful trapping of the aneurysm and occlusion of the parent artery using coils. She developed transient hemiparesis of the right side following the procedure, which was managed conservatively. We performed a complete review of the literature dealing with the radiation-induced intracranial aneurysms, their presentation, treatment and outcome.

OBJECTIVE In children, the repair of skull defects arising from decompressive craniectomy presents a unique set of challenges. Single-center studies have identified different risk factors for the common complications of cranioplasty resorption and infection. The goal of the present study was to determine the risk factors for bone resorption and infection after pediatric cranioplasty. METHODS The authors conducted a multicenter retrospective case study that included all patients who underwent cranioplasty to correct a skull defect arising from a decompressive craniectomy at 13 centers between 2000 and 2011 and were less than 19 years old at the time of cranioplasty. Prior systematic review of the literature along with expert opinion guided the selection of variables to be collected. These included: indication for craniectomy; history of abusive head trauma; method of bone storage; method of bone fixation; use of drains; size of bone graft; presence of other implants, including ventriculoperitoneal (VP) shunt; presence of fluid collections; age at craniectomy; and time between craniectomy and cranioplasty. RESULTS A total of 359 patients met the inclusion criteria. The patients' mean age was 8.4 years, and 51.5% were female. Thirty-eight cases (10.5%) were complicated by infection. In multivariate analysis, presence of a cranial implant (primarily VP shunt) (OR 2.41, 95% CI 1.17-4.98), presence of gastrostomy (OR 2.44, 95% CI 1.03-5.79), and ventilator dependence (OR 8.45, 95% CI 1.10-65.08) were significant risk factors for cranioplasty infection. No other variable was associated with infection. Of the 240 patients who underwent a cranioplasty with bone graft, 21.7% showed bone resorption significant enough to warrant repeat surgical intervention. The most important predictor of cranioplasty bone resorption was age at the time of cranioplasty. For every month of increased age the risk of bone flap resorption decreased by 1% (OR 0.99, 95% CI 0.98-0.99, p < 0.001). Other risk factors for resorption in multivariate models were the use of external ventricular drains and lumbar shunts. CONCLUSIONS This is the largest study of pediatric cranioplasty outcomes performed to date. Analysis included variables found to be significant in previous retrospective reports. Presence of a cranial implant such as VP shunt is the most significant risk factor for cranioplasty infection, whereas younger age at cranioplasty is the dominant risk factor for bone resorption.

Background: Hemifacial spasms are involuntary contractions of the muscles of one side of the face., Case Report: Here, we report a 5-year-old female patient with a significant past medical history, including mucopolysaccharidosis type VI, who also presented with hemifacial spasms. Further investigations showed narrowing of skull foramina and hydrocephalus, but no compression of the facial nerve. Carbamazepine was given to the patient, which caused cessation of the spasms., Discussion: Currently, there is no literature discussing the relationship between hemifacial spasms and mucopolysaccharidosis type VI; we have proposed that mucopolysaccharide deposits or dural thickening may contribute to nerve irritation., Competing Interests: Funding: None. Conflict of Interests: The authors report no conflict of interest. Ethical Statement: This study was reviewed by the authors’ institutional ethics committee and was considered exempted from further review.

Background: With a reported incidence of up to 10% compared to all spinal trauma, spinal injuries in children are less common than in adults. Children can have spine fractures with or without myelopathy, or spinal cord injuries without radiological abnormalities (SCIWORA)., Methods: We retrospectively reviewed the cases of children with spinal injuries treated at a level 1 pediatric trauma centre between 1990 and 2013., Results: A total of 275 children were treated during the study period. The mean age at admission was 12 ± 4.5 years, and the male:female ratio was 1.4:1. Spinal injuries were more common in children of ages 12-16 years, with most injuries among ages 15-16 years. The top 3 mechanisms of spinal injury were motor vehicle-related trauma (53%), sports (28%) and falls (13%). Myelopathy occurred in 12% and SCIWORA occurred in 6%. The most common spine levels injured were L2-sacrum, followed by O-C2. Associated injuries, including head injuries (29%), and fractures/dislocations (27%) occurred in 55% of children. Overall mortality was 3%. Surgical intervention was required in 14%., Conclusion: The creation of a pediatric spinal injury database using this 23-year retrospective review helped identify important clinical concepts; we found that active adolescent boys had the highest risk of spine injury, that noncontiguous spine injuries occured at a rate higher than reported previously and that nonaccidental spine injuries in children are underreported. Our findings also emphasize the importance of maintaining a higher index of suspicion with trauma patients with multiple injuries and of conducting detailed clinical and radiographic examinations of the entire spine in children with a known spinal injury.

Importance: Approximately one-third of children experiencing acute concussion experience ongoing somatic, cognitive, and psychological or behavioral symptoms, referred to as persistent postconcussion symptoms (PPCS). However, validated and pragmatic tools enabling clinicians to identify patients at risk for PPCS do not exist., Objective: To derive and validate a clinical risk score for PPCS among children presenting to the emergency department., Design, Setting, and Participants: Prospective, multicenter cohort study (Predicting and Preventing Postconcussive Problems in Pediatrics [5P]) enrolled young patients (aged 5-<18 years) who presented within 48 hours of an acute head injury at 1 of 9 pediatric emergency departments within the Pediatric Emergency Research Canada (PERC) network from August 2013 through September 2014 (derivation cohort) and from October 2014 through June 2015 (validation cohort). Participants completed follow-up 28 days after the injury., Exposures: All eligible patients had concussions consistent with the Zurich consensus diagnostic criteria., Main Outcomes and Measures: The primary outcome was PPCS risk score at 28 days, which was defined as 3 or more new or worsening symptoms using the patient-reported Postconcussion Symptom Inventory compared with recalled state of being prior to the injury., Results: In total, 3063 patients (median age, 12.0 years [interquartile range, 9.2-14.6 years]; 1205 [39.3%] girls) were enrolled (n = 2006 in the derivation cohort; n = 1057 in the validation cohort) and 2584 of whom (n = 1701 [85%] in the derivation cohort; n = 883 [84%] in the validation cohort) completed follow-up at 28 days after the injury. Persistent postconcussion symptoms were present in 801 patients (31.0%) (n = 510 [30.0%] in the derivation cohort and n = 291 [33.0%] in the validation cohort). The 12-point PPCS risk score model for the derivation cohort included the variables of female sex, age of 13 years or older, physician-diagnosed migraine history, prior concussion with symptoms lasting longer than 1 week, headache, sensitivity to noise, fatigue, answering questions slowly, and 4 or more errors on the Balance Error Scoring System tandem stance. The area under the curve was 0.71 (95% CI, 0.69-0.74) for the derivation cohort and 0.68 (95% CI, 0.65-0.72) for the validation cohort., Conclusions and Relevance: A clinical risk score developed among children presenting to the emergency department with concussion and head injury within the previous 48 hours had modest discrimination to stratify PPCS risk at 28 days. Before this score is adopted in clinical practice, further research is needed for external validation, assessment of accuracy in an office setting, and determination of clinical utility.

Background: 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., Methods: 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., Results: 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., Conclusion: 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.

Background: Sports are a major cause of concussions, and little is known about the symptom experience and health-related quality of life (HRQL) in children who remain symptomatic for over 3 months following such head injuries., Methods: A cross-sectional study of children aged 10-18 years was performed who were referred to the Neurosurgery Clinic at our centre following a head injury. Symptom experience was measured using the modified Concussion Symptom Scale, and HRQL was measured using the Pediatric Quality of Life Inventory (PedsQL). The Immediate Postconcussion Assessment and Cognitive Test (ImPACT) was administered to assess neurocognitive and neurobehavioural sequelae., Results: Symptoms with the highest mean symptom scores on a Likert scale of 0-6 in 35 children at the time of assessment included headaches (3.1), poor concentration (2.7), memory problems (2.1), fatigue (2.1) and sensitivity to noise (2.0). Compared with normative data, children in this study had ImPACT summary scores between the 28th and 38th percentiles and a comparably low Cognitive Efficiency Index score. Mean scores for females were consistently statistically significantly lower (p < 0.05) than for males across all of the HRQL domains. Trouble falling asleep and memory problems explained 62% of the variance in the PedsQL total scores., Conclusions: Children continue to experience many symptoms at least 3 months following sport-related head injuries that significantly impact their HRQL and neurocognitive abilities., (© 2015 S. Karger AG, Basel.)

Objective: Like many sports involving high speeds and body contact, head injuries are a concern for short track speed skating athletes and coaches. While the mandatory use of helmets has managed to nearly eliminate catastrophic head injuries such as skull fractures and cerebral haemorrhages, they may not be as effective at reducing the risk of a concussion. The purpose of this study was to evaluate the performance characteristics of speed skating helmets with respect to managing peak linear and peak rotational acceleration, and to compare their performance against other types of helmets commonly worn within the speed skating sport., Materials and Methods: Commercially available speed skating, bicycle and ice hockey helmets were evaluated using a three-impact condition test protocol at an impact velocity of 4 m/s., Results and Discussion: Two speed skating helmet models yielded mean peak linear accelerations at a low-estimated probability range for sustaining a concussion for all three impact conditions. Conversely, the resulting mean peak rotational acceleration values were all found close to the high end of a probability range for sustaining a concussion. A similar tendency was observed for the bicycle and ice hockey helmets under the same impact conditions., Conclusion: Speed skating helmets may not be as effective at managing rotational acceleration and therefore may not successfully protect the user against risks associated with concussion injuries.

Objectives: Anxiety modulates symptom perception in adults following concussion, prolonging the time to full recovery. The authors sought to determine whether parental anxiety was associated with persistent postconcussive symptoms (PCS) in their children following concussion., Methods: A prospective observational cohort with 98 children aged 5 to 17 years following concussion participated from a tertiary pediatric emergency department (ED). The main exposure was parental anxiety at the time of acute presentation following pediatric concussion, measured using the self-administered, validated Spielberger State-Trait Anxiety Inventory-State Anxiety Scale (STAI-S). The primary outcome measured was presence of PCS in the child at 1 month, per the validated Post-Concussive Symptom Inventory (PCSI). Secondary outcome measures included parental anxiety score over time, school absenteeism, and return to sports. Data were collected during the initial ED visit and at 3-day, 7-day, 2-week, 1-month, and 3-month follow-ups., Results: Of 98 children enrolled, 27% (95% confidence interval [CI] = 19% to 36%) developed PCS at 1 month. No significant associations were detected between parental anxiety at the index visits and the number of previous pediatric concussions (p = 0.73), sex (p = 0.61), loss of consciousness (p = 0.43), history of migraines (p = 0.31), or history of anxiety diagnosed in the patients (p = 0.09). A significant association was noted between patient diagnosis of attention deficit hyperactivity disorder (ADHD) and parental anxiety at the index visits (p = 0.001). Parental anxiety at acute presentation was not associated with children's prolonged symptoms at 1 month (p = 0.63). Parental anxiety remained elevated in parents whose children had prolonged symptoms compared to those parents whose children's symptoms resolved (median = 30, interquartile range [IQR] = 22 to 44; and median = 21, IQR = 20 to 25, respectively; p < 0.001). Initial parental anxiety presentation was not associated with school absenteeism (p = 0.23) or not returning to sport or gym class (p = 0.89). There were no significant effects involving ADHD alone (p = 0.44) or together with baseline parental anxiety (p = 0.36 for ADHD and p = 0.55 for anxiety) using logistic regression analysis to examine potential predictive effects of child's ADHD combined with parental anxiety at the index visit on persistent symptoms at 1 month., Conclusions: Parental anxiety at time of acute presentation does not appear to be associated with prolongation of postconcussive symptoms in their children. However, parents of persistently symptomatic children remain significantly more anxious than those whose children's symptoms have resolved. Future research should attempt to reduce the familial burden of concussion through expectation management strategies., (© 2013 by the Society for Academic Emergency Medicine.)

Purpose: Positional plagiocephaly is an acquired deformation of an intrinsically normal infant skull by sustained or excessive extrinsic forces. Non-surgical techniques include counter-positioning, supervised prone time and orthotic molding for more refractory cases. Long-term effects of positional plagiocephaly on development remain undefined, and this study evaluated cosmetic and cognitive outcomes of plagiocephaly management., Method: Surveys were administered to parents of patients treated for positional plagiocephaly through the Children's Hospital of Eastern Ontario. Categorical responses interrogated cosmetic outcome, school performance, language skills, cognitive development and societal function. Pearson coefficient analysis tested outcomes dependency on gender, age, and plagiocephaly side at the 0.05 level of significance., Results: Eighty respondents (51 male, 29 female) were divided as 58 right- and 22 left-sided pathology. Positional therapy was uniformly applied, and a helmet orthosis was utilized in 36% of cases. Median follow-up age was nine years with normal head appearance in 75% of cases. Only 4% of parents and 9% of patients observed significant residual asymmetry. These results did not vary by gender, age or deformity side. Left-sided disease predicted poorer language development and academic performance. Expressive speech abnormality occurred in twice as many patients with left-sided disease (36% versus 16%, p=0.04) along with three-fold greater special education requirements (27% versus 10%, p=0.04)., Conclusions: Non-surgical plagiocephaly management achieved good cosmetic outcome among patients in this study. Children with left-sided disease frequently encountered difficulties with cognitive and scholastic endeavors, although the roles of the underlying disease and the treatment measures in this delay cannot be differentiated.

Object: The purpose of this study was to evaluate how currently used helmets would perform for winter play activities, such as tobogganing. In Canada and northern parts of the US, the advent of winter is followed by an increase in visits to hospital emergency departments by young children presenting with head injuries resulting from winter activities. Sliding, skating, skiing, and snowboarding all involve risks of head injury from situations such as falling on ice or sliding into stationary objects. This study compared the protective characteristics of helmets used by young children (< 7 years of age) participating in winter recreational activities., Methods: Ice hockey, alpine ski, and bicycling helmets were impacted at 2.0, 4.0, 6.0, and 8.0 m/second at the front and side impact location by using a monorail drop rig., Results: The results for the front impact showed that the ice hockey helmet protected the child significantly better at 2 and 4 m/second when considering both linear and angular peak acceleration. The bicycle helmet performed significantly better than the other 2 helmets at 8 m/second for the front location and only angularly for the side impacts., Conclusions: Depending on the impact velocity of the hazard, the type of helmet significantly affected the risk of brain injury.

Background: The ability to provide an accurate prognosis for children with traumatic brain injury (TBI) would be useful for the children's families and the caregivers. In this study we examined whether an appropriate mathematical model can predict survival in this patient population., Methods: Data from the Children's Hospital of Eastern Ontario (CHEO) TBI registry was analyzed. First, a series of univariate logistic regressions was performed to ascertain the significance of individual predictors, such as age, maximum Glasgow Coma Scale (GCS) score, maximum head injury Abbreviated Injury Scores (AIS) and the Injury Severity Score (ISS). Second, a multinomial logistic regression was fitted using only individually significant predictors and inmodel predictor significance, and interactions were tested. Only two significant predictors were kept in the final model. This final model was subsequently used to predict survival for each individual patient using the n-1 training set (i.e. Lachenbruch's leave-one-out method). The receiver operating characteristics (ROC) method was used to ascertain specificity-sensitivity trade-offs at different probability cut-offs in order to predict survival., Results: Only the maximum GCS and head injury AIS remained significant, both individually and in the polynomial logistic regression. Empiric ROC curve analyses from leave-one-out survival predictions showed statistical significance (area under the curve = 0.87, Z = 6.8, p < 0.001). Only 12% of cases were misclassified using the 'best' cut-off., Conclusion: An outcome predictive model for pediatric TBI can be devised using an appropriate mathematical model. It may help to estimate expected outcomes in pediatric TBI more objectively., (Copyright © 2012 S. Karger AG, Basel.)

Background/aim: Sectioning of the filum terminale is performed when spinal cord tethering is suspected, sometimes without clinical symptoms. Retethering can occur and require reoperation due to the presentation of either recurrent or new symptoms. The purpose of this institutional review was to identify the retethering rate in children, especially in those who were initially asymptomatic, and to discuss the role of surgery., Methods: The medical records of all children at the Children's Hospital of Eastern Ontario (CHEO) who underwent tethered cord surgery between 1978 and 2009 for a thickened filum terminale were retrospectively reviewed, as well as those who retethered., Results: A total of 146 patients with a mean age of 4.3 years underwent a low lumbar single or partial laminectomy for sectioning of the filum terminale; 44 patients (30.1%) were asymptomatic at the time of surgery, 51.4% had bladder and bowel dysfunction, 26.7% had neuroorthopedic findings, 15.8% had pain and 6.2% had progressive scoliosis; 11 children with a median age of 8.9 years had symptoms of retethering requiring reoperation (median time to retether was 4.3 years) and 4 were initially asymptomatic. Repeat surgery was successful at alleviating the new symptoms that occurred as a result of retethering., Conclusions: Of the 146 patients at CHEO who underwent surgery, 7.5% retethered, with 36% being initially asymptomatic. Those operated in the first year of life were not found to be at a higher risk. The level of the conus medullaris did not influence the rate or retethering or urological dysfunction. Children who were initially asymptomatic improved after surgery for retethering, but may not have required surgery in the first place., (Copyright © 2013 S. Karger AG, Basel.)

Optic pathway gliomas (OPG) in neurofibromatosis type 1 (NF1) usually remain localized to the anterior visual pathway. However, a small number can demonstrate widespread dissemination. We describe three children with NF1 OPGs and extensive central nervous system involvement. In one case, a postmortem examination revealed tumor cells extending continuously from the optic nerves to the conus medullaris. This is the most widespread NF1 OPG reported in the literature. We suggest that rapid visual deterioration in the absence of radiographic changes or increased intracranial pressure can be caused by increasing tumor infiltration within the central nervous system., (Copyright © 2011 Wiley-Liss, Inc.)

Object: This work assessed the value of shunt series in the evaluation of children with CSF shunts, a test that is frequently ordered as part of the assessment of shunt integrity., Methods: The medical records of all children who underwent shunt series at Children's Hospital of Eastern Ontario between 1975 and 2007 were reviewed. Ancillary studies that were performed within 2 days of the shunt series (brain CT, MR imaging, ultrasonography, and radionuclide shuntography) were noted, as well as the subsequent requirement for a shunt revision. Shunt series and ancillary studies were categorized as either positive (revealing an observable sign that a shunt-related problem was present, such as shunt discontinuity on the shunt series or enlargement of the ventricles on the brain CT scan) or negative (no clear sign of a shunt-related problem). Shunt series were further grouped into shunt series performed for diagnostic reasons, shunt series performed on a routine basis during follow-up in clinic, and shunt series performed postoperatively. The length of time between shunt insertion and shunt fracture was noted. Statistical analyses were performed, including the derivation of sensitivities and specificities., Results: There were 3416 shunt series in 394 patients, of which 3004 were performed with ancillary studies. On average, patients underwent 9 shunt series, with a minimum of 1 and a maximum of 43, during the follow-up period (range 3 weeks to 19 years). A total of 2493 shunt series and ancillary studies (83%) yielded negative results and no surgery was performed. One hundred thirty shunts series were negative with a positive ancillary study (4.3%) and no surgery was required. In 8.7% (261 shunt series negative, ancillary studies positive) shunt revision surgery was necessary. The opposite trend was far less prevalent (17 shunt series positive, ancillary test negative; 0.6%) in which surgery was required. There were 87 patients with 96 shunt fractures (2.8% of shunt series). The average time between shunt insertion and shunt fracture was 7.9 years (range 3 months to 18 years). Shunt series had a sensitivity of 18% and a specificity of 97%. The ancillary studies had a sensitivity of 84% and a specificity of 85%., Conclusions: The routine utilization of shunt series in the evaluation of a child with a CSF shunt is not necessary. This study showed that a very small number (0.6%) of shunt series helped in surgical decision making. Shunt series can be performed in selected cases, especially preoperatively in the absence of a baseline study to obtain information necessary for surgical planning.

Background: Treatment options for Chiari I malformations include posterior fossa decompression (PFD) with additional techniques including laminectomy, intradural exploration, and duraplasty. Neuroimaging findings of cisterna magna volume, syringomyelia, and intraoperative ultrasonography may tailor surgical intervention., Methods: We developed an algorithm classifying symptomatic Chiari I patients into three groups to define minimum operation. Without syringomyelia, the presence of cisterna magna defined Group A and the absence defined Group B. Patients with syrinx formed Group C. Mild structural pathology (Group A) or adequate space following PFD (Group B, normal intraoperative ultrasound (IOUS)) should be treated by PFD alone. Conversely, presence of syringomyelia (Group C) or inadequate space following PFD (Group B, abnormal IOUS) should additionally have duraplasty. We applied this algorithm to patients treated at a single institution over 16 years., Results: Twenty-four symptomatic Chiari I malformation patients were divided into three groups that did not differ by age, gender, or extent of tonsillar ectopia. All patients treated by this algorithm experienced clinical and radiographic improvement. This included eight Group B patients who underwent PFD only (n=6) or additional duraplasty (n=2) decided by IOUS., Conclusion: Treatment of symptomatic Chiari I malformation may have inadequate outcome with conservative strategy or complications with aggressive strategy. This algorithm utilizes preoperative neuroimaging and intraoperative ultrasound to tailor intervention, with excellent clinical outcome and radiographic syrinx resolution on application to 24 patients. Further validation requires prospective multicenter evaluation with larger patient population.

Introduction: One seventh of pediatric brain tumors are diagnosed in the first year of life. With more widespread and accessible neuroimaging, these lesions are being diagnosed earlier, but there remains scant literature about their natural history., Methods: A retrospective review was performed of brain tumor patients presenting to the Children's Hospital of Eastern Ontario (CHEO) through the last 34 years. Patients presenting in the first year of life, including symptoms, management features, and functional outcome, were analyzed using ANOVA and chi (2) statistics., Results: Eighteen cases of brain tumors in the first year of life were identified: 12 suptratentorial, eight with benign histology, and six infratentorial all with malignant histology. Median age of presentation differed by lesion location (p = 0.05) and glial tumors were most common. Raised intracranial pressure was more than twice as prevalent with posterior fossa lesions (p < 0.01) with equivalent likelihood of increasing head circumference (p = 0.74), whereas seizures were more frequent with supratentorial tumors (p = 0.04). Gross total resection was achieved in 47% of patients, cerebrospinal fluid diversion was more frequently necessary among infratentorial lesions (p = 0.02), and adjuvant therapy was more utilized for infratentorial lesions (p < 0.01). Among eight surviving infants, seven had supratentorial tumors, five survived to adulthood, and six are functionally independent., Conclusions: Brain tumors in the first year of life represent 4.8% of patients treated at CHEO. Mode of presentation, utilization of adjuvant therapy, and survival depend on tumor location and histology, with worse prognosis for infratentorial lesions. One third of patients had acceptable functional outcome requiring no special assistance.

Introduction: Patients with low grade astrocytomas generally have good prognosis when total resection can be achieved, but surveillance neuroimaging is commonly performed to detect recurrence or progression. This study evaluated the utility and yield of such strategy for pilocytic and non-pilocytic cerebellar astrocytomas., Methods: A 20-year retrospective review was performed of patients undergoing resection of cerebellar astrocytoma at a single institution. A negative MRI string (NMS) ratio was computed as the fraction of total follow-up period over which surveillance neuroimaging was negative for recurrence or progression. Chi-squared analysis differentiated NMS ratio by resection extent and lesion histopathology., Results: Twenty-eight patients with pilocytic (n=15) and non-pilocytic (n=13) astrocytoma underwent 34 craniotomies, with total resection in 19 cases. Surveillance MRIs (n=167) among total resection patients were uniformly negative for recurrent disease at average seven years follow-up (NMS ratio = 1.0). The 43 surveillance MRIs among subtotal resection patients revealed disease progression in two patients within six months of operation (NMS ratio = 0.78, p<0.05). No differences in NMS ratio were observed between pilocytic and non-pilocytic astrocytoma subtypes., Discussion: This study illustrates pediatric patients with low-grade cerebellar astrocytomas undergoing total resection may not benefit from routine surveillance neuroimaging, primarily because of low recurrence likelihood. Patients with subtotal resection may benefit from surveillance of residual disease, with further work aimed at exploring the schedule of such follow-up.

Background: Head injury causes substantial morbidity and mortality in children. The ThinkFirst For Kids (TFFK) program improves knowledge of safe behaviours in kindergarten to Grade 6 students., Methods: This study evaluated the TFFK curriculum for grade 7/8 students. Knowledge acquisition was assessed quantitatively by an injury prevention test at baseline, at curriculum completion, and six weeks later. Participant experiences and behaviours were explored qualitatively by interviews and focus groups., Results: Students (n=204) and teachers (n=6) from four schools participated in this study. Test scores improved from baseline (26.48+/-0.17, n=204), to completion (27.75+/-0.16, n=176), to six weeks post-completion (28.65+/-0.13, n=111) (p<0.05). Most students reported their decision-making and participation in risky behaviors was altered by the curriculum., Interpretation: The TFFK curriculum may promote education about head injury prevention among Grade 7/8 students, with a suggestion of long-term knowledge retention. The curriculum was well-received and may be suitable to reduce risky behavior and injuries in children.

Background: Dural arteriovenous fistulas are uncommon lesions in children, with traumatic lesions suspected to arise from incomplete arterial injury in proximity to a vein or draining sinus. Management of symptomatic acquired lesions requires evaluation of patient presentation, neurological status, and pathoanatomic configuration, with special consideration required for surgery secondary to failed endovascular technique., Case Report: A 12-year-old male sustained a bicycle fall causing a right temporo-parietal skull fracture associated with non-surgical right epidural hematoma and left contre-coup parietal contusion. Six-weeks later, he complained of a right temporal bruit with subsequent cerebral angiography demonstrating a dural-based fistula between the right middle meningeal artery and a dural vein draining into the sigmoid sinus., Intervention: Endovascular treatment of this lesion with glue embolization and coiling was unsuccessful, with angiographic illustration of previously unobserved collateral vessels and coils occupying the sigmoid sinus. A right temporo-parietal craniectomy was required to excise the dural-based fistula, followed by dural defect repair with bovine pericardium and subsequent cranioplasty. Six years later the patient remains neurologically intact with no headaches or bruit., Conclusions: Dural arteriovenous fistula can uncommonly occur following traumatic injury in children. Partial injury to the middle mengineal artery may have established arterial communication with the draining vein that became ectatic and tortuous under high pressure. Failure of primary endovascular treatment may complicate secondary surgical intervention.

Introduction: Congenital brain tumors in the younger pediatric population are rare lesions that are histologically distinct from those in the older pediatric population. Malignant histology is common, with persistently poor outcomes despite accessible neuroimaging and evolving adjuvant therapy. There remains scant literature about the natural history of these patients because of rarity and varied institutional experiences., Methods: A retrospective review was performed of congenital brain tumor patients surgically treated at the Montreal Children's Hospital (MCH) over a 22-year period. Patients presenting in the first year of life were evaluated for demographic information, presenting symptoms, lesion location, and management. Analysis was by median rank test and chi(2) statistics., Results: 13 cases of congenital brain tumors were identified: 5 supratentorial and 8 infratentorial. Median age (p = 0.93) and gender (p = 0.57) did not differ by location, and predominant histologies were choroid plexus papilloma and primitive neuroectodermal tumor. Seizure activity was exclusive to supratentorial lesions (40%, p = 0.03), with hypotonia observed only among infratentorial lesions (50%, p = 0.02). There was equal incidence of hydrocephalus (69%, p = 0.57) and increasing head circumference (38%, p = 0.27) by lesion location. Supratentorial lesions were treated by total resection (n = 3), subtotal resection (n = 1), and biopsy (n = 1). Infratentorial lesions were treated by total resection (n = 1), subtotal resection (n = 2), biopsy (n = 1), no operation (n = 2), and decompressive laminectomy for two spinal lesions., Conclusions: Congenital brain tumor patients represent fewer than 2% of patients treated at MCH. An evolving understanding of management objectives for these lesions requires understanding institutional experiences. Patients with supratentorial lesions frequently present with seizures, hydrocephalus, and macrocrania, and more frequently underwent total resection at surgery., (Copyright 2009 S. Karger AG, Basel.)

Objective: This work evaluated the mortality and functional outcomes of premature infants sustaining intraventricular hemorrhage (IVH). These outcomes were analyzed for their association with IVH severity, development of hydrocephalus and need for ventriculoperitoneal (VP) shunt insertion., Methods: A retrospective review was performed of neonates at the Children's Hospital of Eastern Ontario who sustained IVH (1989-2005). Logistic regression tested demographic predictors of IVH severity, development of hydrocephalus and mortality. Chi(2) analysis differentiated functional outcomes and mortality by IVH grade, development of hydrocephalus and intervention for cerebrospinal fluid diversion. All analyses used the 0.05 significance level., Results: Data were available for 284 patients with an average follow-up of 5.1 years. These were distributed as grade I (n = 135), grade II (n = 52), grade III (n = 43) and grade IV (n = 54), with lower gestational age and birth weight predicting disease severity. Hydrocephalus developed in 21% of patients, of whom 39% required VP shunt insertion. Overall mortality of 20% depended on IVH grade and was highest for grade IV patients (59%). Functional independence diminished with IVH severity, and patients with hydrocephalus fared worse than those without this sequel. Outcomes of patients developing hydrocephalus were similar whether or not they eventually required VP shunt insertion., Conclusions: This study describes a large cohort of neonatal IVH, describing how disease severity affects mortality and functional outcome. The overall mortality of nearly 1 in 5 patients is primarily of grade IV patients, with no difference between grade II and grade III. Further, patients surviving their hydrocephalus exhibited no worse functional deterioration if they required surgical intervention., (2009 S. Karger AG, Basel)

Objectives: Cerebral vascular disease has been reported as a long-term complication of cranial radiotherapy. The purpose of this study was to examine the frequency and risk factors associated with development of cerebral vascular disease in children after cranial radiation., Materials and Methods: A retrospective chart review of all cancer patients treated between 1985 and 2003 who were under the age of 18 years at the time of initial radiotherapy was performed. Variables examined include diagnosis and site of malignancy, age at the time of radiotherapy, sex, total radiation dosage, number of fractions, duration, and whether the patient had proven cerebral vascular event., Results: Two hundred and forty-four patients met the study criteria. One hundred and 13 cases involved tumors of the central nervous system. The remaining patients had systemic neoplastic disease. Post radiation cerebral vascular disease occurred in 11 (5%) patients, and all but one patient had a tumor involving the central nervous system (mainly in the posterior fossa and supratentorial midline)., Conclusion: There is an increased risk of cerebral vascular disease after radiation therapy in childhood, especially in children who received high dose radiation at the posterior fossa and supratentorial axial region.