Aortic Valve Thrombus, Stroke, and Endovascular Thrombectomy in a Child With APML and Trisomy 21.

APML, a subtype of acute myeloid leukemia, is highly curable, with cure rates over 90%. Despite its therapeutic success, APML poses elevated bleeding risks due to frequent prior disseminated intravascular coagulation. Less commonly recognized but critical is the thrombotic risk. We document a unique pediatric case: a 13-year-old with trisomy 21 diagnosed with APML had an asymptomatic aortic valve thrombus leading to thromboembolic arterial ischemic stroke. Through endovascular thrombectomy, cerebral circulation was re-established, extracting a fibrin thrombus with APML cells. Neurological recovery was swift. This report underscores the importance of vigilance for thrombotic complications in APML, highlighting the potential severity of overlooked risks.

Can quantitative susceptibility mapping help diagnose and predict recovery of concussion in children? An A-CAP study.

BACKGROUND: Quantitative susceptibility mapping (QSM) is an MRI technique that is a potential biomarker for concussion. We performed QSM in children following concussion or orthopaedic injury (OI), to assess QSM performance as a diagnostic and prognostic biomarker. METHODS: Children aged 8-17 years with either concussion (N=255) or OI (N=116) were recruited from four Canadian paediatric emergency departments and underwent QSM postacutely (2-33 days postinjury) using 3 Tesla MRI. QSM Z-scores within nine regions of interest (ROI) were compared between groups. QSM Z-scores were also compared with the 5P score, the current clinical benchmark for predicting persistent postconcussion symptoms (PPCS), at 4 weeks postinjury, with PPCS defined using reliable change methods based on both participant and parent reports. RESULTS: Concussion and OI groups did not differ significantly in QSM Z-scores for any ROI. Higher QSM Z-scores within frontal white matter (WM) independently predicted PPCS based on parent ratings of cognitive symptoms (p=0.001). The combination of frontal WM QSM Z-score and 5P score was better at predicting PPCS than 5P score alone (p=0.004). The area under the curve was 0.72 (95% CI 0.63 to 0.81) for frontal WM susceptibility, 0.69 (95% CI 0.59 to 0.79) for the 5P score and 0.74 (95% CI 0.65 to 0.83) for both. CONCLUSION: The findings suggest that QSM is a potential MRI biomarker that can help predict PPCS in children with concussion, over and above the current clinical benchmark, and thereby aid in clinical management. They also suggest a frontal lobe substrate for PPCS, highlighting the potential for QSM to clarify the neurophysiology of paediatric concussion.

Treatment of hydrocephalus following posterior fossa tumor resection: a multicenter collaboration from the Hydrocephalus Clinical Research Network.

OBJECTIVE: Persistent hydrocephalus following posterior fossa brain tumor (PFBT) resection is a common cause of morbidity in pediatric brain tumor patients, for which the optimal treatment is debated. The purpose of this study was to compare treatment outcomes between VPS and ETV in patients with persistent hydrocephalus following surgical resection of a PFBT. METHODS: A post-hoc analysis was performed of the Hydrocephalus Clinical Research Network (HCRN) prospective observational study evaluating VPS and ETV for pediatric patients. Children who experienced hydrocephalus secondary to PFBT from 2008 to 2021 were included. Primary outcomes were VPS/ETV treatment failure and time-to-failure (TTF). RESULTS: Among 241 patients, the VPS (183) and ETV (58) groups were similar in age, extent of tumor resection, and preoperative ETV Success Score. There was no difference in overall treatment failure between VPS and ETV (33.9% vs 31.0%, p = 0.751). However, mean TTF was shorter for ETV than VPS (0.45 years vs 1.30 years, p = 0.001). While major complication profiles were similar, compared to VPS, ETV patients had relatively higher incidence of minor CSF leak (10.3% vs. 1.1%, p = 0.003) and pseudomeningocele (12.1% vs 3.3%, p = 0.02). No ETV failures were identified beyond 3 years, while shunt failures occurred beyond 5 years. Shunt infections occurred in 5.5% of the VPS cohort. CONCLUSIONS: ETV and VPS offer similar overall success rates for PFBT-related postoperative hydrocephalus. ETV failure occurs earlier, while susceptibility to VPS failure persists beyond 5 years. Tumor histology and grade may be considered when selecting the optimal means of CSF diversion.

Neurosurgeons' Perspectives on Vascular Entrustable Professional Activities.

This article discusses subspecialty Canadian neurosurgeons' perceptions of entrustable professional activities (EPAs) assessments and variabilities prior to the implementation of the Competence by Design (CBD) system in Canada. Vascular neurosurgeons were asked to reflect on how they would evaluate and give feedback to neurosurgery residents concerning the EPA "Performing surgery for patients with an intracranial aneurysm." Interviews were transcribed and analyzed using a deductive approach. Themes were derived from these interviews and reflected on the subjectivity and biases present in the EPA assessment forms. Indeed, faculty may require more training in the transitioning to a CBD evaluation system.

Validation of the Calgary Postoperative Pain after Spine Surgery Score for Poor Postoperative Pain Control after Spine Surgery.

OBJECTIVE: The Calgary Postoperative Pain after Spine Surgery (CAPPS) score was developed to identify patients at risk of experiencing poorly controlled pain after spine surgery. The goal of this study was to independently validate the CAPPS score on a prospectively collected patient sample. METHODS: Poor postoperative pain control was defined as a mean numeric rating scale (NRS) for pain >4 at rest in the first 24 hours after surgery. Baseline characteristics in this study (validation cohort) were compared to those of the development cohort used to create the CAPPS score. Predictive performance of the CAPPS score was assessed by the area under the curve (AUC) and percentage misclassification for discrimination. A graphical comparison between predicted probability vs. observed incidence of poorly controlled pain was performed for calibration. RESULTS: Fifty-two percent of 201 patients experienced poorly controlled pain. The validation cohort exhibited lower depression scores and a higher proportion using daily opioid medications compared to the development cohort. The AUC was 0.74 [95%CI = 0.68-0.81] in the validation cohort compared to 0.73 [95%CI = 0.69-0.76] in the development cohort for the eight-tier CAPPS score. When stratified between the low- vs. extreme-risk and low- vs. high-risk groups, the percentage misclassification was 21.2% and 30.7% in the validation cohort, compared to 29.9% and 38.0% in the development cohort, respectively. The predicted probability closely mirrored the observed incidence of poor pain control across all scores. CONCLUSIONS: The CAPPS score, based on seven easily obtained and reliable prognostic variables, was validated using a prospectively collected, independent sample of patients.

Longitudinal CSF Iron Pathway Proteins in Posthemorrhagic Hydrocephalus: Associations with Ventricle Size and Neurodevelopmental Outcomes.

OBJECTIVE: Iron has been implicated in the pathogenesis of brain injury and hydrocephalus after preterm germinal matrix hemorrhage-intraventricular hemorrhage, however, it is unknown how external or endogenous intraventricular clearance of iron pathway proteins affect the outcome in this group. METHODS: This prospective multicenter cohort included patients with posthemorrhagic hydrocephalus (PHH) who underwent (1) temporary and permanent cerebrospinal fluid (CSF) diversion and (2) Bayley Scales of Infant Development-III testing around 2 years of age. CSF proteins in the iron handling pathway were analyzed longitudinally and compared to ventricle size and neurodevelopmental outcomes. RESULTS: Thirty-seven patients met inclusion criteria with a median estimated gestational age at birth of 25 weeks; 65% were boys. Ventricular CSF levels of hemoglobin, iron, total bilirubin, and ferritin decreased between temporary and permanent CSF diversion with no change in CSF levels of ceruloplasmin, transferrin, haptoglobin, and hepcidin. There was an increase in CSF hemopexin during this interval. Larger ventricle size at permanent CSF diversion was associated with elevated CSF ferritin (p = 0.015) and decreased CSF hemopexin (p = 0.007). CSF levels of proteins at temporary CSF diversion were not associated with outcome, however, higher CSF transferrin at permanent CSF diversion was associated with improved cognitive outcome (p = 0.015). Importantly, longitudinal change in CSF iron pathway proteins, ferritin (decrease), and transferrin (increase) were associated with improved cognitive (p = 0.04) and motor (p = 0.03) scores and improved cognitive (p = 0.04), language (p = 0.035), and motor (p = 0.008) scores, respectively. INTERPRETATION: Longitudinal changes in CSF transferrin (increase) and ferritin (decrease) are associated with improved neurodevelopmental outcomes in neonatal PHH, with implications for understanding the pathogenesis of poor outcomes in PHH. ANN NEUROL 2021;90:217-226.

Implementation of competence by design in Canadian neurosurgery residency programs.

OBJECTIVE: The Royal College of Physicians and Surgeons of Canada (RCPSC) recently redesigned the Canadian neurosurgery residency training curriculum by implementing a competency-based model of training known as Competence by Design (CBD) centered around the assessment of Entrustable Professional Activities (EPAs). This sequential explanatory mixed-methods study evaluated potential benefits and pitfalls of CBD in Canadian neurosurgery residency education. METHODS: Two four-month interval surveys were distributed to all Canadian neurosurgery residents participating in CBD. The surveys assessed important educational components: CBD knowledge of key stakeholders, potential system barriers, and educational/psychological impacts on residents. Paired t-tests were done to assess changes over time. Based on longitudinal survey responses, semi-structured interviews were conducted to investigate in-depth residents' experience with CBD in neurosurgery. The qualitative analysis followed an explanatory approach, and a thematic analysis was performed. RESULTS: Surveys had 82% average response rate (n = 25). Over time, most residents self-reported that they retrospectively understood concepts around CBD intentions (p = 0.02). Perceived benefits included faculty evaluations with more feedback that was clearer and more objective (53% and 51%). Pitfalls included the amount of time needed to navigate through EPAs (90%) and residents forgetting to initiate EPA forms (71%). There was no significant change over time. During interviews, five key themes were found. Potential solutions identified by residents to enhance their experience included learning analytics data availability, mobile app refinement, and dedicated time to integrate EPAs in the workflow. CONCLUSION: This study was the first to assess resident-perceived benefits and pitfalls of the neurosurgery CBD training program in an educational framework context. In general, residents believed that theoretical principles behind CBD were valuable, but that technological ability and having enough time to request EPA assessments were significant barriers to success. Long-term studies are required to determine the definitive outcomes of CBD on residents' performance and ultimately, on patient care.

Primary and metastatic glioblastoma of the spine in the pediatric population: a systematic review.

Pediatric glioblastoma multiforme (GBM) involving the spine is an aggressive tumor with a poor quality of life for patients. Despite this, there is only a limited number of reports describing the outcomes of pediatric spinal GBMs, both as primary spinal GBMs and metastases from an intracranial tumor. Here, we performed an individual patient meta-analysis to characterize factors affecting prognosis of pediatric spinal GBM. MEDLINE, Embase, and the Cochrane databases were searched for published studies on GBMs involving the spine in pediatric patients (age ≤ 21 years old). Factors associated with the survival were assessed with multi-factor ANOVAs, Cox hazard regression, and Kaplan-Meier analyses. We extracted data on 61 patients with spinal GBM from 40 studies that met inclusion criteria. Median survival was significantly longer in the primary spinal GBM compared that those with metastatic GBM (11 vs 3 months, p < 0.001). However, median survival of metastatic GBM patients was 10 months following diagnosis of their primary brain tumor, which was not different from that of primary spinal GBM patients (p = 0.457). Among primary spinal GBM patients, chemotherapy (hazard ratio (HR) = 0.255 [0.106-0.615], p = 0.013) and extent of resection (HR = 0.582 [0.374-0.905], p = 0.016) conferred a significant survival benefit. Younger age (less than 14 years) was associated with longer survival in patients treated with chemotherapy than those who did not undergo chemotherapy (ß = - 1.12, 95% CI [- 2.20, - 0.03], p < 0.05). In conclusion, survival after presentation of metastases from intracranial GBM is poor in the pediatric population. In patients with metastatic GBM, chemotherapy may have provided the most benefit in young patients, and its efficacy might have an association with extent of surgical resection.

Machine learning predicts risk of cerebrospinal fluid shunt failure in children: a study from the hydrocephalus clinical research network.

PURPOSE: While conventional statistical approaches have been used to identify risk factors for cerebrospinal fluid (CSF) shunt failure, these methods may not fully capture the complex contribution of clinical, radiologic, surgical, and shunt-specific variables influencing this outcome. Using prospectively collected data from the Hydrocephalus Clinical Research Network (HCRN) patient registry, we applied machine learning (ML) approaches to create a predictive model of CSF shunt failure. METHODS: Pediatric patients (age < 19 years) undergoing first-time CSF shunt placement at six HCRN centers were included. CSF shunt failure was defined as a composite outcome including requirement for shunt revision, endoscopic third ventriculostomy, or shunt infection within 5 years of initial surgery. Performance of conventional statistical and 4 ML models were compared. RESULTS: Our cohort consisted of 1036 children undergoing CSF shunt placement, of whom 344 (33.2%) experienced shunt failure. Thirty-eight clinical, radiologic, surgical, and shunt-design variables were included in the ML analyses. Of all ML algorithms tested, the artificial neural network (ANN) had the strongest performance with an area under the receiver operator curve (AUC) of 0.71. The ANN had a specificity of 90% and a sensitivity of 68%, meaning that the ANN can effectively rule-in patients most likely to experience CSF shunt failure (i.e., high specificity) and moderately effective as a tool to rule-out patients at high risk of CSF shunt failure (i.e., moderately sensitive). The ANN was independently validated in 155 patients (prospectively collected, retrospectively analyzed). CONCLUSION: These data suggest that the ANN, or future iterations thereof, can provide an evidence-based tool to assist in prognostication and patient-counseling immediately after CSF shunt placement.

Comparison of anticoagulation and antiplatelet therapy for treatment of blunt cerebrovascular injury in children <10 years of age: a multicenter retrospective cohort study.

PURPOSE: Blunt cerebrovascular injury (BCVI) is uncommon in the pediatric population. Among the management options is medical management consisting of antithrombotic therapy with either antiplatelets or anticoagulation. There is no consensus on whether administration of antiplatelets or anticoagulation is more appropriate for BCVI in children < 10 years of age. Our goal was to compare radiographic and clinical outcomes based on medical treatment modality for BCVI in children < 10 years. METHODS: Clinical and radiographic data were collected retrospectively for children screened for BCVI with computed tomography angiography at 5 academic pediatric trauma centers. RESULTS: Among 651 patients evaluated with computed tomography angiography to screen for BCVI, 17 patients aged less than 10 years were diagnosed with BCVI (7 grade I, 5 grade II, 1 grade III, 4 grade IV) and received anticoagulation or antiplatelet therapy for 18 total injuries: 11 intracranial carotid artery, 4 extracranial carotid artery, and 3 extracranial vertebral artery injuries. Eleven patients were treated with antiplatelets (10 aspirin, 1 clopidogrel) and 6 with anticoagulation (4 unfractionated heparin, 2 low-molecular-weight heparin, 1 transitioned from the former to the latter). There were no complications secondary to treatment. One patient who received anticoagulation died as a result of the traumatic injuries. In aggregate, children treated with antiplatelet therapy demonstrated healing on 52% of follow-up imaging studies versus 25% in the anticoagulation cohort. CONCLUSION: There were no observed differences in the rate of hemorrhagic complications between anticoagulation and antiplatelet therapy for BCVI in children < 10 years, with a nonsignificantly better rate of healing on follow-up imaging in children who underwent antiplatelet therapy; however, the study cohort was small despite including patients from 5 hospitals.

Hydrocephalus treatment in patients with craniosynostosis: an analysis from the Hydrocephalus Clinical Research Network prospective registry.

OBJECTIVE: Hydrocephalus may be seen in patients with multisuture craniosynostosis and, less commonly, single-suture craniosynostosis. The optimal treatment for hydrocephalus in this population is unknown. In this study, the authors aimed to evaluate the success rate of ventriculoperitoneal shunt (VPS) treatment and endoscopic third ventriculostomy (ETV) both with and without choroid plexus cauterization (CPC) in patients with craniosynostosis. METHODS: Utilizing the Hydrocephalus Clinical Research Network (HCRN) Core Data Project (Registry), the authors identified all patients who underwent treatment for hydrocephalus associated with craniosynostosis. Descriptive statistics, demographics, and surgical outcomes were evaluated. RESULTS: In total, 42 patients underwent treatment for hydrocephalus associated with craniosynostosis. The median gestational age at birth was 39.0 weeks (IQR 38.0, 40.0); 55% were female and 60% were White. The median age at first craniosynostosis surgery was 0.6 years (IQR 0.3, 1.7), and at the first permanent hydrocephalus surgery it was 1.2 years (IQR 0.5, 2.5). Thirty-three patients (79%) had multiple different sutures fused, and 9 had a single suture: 3 unicoronal (7%), 3 sagittal (7%), 2 lambdoidal (5%), and 1 unknown (2%). Syndromes were identified in 38 patients (90%), with Crouzon syndrome being the most common (n = 16, 42%). Ten patients (28%) received permanent hydrocephalus surgery before the first craniosynostosis surgery. Twenty-eight patients (67%) underwent VPS treatment, with the remaining 14 (33%) undergoing ETV with or without CPC (ETV ± CPC). Within 12 months after initial hydrocephalus intervention, 14 patients (34%) required revision (8 VPS and 6 ETV ± CPC). At the most recent follow-up, 21 patients (50%) required a revision. The revision rate decreased as age increased. The overall infection rate was 5% (VPS 7%, 0% ETV ± CPC). CONCLUSIONS: This is the largest prospective study reported on children with craniosynostosis and hydrocephalus. Hydrocephalus in children with craniosynostosis most commonly occurs in syndromic patients and multisuture fusion. It is treated at varying ages; however, most patients undergo surgery for craniosynostosis prior to hydrocephalus treatment. While VPS treatment is performed more frequently, VPS and ETV are both reasonable options, with decreasing revision rates with increasing age, for the treatment of hydrocephalus associated with craniosynostosis.

Evaluation of pediatric glioma outcomes using intraoperative MRI: a multicenter cohort study.

BACKGROUND: The use of intraoperative MRI (iMRI) during treatment of gliomas may increase extent of resection (EOR), decrease need for early reoperation, and increase progression-free and overall survival, but has not been fully validated, particularly in the pediatric population. OBJECTIVE: To assess the accuracy of iMRI to identify residual tumor in pediatric patients with glioma and determine the effect of iMRI on decisions for resection, complication rates, and other outcomes. METHODS: We retrospectively analyzed a multicenter database of pediatric patients (age ≤ 18 years) who underwent resection of pathologically confirmed gliomas. RESULTS: We identified 314 patients (mean age 9.7 ± 4.6 years) with mean follow-up of 48.3 ± 33.6 months (range 0.03-182.07 months) who underwent surgery with iMRI. There were 201 (64.0%) WHO grade I tumors, 57 (18.2%) grade II, 24 (7.6%) grade III, 9 (2.9%) grade IV, and 23 (7.3%) not classified. Among 280 patients who underwent resection using iMRI, 131 (46.8%) had some residual tumor and underwent additional resection after the first iMRI. Of the 33 tissue specimens sent for pathological analysis after iMRI, 29 (87.9%) showed positive tumor pathology. Gross total resection was identified in 156 patients (55.7%), but this was limited by 69 (24.6%) patients with unknown EOR. CONCLUSIONS: Analysis of the largest multicenter database of pediatric gliomas resected using iMRI demonstrated additional tumor resection in a substantial portion of cases. However, determining the impact of iMRI on EOR and outcomes remains challenging because iMRI use varies among providers nationally. Continued refinement of iMRI techniques for use in pediatric patients with glioma may improve outcomes.

Interhypothalamic adhesions in endoscopic third ventriculostomy.

INTRODUCTION: An interhypothalamic adhesion (IHA) is a gray mater-like band of tissue traversing across the third ventricle anterior to the mammillary bodies and is similar but distinct from an interthalamic adhesion. These rare anatomic anomalies can be detected with magnetic resonance imaging or, incidentally, during endoscopic ventricular surgery. METHODS: All cases of interhypothalamic adhesions visualized during endoscopic third ventriculotomy (ETV), outside of the myelomeningocele setting, were identified from two institutions. Retrospective chart and imaging reviews were conducted and compared to intraoperative videos and photos for all cases. IHA variables collected included the following size, location, multiplicity, and associated anatomic anomalies. RESULTS: Four cases of interhypothalamic adhesions were identified during ETV-all of which, either partially or completely, obscured access to the third ventricular floor. The IHAs in our cohort were duplicated in two patients, large (> 3 mm and severely obstructing access to the third ventricular floor) in three patients, and adherent to the floor of the third ventricle in three patients. All four patients had primary absence of the septum pellucidum. Previous reports found associations of IHAs with other congenital, particularly midline, abnormalities. The IHAs in our cohort affected the surgery in three of four cases including misdirecting the ventriculostomy and requiring retraction or division of the IHA. In no case was postoperative pituitary or hypothalamic dysfunction observed. CONCLUSIONS: Although interhypothalamic adhesions are rare, these anomalies must be recognized as they may hinder access to the third ventricular floor. IHAs may be large, multiple, or adherent to adjacent ventricular structures, they can misdirect or occlude the ventriculostomy or impart risk of bleeding and hypothalamic injury. Techniques for management of IHA include aborting the attempt, re-siting the ventriculostomy, or retracting or dividing the IHA, which enabled technically successful ETV in three of four patients in this series.

Systematic review of health economic studies in cranial neurosurgery.

OBJECTIVE As the cost of health care continues to increase, there is a growing emphasis on evaluating the relative economic value of treatment options to guide resource allocation. The objective of this systematic review was to evaluate the current evidence regarding the cost-effectiveness of cranial neurosurgery procedures. METHODS The authors performed a systematic review of the literature using PubMed, EMBASE, and the Cochrane Library, focusing on themes of economic evaluation and cranial neurosurgery following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Included studies were publications of cost-effectiveness analysis or cost-utility analysis between 1995 and 2017 in which health utility outcomes in life years (LYs), quality-adjusted life years (QALYs), or disability-adjusted life years (DALYs) were used. Three independent reviewers conducted the study appraisal, data abstraction, and quality assessment, with differences resolved by consensus discussion. RESULTS In total, 3485 citations were reviewed, with 53 studies meeting the inclusion criteria. Of those, 34 studies were published in the last 5 years. The most common subspecialty focus was cerebrovascular (32%), followed by neurooncology (26%) and functional neurosurgery (24%). Twenty-eight (53%) studies, using a willingness to pay threshold of US$50,000 per QALY or LY, found a specific surgical treatment to be cost-effective. In addition, there were 11 (21%) studies that found a specific surgical option to be economically dominant (both cost saving and having superior outcome), including endovascular thrombectomy for acute ischemic stroke, epilepsy surgery for drug-refractory epilepsy, and endoscopic pituitary tumor resection. CONCLUSIONS There is an increasing number of cost-effectiveness studies in cranial neurosurgery, especially within the last 5 years. Although there are numerous procedures, such as endovascular thrombectomy for acute ischemic stroke, that have been conclusively proven to be cost-effective, there remain promising interventions in current practice that have yet to meet cost-effectiveness thresholds.

Development and Prospective Validation of Tools to Accurately Identify Neurosurgical and Critical Care Events in Children With Traumatic Brain Injury.

OBJECTIVE: To develop and validate case definitions (computable phenotypes) to accurately identify neurosurgical and critical care events in children with traumatic brain injury. DESIGN: Prospective observational cohort study, May 2013 to September 2015. SETTING: Two large U.S. children's hospitals with level 1 Pediatric Trauma Centers. PATIENTS: One hundred seventy-four children less than 18 years old admitted to an ICU after traumatic brain injury. MEASUREMENTS AND MAIN RESULTS: Prospective data were linked to database codes for each patient. The outcomes were prospectively identified acute traumatic brain injury, intracranial pressure monitor placement, craniotomy or craniectomy, vascular catheter placement, invasive mechanical ventilation, and new gastrostomy tube or tracheostomy placement. Candidate predictors were database codes present in administrative, billing, or trauma registry data. For each clinical event, we developed and validated penalized regression and Boolean classifiers (models to identify clinical events that take database codes as predictors). We externally validated the best model for each clinical event. The primary model performance measure was accuracy, the percent of test patients correctly classified. The cohort included 174 children who required ICU admission after traumatic brain injury. Simple Boolean classifiers were greater than or equal to 94% accurate for seven of nine clinical diagnoses and events. For central venous catheter placement, no classifier achieved 90% accuracy. Classifier accuracy was dependent on available data fields. Five of nine classifiers were acceptably accurate using only administrative data but three required trauma registry fields and two required billing data. CONCLUSIONS: In children with traumatic brain injury, computable phenotypes based on simple Boolean classifiers were highly accurate for most neurosurgical and critical care diagnoses and events. The computable phenotypes we developed and validated can be used in any observational study of children with traumatic brain injury and can reasonably be applied in studies of these interventions in other patient populations.

Variability in Management of First Cerebrospinal Fluid Shunt Infection: A Prospective Multi-Institutional Observational Cohort Study.

OBJECTIVES: To describe the variation in approaches to surgical and antibiotic treatment for first cerebrospinal fluid (CSF) shunt infection and adherence to Infectious Diseases Society of America (IDSA) guidelines. STUDY DESIGN: We conducted a prospective cohort study of children undergoing treatment for first CSF infection at 7 Hydrocephalus Clinical Research Network hospitals from April 2008 through December 2012. Univariate analyses were performed to describe the study population. RESULTS: A total of 151 children underwent treatment for first CSF shunt-related infection. Most children had undergone initial CSF shunt placement before the age of 6 months (n = 98, 65%). Median time to infection after shunt surgery was 28 days (IQR 15-52 days). Surgical management was most often shunt removal with interim external ventricular drain placement, followed by new shunt insertion (n = 122, 81%). Median time from first negative CSF culture to final surgical procedure was 14 days (IQR 10-21 days). Median duration of intravenous (IV) antibiotic use duration was 19 days (IQR 12-28 days). For 84 infections addressed by IDSA guidelines, 7 (8%) met guidelines and 61 (73%) had longer duration of IV antibiotic use than recommended. CONCLUSIONS: Surgical treatment for infection frequently adheres to IDSA guidelines of shunt removal with external ventricular drain placement followed by new shunt insertion. However, duration of IV antibiotic use in CSF shunt infection treatment was consistently longer than recommended by the 2004 IDSA guidelines.

Chiari-related hydrocephalus: assessment of clinical risk factors in a cohort of 297 consecutive patients.

OBJECTIVE Patients treated for Chiari I malformation (CM-I) with posterior fossa decompression (PFD) may occasionally and unpredictably develop postoperative hydrocephalus. The clinical risk factors predictive of this type of Chiari-related hydrocephalus (CRH) are unknown. The authors' objective was to evaluate their experience to identify risk factors that may predict which of these patients undergoing PFD will develop CRH after surgery. METHODS The authors performed a retrospective clinical chart review of all patients who underwent PFD surgery and duraplasty for CM-I at the Primary Children's Hospital in Utah from June 1, 2005, through May 31, 2015. Patients were dichotomized based on the need for long-term CSF diversion after PFD. Analysis included both univariate and multivariable logistic regression analyses. RESULTS The authors identified 297 decompressive surgeries over the period of the study, 22 of which required long-term postoperative CSF diversion. On multivariable analysis, age < 6 years old (OR 3.342, 95% CI 1.282-8.713), higher intraoperative blood loss (OR 1.003, 95% CI 1.001-1.006), and the presence of a fourth ventricular web (OR 3.752, 95% CI 1.306-10.783) were significantly associated with the need for long-term CSF diversion after decompressive surgery. CONCLUSIONS Younger patients, those with extensive intraoperative blood loss, and those found during surgery to have a fourth ventricular web were at higher risk for the development of CRH. Clinicians should be alert to evidence of CRH in this patient population after PFD surgery.

Management of posterior fossa tumors and hydrocephalus in children: a review.

OBJECT: Most pediatric patients that present with a posterior fossa tumor have concurrent hydrocephalus. There is significant debate over the best management strategy of hydrocephalus in this situation. The objectives of this paper were to review the pathophysiology model of posterior fossa tumor hydrocephalus, describe the individual risks factors of persistent hydrocephalus, and discuss the current management options. Specifically, the debate over preresection cerebrospinal fluid diversion is discussed. RESULTS: Only 10-40 % demonstrate persistent hydrocephalus after posterior fossa tumor resection. It appears that young age, moderate to severe hydrocephalus, transependymal edema, the presence of cerebral metastases, and tumor pathology (medulloblastoma and ependymoma) on presentation predict postresection or persistent hydrocephalus. The Canadian Preoperative Prediction Rule for Hydrocephalus (CPPRH), a validated prediction model, can be used to stratify patients at point of first contact into high and low risk for persistent hydrocephalus. CONCLUSIONS: A protocol is proposed for managing hydrocephalus that utilizes the CPPRH. Low-risk patients can be monitored conservatively with or without an intraoperative extraventricular drain, while high-risk patients require the use of an intraoperative extraventricular drain, higher postoperative hydrocephalus surveillance, and even consideration for a preoperative endoscopic third ventriculostomy.

Cervical spine imaging in hospitalized children with traumatic brain injury.

OBJECTIVES: The purposes of this study, in children with traumatic brain injury (TBI), to describe cervical spine imaging practice, to assess for recent changes in imaging practice, and to determine whether cervical spine computed tomography (CT) is being used in children at low risk for cervical spine injury. METHODS: The setting was children's hospitals participating in the Pediatric Health Information System database, from January 2001 to June 2011. Participants were children (younger than 18 y) with TBI who were evaluated in the emergency department, admitted to the hospital, and received a head CT scan on the day of admission. The primary outcome measures were cervical spine imaging studies. This study was exempted from institutional review board assessment. RESULTS: A total of 30,112 children met study criteria. Overall, 52% (15,687/30,112) received cervical spine imaging. The use of cervical spine radiographs alone decreased between 2001 (47%) and 2011 (23%), with an annual decrease of 2.2% (95% confidence interval [CI], 1.1%-3.3%), and was largely replaced by an increased use of CT, with or without radiographs (8.6% in 2001 and 19.5% in 2011, with an annual increase of 0.9%; 95% CI, 0.1%-1.8%). A total of 2545 children received cervical spine CT despite being discharged alive from the hospital in less than 72 hours, and 1655 of those had a low-risk mechanism of injury. CONCLUSIONS: The adoption of CT clearance of the cervical spine in adults seems to have influenced the care of children with TBI, despite concerns about radiation exposure.

Risk factors for first cerebrospinal fluid shunt infection: findings from a multi-center prospective cohort study.

OBJECTIVE: To quantify the extent to which cerebrospinal fluid (CSF) shunt revisions are associated with increased risk of CSF shunt infection, after adjusting for patient factors that may contribute to infection risk. STUDY DESIGN: We used the Hydrocephalus Clinical Research Network registry to assemble a large prospective 6-center cohort of 1036 children undergoing initial CSF shunt placement between April 2008 and January 2012. The primary outcome of interest was first CSF shunt infection. Data for initial CSF shunt placement and all subsequent CSF shunt revisions prior to first CSF shunt infection, where applicable, were obtained. The risk of first infection was estimated using a multivariable Cox proportional hazard model accounting for patient characteristics and CSF shunt revisions, and is reported using hazard ratios (HRs) with 95% CI. RESULTS: Of the 102 children who developed first infection within 12 months of placement, 33 (32%) followed one or more CSF shunt revisions. Baseline factors independently associated with risk of first infection included: gastrostomy tube (HR 2.0, 95% CI, 1.1, 3.3), age 6-12 months (HR 0.3, 95% CI, 0.1, 0.8), and prior neurosurgery (HR 0.4, 95% CI, 0.2, 0.9). After controlling for baseline factors, infection risk was most significantly associated with the need for revision (1 revision vs none, HR 3.9, 95% CI, 2.2, 6.5; ≥2 revisions, HR 13.0, 95% CI, 6.5, 24.9). CONCLUSIONS: This study quantifies the elevated risk of infection associated with shunt revisions observed in clinical practice. To reduce risk of infection risk, further work should optimize revision procedures.