Search

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., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objectives: The acute cerebral physiologic effects of ketamine in children have been incompletely described. We assessed the acute effects of ketamine on intracranial pressure (ICP) and cerebral perfusion pressure (CPP) in children with severe traumatic brain injury (TBI)., Design: In this retrospective observational study, patients received bolus doses of ketamine for sedation or as a treatment for ICP crisis (ICP > 20 mm Hg for > 5 min). Administration times were synchronized with ICP and CPP recordings at 1-minute intervals logged in an automated database within the electronic health record. ICP and CPP were each averaged in epochs following drug administration and compared with baseline values. Age-based CPP thresholds were subtracted from CPP recordings and compared with baseline values. Trends in ICP and CPP over time were assessed using generalized least squares regression., Setting: A 30-bed tertiary care children's hospital PICU., Patients: Children with severe TBI who underwent ICP monitoring., Interventions: None., Measurements and Main Results: We analyzed data from 33 patients, ages 1 month to 16 years, 22 of whom received bolus doses of ketamine, with 127 doses analyzed. Demographics, patient, and injury characteristics were similar between patients who did versus did not receive ketamine boluses. In analysis of the subset of ketamine doses used only for sedation, there was no significant difference in ICP or CPP from baseline. Eighteen ketamine doses were given during ICP crises in 11 patients. ICP decreased following these doses and threshold-subtracted CPP rose., Conclusions: In this retrospective, exploratory study, ICP did not increase following ketamine administration. In the setting of a guidelines-based protocol, ketamine was associated with a reduction in ICP during ICP crises. If these findings are reproduced in a larger study, ketamine may warrant consideration as a treatment for intracranial hypertension in children with severe TBI., Competing Interests: Dr. Betters’ institution received funding from the National Institutes of Health (R61HL151951). Dr. Wellons disclosed the off-label product use of ketamine for intracranial pressure. Dr. Slaughter received funding from the Department of Pediatrics for statistical support. The remaining authors have disclosed that they do not have any potential conflicts of interest., (Copyright © 2023 by the Society of Critical Care Medicine and Wolters Kluwer Health, Inc. All Rights Reserved.)

Introduction: Intrauterine myelomeningocele repair (IUMR) and postnatal myelomeningocele repair (PNMR) differ in terms of both setting and surgical technique. A simplified technique in IUMR, in which a dural onlay is used followed by skin closure, has been adopted at our institution. The goal of this study was to compare the rates of clinical tethering in IUMR and PNMR patients, as well as to evaluate the appearance on MRI., Methods: We conducted a retrospective review of 36 patients with MMC repaired at our institution, with 2:1 PNMR to IUMR matching based on lesion level. A pediatric neuroradiologist blinded to the clinical details reviewed the patients' lumbar spine MRIs for the distance from neural tissue to skin and the presence or absence of a syrinx. An EMR review was then done to evaluate for detethering procedures and need for CSF diversion., Results: Mean age at MRI was 4.0 years and mean age at last follow-up was 6.1 years, with no significant difference between the PNMR and IUMR groups. There was no significant difference between groups in the distance from neural tissue to skin (PNMR 13.5 mm vs IUMR 17.6 mm; p = 0.5). There was no difference in need for detethering operations between groups (PNMR 12.5% vs IUMR 16.7%; RR 0.75; CI 0.1-5.1)., Conclusions: There was no significant difference between postnatal- and intrauterine-repaired myelomeningocele on MRI or in need for detethering operations. These results imply that a more straightforward and time-efficient IUMR closure technique does not lead to an increased rate of tethering when compared to the multilayered PNMR., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The current nomenclature of Chiari malformations includes the standard designations, Chiari 1-4, which were described by Hans Chiari in the late nineteenth century, and more recent additions, Chiari 0, 0.5, and 1.5, which emerged when the standard nomenclature failed to include important anatomical variations. The authors describe these entities and propose that to best optimize clinical care and research, it would be wise to place less focus on the eponyms and more effort on developing a descriptive or pathophysiological nomenclature., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Children with acute neurologic illness are at high risk of mortality and long-term neurologic disability. Severe traumatic brain injury, cardiac arrest, stroke, and central nervous system infection are often complicated by cerebral hypoxia, hypoperfusion, and edema, leading to secondary neurologic injury and worse outcome. Owing to the paucity of targeted neuroprotective therapies for these conditions, management emphasizes close physiologic monitoring and supportive care. In this review, we will discuss advanced neurologic monitoring strategies in pediatric acute neurologic illness, emphasizing the physiologic concepts underlying each tool. We will also highlight recent innovations including novel monitoring modalities, and the application of neurologic monitoring in critically ill patients at risk of developing neurologic sequelae., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: Burnout is experienced by up to two thirds of neurosurgery residents. Team sport participation as an adolescent protects against adverse mental health outcomes in adulthood. The objective of this study was to determine whether high school or collegiate team sport participation is associated with improved psychological well-being during neurosurgery residency., Methods: A cross-sectional survey study of U.S. neurosurgery residents was conducted between June 2020 and February 2021. Outcomes included self-ratings of sadness, anxiety, stress, burnout, optimism, and fulfillment, on 100-point scales, which were averaged into a "Burnout Composite Score" (BCS). Respondents were grouped and compared according to their prior self-reported participation in team sports (participants vs. nonparticipants). A 3-way analysis of variance tested the effects of resident level, exercise days, and team sport participation on BCS., Results: Of 229 submitted responses, 228 (99.5%) provided complete data and 185 (81.1%) residents participated in team sports. Days/week of exercise was similar across groups (2.5 ± 1.8 vs. 2.1 ± 1.8, P = 0.20). The team sport group reported lower mean BCS (37.1 vs. 43.6 P = 0.030, Cohen d = 0.369). There was a significant interaction between prior team sport participation and exercise regimen on BCS (F [3, 211] = 3.39, P = 0.019, n 2  = 0.046), such that more exercise days per week was associated with decreased BCS for prior team sport athletes (F [3, 211] = 11.10, P < 0.0005), but not for nonparticipants (F [3, 211] = 0.476, P = 0.699). The positive impact of prior team sport participation was more pronounced for senior residents (-11.5 points, P = 0.016) than junior residents (-4.3 points, P = 0.29)., Conclusions: Prior team sport participation was associated with lower BCS among neurosurgery residents, an effect more pronounced during senior residency. Lessons imparted during early team sport experience may have profound impacts on reducing burnout throughout a 7-year neurosurgery residency., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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., (© 2021 American Neurological Association.)

We conducted PrediXcan analysis of hydrocephalus risk in ten neurological tissues and whole blood. Decreased expression of MAEL in the brain was significantly associated (Bonferroni-adjusted p < 0.05) with hydrocephalus. PrediXcan analysis of brain imaging and genomics data in the independent UK Biobank (N = 8,428) revealed that MAEL expression in the frontal cortex is associated with white matter and total brain volumes. Among the top differentially expressed genes in brain, we observed a significant enrichment for gene-level associations with these structural phenotypes, suggesting an effect on disease risk through regulation of brain structure and integrity. We found additional support for these genes through analysis of the choroid plexus transcriptome of a murine model of hydrocephalus. Finally, differential protein expression analysis in patient cerebrospinal fluid recapitulated disease-associated expression changes in neurological tissues, but not in whole blood. Our findings provide convergent evidence highlighting the importance of tissue-specific pathways and mechanisms in the pathophysiology of hydrocephalus., Competing Interests: Declaration of interests E.R.G. receives an honorarium from the journal Circulation Research of the American Heart Association as a member of the Editorial Board., (Copyright © 2021 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Preterm infants with post-hemorrhagic hydrocephalus (PHH) are often treated with temporizing measures such as ventricular access devices (VADs) in order to drain cerebrospinal fluid (CSF) prior to permanent diversion with ventriculoperitoneal shunt (VPS) placement., Local Problem: There is little consensus on the timing and management of VADs and VPSs. This leads to marked practice variations among treating services that can adversely affect patient outcomes., Methods: This is a quality improvement study evaluating practices from February 2011 to September 2017 including infants with PHH in a single level IV NICU., Interventions: A multidisciplinary team created a local clinical pathway modified from the Hydrocephalus Clinical Research Network's Shunting Outcomes in Post-Hemorrhagic Hydrocephalus protocol to manage infants with PHH. Methods of CSF diversion and shunt timing were based on weight. Neonatal care providers performed VAD aspiration; timing was guided by imaging and clinical exam criteria. Surgical procedures were performed in the NICU., Results: There were 78 patients eligible for the study. Prior to pathway implementation, infections occurred in 4% of VAD and 3% of VPS patients. There have been no infections since inception of the pathway. With pathway implementation, treatment compliance improved from 55 to 86% while conversion compliance rate improved from 89 to 100%., Conclusions: Standardization of care for PHH infants leads to improvement in patient outcomes such as a decrease in time to VAD placement. Reservoir aspirations by the neonatology team did not result in an increase in infection rate.

Background: Preclinical neurosurgery electives have been shown to increase student familiarity with neurosurgery, yet the impact on students without a home neurosurgery program is unknown. We conducted a preclinical neurosurgery elective in a mixed cohort of students with and without home neurosurgery programs to 1) evaluate changes in neurosurgery perceptions, 2) discern differences between cohorts, and 3) identify important factors in those considering neurosurgery., Methods: A yearly elective was offered to students at Vanderbilt University School of Medicine (VUSM; home program) or Meharry Medical College (MMC; no home program) from 2017 to 2018. Each class included a student-led presentation, faculty academic lecture, and faculty round-table discussion. Precourse and postcourse surveys were completed., Results: Thirty-two students completed the course. VUSM students (n = 15) showed no changes in initial perceptions, whereas MMC students (n = 17) had multiple improved perceptions, including collegiality (P = 0.001) and family achievability (P = 0.010), and believed residency to be less rigorous than their initial perceptions (P = 0.046). Fourteen students (44%) showed an increase in the likelihood of considering a neurosurgical career; eight (57%) were MMC students. These 14 students had improved perceptions of neurosurgery as less emotionally draining (P = 0.042), with favorable collegiality (P = 0.003) and work/life balance (P = 0.001) but did not believe residency to be less difficult (P = 0.102) or have added financial security (P = 0.380)., Conclusions: Early exposure to neurosurgery at medical schools without home programs through preclinical electives may improve students' perceptions of neurosurgery, provide valuable information about the benefits and rigors of neurosurgery, and allow students to make informed decisions about further pursuit of neurosurgery., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Textual analysis of obituaries provides insight into the shared values of a profession or community. Neurosurgeon obituaries are frequently published in both the medical literature and the lay press, but the content of these works has never been analyzed., Methods: Using obituary pieces from Neurosurgery, Journal of Neurosurgery, and the New York Times, frequent terms were quantified through preliminary text analysis to derive the relative importance of concepts such as innovation, research, training and family. The sentiment of these obituaries was qualitatively reviewed to approximate perceptions of neurosurgical legacy within the profession and the general public., Results: Thirty relevant obituaries with full text available were identified in the Journal of Neurosurgery, 14 were identified in Neurosurgery, and 23 were identified in the New York Times. Both neurosurgical journals and lay press articles relied on linear narratives, with greater emphasis on professional leadership and residency training in neurosurgical journals and proportionally greater mention of family in the lay press., Conclusions: Our preliminary text analysis of neurosurgeon obituaries suggest what values are shared among the professional community and general public regarding the legacy of a neurosurgeon., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background and Purpose: The purpose of the study is to characterize diffusion tensor imaging indices in the developing spinal cord, evaluating differences based on age and cord region. Describing the progression of DTI indices in the pediatric cord increases our understanding of spinal cord development., Materials and Methods: A retrospective analysis was performed on DTI acquired in 121 pediatric patients (mean, 8.6 years; range, 0.3-18.0 years) at Monroe Carell Jr. Children's Hospital at Vanderbilt from 2017 to 2018. Diffusion-weighted images (15 directions; b = 750 s/mm 2 ; slice thickness, 5 mm; in-plane resolution, 1.0 × 1.0 mm 2 ) were acquired on a 3T scanner in the cervicothoracic and/or thoracolumbar cord. Manual whole-cord segmentation was performed. Images were masked and further segmented into cervical, upper thoracic, thoracolumbar, and conus regions. Analyses of covariance were performed for each DTI-derived index to investigate how age affects diffusion across cord regions, and 95% confidence intervals were calculated across age for each derived index and region. Post hoc testing was performed to analyze regional differences., Results: Analyses of covariance revealed significant correlations of age with axial diffusivity, mean diffusivity, and fractional anisotropy (all, P < .001). There were also significant differences among cord regions for axial diffusivity, radial diffusivity, mean diffusivity, and fractional anisotropy (all, P < .001)., Conclusions: This research demonstrates that diffusion evolves in the pediatric spinal cord during development, dependent on both cord region and the diffusion index of interest. Future research could investigate how diffusion may be affected by common pediatric spinal pathologies., (© 2019 by American Journal of Neuroradiology.)

Introduction: Myelomeningocele is the most common open neural tube defect. A quadruple rotation-VY advancement flap (butterfly flap) was recently reported for closure of large myelomeningocele defects; however, no series has been reproduced to evaluate this technique. The objective of this study was to describe our experience with this technique., Materials and Methods: We reviewed all infants born with large myelomeningocele defects who underwent butterfly flap closure over a 2-year period. Demographics, defect size, operative details, and complications were used to generate descriptive statistics., Results: From June 2015 to January 2018, 7 infants met inclusion criteria. Mean defect width was 52% ± 0.11 of the back, representing 21% ± 0.09 of the total back area. Only 1 child had central breakdown. All patients had some peripheral skin dehiscence that occurred on postoperative day 12 ± 7, and these were treated with outpatient wound care. Four patients returned to the operating room for dehiscence electively. There were no incidences of total flap loss. There were no cases of meningitis or myelomeningocele dehiscence. All patients had successful closure of their myelomeningocele without the use of skin grafts., Conclusions: The butterfly flap is able to close large myelomeningocele defects and has the potential to improve contour. There are minor wound-healing complications, but in the rare event of central dehiscence, quadruple rotation-VY advancement flaps can be re-advanced. In all cases, a large myelomeningocele was successfully reconstructed with robust full-thickness flaps, and there was no need for skin grafting of donor sites.

Background: Neurosurgical educators strive to identify the best applicants, yet formal study of resident selection has proved difficult. We conducted a systematic review to answer the following question: What objective and subjective preresidency factors predict resident success?, Methods: PubMed, ProQuest, Embase, and the CINAHL databases were queried from 1952 to 2015 for literature reporting the impact of preresidency factors (PRFs) on outcomes of residency success (RS), among neurosurgery and all surgical subspecialties. Due to heterogeneity of specialties and outcomes, a qualitative summary and heat map of significant findings were constructed., Results: From 1489 studies, 21 articles met inclusion criteria, which evaluated 1276 resident applicants across five surgical subspecialties. No neurosurgical studies met the inclusion criteria. Common objective PRFs included standardized testing (76%), medical school performance (48%), and Alpha Omega Alpha (43%). Common subjective PRFs included aggregate rank scores (57%), letters of recommendation (38%), research (33%), interviews (19%), and athletic or musical talent (19%). Outcomes of RS included faculty evaluations, in-training/board exams, chief resident status, and research productivity. Among objective factors, standardized test scores correlated well with in-training/board examinations but poorly correlated with faculty evaluations. Among subjective factors, aggregate rank scores, letters of recommendation, and athletic or musical talent demonstrated moderate correlation with faculty evaluations., Conclusion: Standardized testing most strongly correlated with future examination performance but correlated poorly with faculty evaluations. Moderate predictors of faculty evaluations were aggregate rank scores, letters of recommendation, and athletic or musical talent. The ability to predict success of neurosurgical residents using an evidence-based approach is limited, and few factors have correlated with future resident performance. Given the importance of recruitment to the greater field of neurosurgery, these data provide support for a national, prospective effort to improve the study of neurosurgery resident selection., (Copyright © 2017 Elsevier Inc. All rights reserved.)

The authors present a case of monozygotic twins with hereditary hemorrhagic telangiectasia (HHT) who experienced cerebral arteriovenous malformation (AVM) hemorrhage at a very young age. The clinical variables influencing HHT-related AVM rupture are discussed, and questions surrounding the timing of screening and intervention are explored. This is only the second known case of monozygotic HHT twins published in the medical literature, and the youngest pair of first-degree relatives to experience AVM-related cerebral hemorrhage. Evidence guiding the screening and management of familial HHT is lacking, and cases such as this underscore the need for objective and validated protocols.

Objective: To quantify the proportion of academic neurosurgeons practicing in the United States who acquired residency training outside of the United States and compare their training backgrounds and academic success with those who received their residency training in the United States., Methods: We identified 1338 clinically active academic neurosurgeons from 104 programs that participated in the neurosurgery residency match in the United States in January-February 2015. Their training backgrounds, current academic positions, and history of National Institutes of Health (NIH) grant awards between 2005 and 2014 were retrieved from publicly accessible sources., Results: Eighty-four U.S. academic neurosurgeons (6.3%) received their residency training in 20 different countries outside of the United States/Puerto Rico, representing all major regions of the world. The majority trained in Canada (n = 48). We found no major differences between the foreign-trained and U.S.-trained neurosurgeons in male:female ratio, year of starting residency, proportion with positions in medical schools ranked in the top 15 by the U.S. News and World Report, general distribution of academic positions, and proportion with an NIH grant. Compared with U.S.-trained academic neurosurgeons, foreign-trained academic neurosurgeons had a significantly higher proportion of Ph.D. degrees (32.1% vs. 12.3%; P < 0.0001) and held more associate professorships (34.5% vs. 23.1%; P = 0.02). The academic practices of the foreign-trained neurosurgeons were widely distributed throughout the United States., Conclusions: A small group of U.S. academic neurosurgeons (6.3%) have acquired residency training outside of the United States, representing all major regions of the world. Their general demographic data and academic accomplishments are comparable to those of U.S.-trained neurosurgeons., (Copyright © 2017 Elsevier Inc. All rights reserved.)

OBJECTIVE Previous Hydrocephalus Clinical Research Network (HCRN) retrospective studies have shown a 15% difference in rates of conversion to permanent shunts with the use of ventriculosubgaleal shunts (VSGSs) versus ventricular reservoirs (VRs) as temporization procedures in the treatment of hydrocephalus due to high-grade intraventricular hemorrhage (IVH) of prematurity. Further research in the same study line revealed a strong influence of center-specific decision-making on shunt outcomes. The primary goal of this prospective study was to standardize decision-making across centers to determine true procedural superiority, if any, of VSGS versus VR as a temporization procedure in high-grade IVH of prematurity. METHODS The HCRN conducted a prospective cohort study across 6 centers with an approximate 1.5- to 3-year accrual period (depending on center) followed by 6 months of follow-up. Infants with premature birth, who weighed less than 1500 g, had Grade 3 or 4 IVH of prematurity, and had more than 72 hours of life expectancy were included in the study. Based on a priori consensus, decisions were standardized regarding the timing of initial surgical treatment, upfront shunt versus temporization procedure (VR or VSGS), and when to convert a VR or VSGS to a permanent shunt. Physical examination assessment and surgical technique were also standardized. The primary outcome was the proportion of infants who underwent conversion to a permanent shunt. The major secondary outcomes of interest included infection and other complication rates. RESULTS One hundred forty-five premature infants were enrolled and met criteria for analysis. Using the standardized decision rubrics, 28 infants never reached the threshold for treatment, 11 initially received permanent shunts, 4 were initially treated with endoscopic third ventriculostomy (ETV), and 102 underwent a temporization procedure (36 with VSGSs and 66 with VRs). The 2 temporization cohorts were similar in terms of sex, race, IVH grade, head (orbitofrontal) circumference, and ventricular size at temporization. There were statistically significant differences noted between groups in gestational age, birth weight, and bilaterality of clot burden that were controlled for in post hoc analysis. By Kaplan-Meier analysis, the 180-day rates of conversion to permanent shunts were 63.5% for VSGS and 74.0% for VR (p = 0.36, log-rank test). The infection rate for VSGS was 14% (5/36) and for VR was 17% (11/66; p = 0.71). The overall compliance rate with the standardized decision rubrics was noted to be 90% for all surgeons. CONCLUSIONS A standardized protocol was instituted across all centers of the HCRN. Compliance was high. Choice of temporization techniques in premature infants with IVH does not appear to influence rates of conversion to permanent ventricular CSF diversion. Once management decisions and surgical techniques are standardized across HCRN sites, thus minimizing center effect, the observed difference in conversion rates between VSGSs and VRs is mitigated.

OBJECTIVE The authors aimed to provide an updated and consolidated report on the epidemiology, management, and functional outcome of cases of myelomeningocele (MMC) in patients with scoliosis. METHODS A comprehensive literature search was performed using MEDLINE, Embase, Google Scholar, and the Cochrane Database of Systematic Reviews on cases of MMC in patients with scoliosis between 1980 and 2016. The initial search yielded 670 reports. After removing duplicates and applying inclusion criteria, we included 32 full-text original articles in this study. RESULTS Pooled statistical analysis of the included articles revealed the prevalence of scoliosis in MMC patients to be 53% (95% CI 0.42-0.64). Slightly more females (56%) are affected with both MMC and scoliosis than males. Motor level appears to be a significant predictor of prevalence, but not severity, of scoliosis in MMC patients. Treatment options for these patients include tethered cord release (TCR) and fusion surgeries. Curvature improvement and stabilization after TCR may be limited to patients with milder (< 50°) curves. Meanwhile, more aggressive fusion procedures such as a combined anterior-posterior approach may result in more favorable long-term scoliosis correction, albeit with greater complication rates. Quality of life metrics including ambulatory status and sitting stability are influenced by motor level of the lesion as well as the degree of the scoliosis curvature. CONCLUSIONS Scoliosis is among the most common and challenging comorbidities from which patients with MMC suffer. Although important epidemiological and management trends are evident, larger, prospective studies are needed to discover ways to more accurately counsel and more optimally treat these patients.

OBJECTIVE Up to one-third of patients with a posterior fossa brain tumor (PFBT) will experience persistent hydrocephalus mandating permanent CSF diversion. The optimal hydrocephalus treatment modality is unknown; the authors sought to compare the durability between endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunt (VPS) therapy in the pediatric population. METHODS The authors conducted a systematic review of articles indexed in PubMed between 1986 and 2016 describing ETV and/or VPS treatment success/failure and time-to-failure rate in patients < 19 years of age with hydrocephalus related to a PFBT. Additionally, the authors conducted a retrospective review of their institutional series of PFBT patients requiring CSF diversion. Patient data from the systematic review and from the institutional series were aggregated and a time-to-failure analysis was performed comparing ETV and VPS using the Kaplan-Meier method. RESULTS A total of 408 patients were included from 12 studies and the authors' institutional series: 284 who underwent ETV and 124 who underwent VPS placement. The analysis included uncontrolled studies with variable method and timing of CSF diversion and were subject to surgeon bias. No significant differences between cohorts were observed with regard to age, sex, tumor grade or histology, metastatic status, or extent of resection. The cumulative failure rate of ETV was 21%, whereas that of VPS surgery was 29% (p = 0.105). The median time to failure was earlier for ETV than for VPS surgery (0.82 [IQR 0.2-1.8] vs 4.7 months [IQR 0.3-5.7], p = 0.03). Initially the ETV survival curve dropped sharply and then stabilized around 2 months. The VPS curve fell gradually but eventually crossed below the ETV curve at 5.7 months. Overall, a significant survival advantage was not demonstrated for one procedure over the other (p = 0.21, log-rank). However, postoperative complications were higher following VPS (31%) than ETV (17%) (p = 0.012). CONCLUSIONS ETV failure occurred sooner than VPS failure, but long-term treatment durability may be higher for ETV. Complications occurred more commonly with VPS than with ETV. Limited clinical conclusions are drawn using this methodology; the optimal treatment for PFBT-related hydrocephalus warrants investigation through prospective studies.

Background: Antiepileptic drugs (AEDs) are frequently administered prophylactically to mitigate seizures following craniotomy for brain tumor resection. However, conflicting evidence exists regarding the efficacy of AEDs, and their influence on surgery-related outcomes is limited., Objective: To evaluate the influence of perioperative AEDs on postoperative seizure rate and hospital-reported quality metrics., Methods: A retrospective cohort study was conducted, incorporating all adult patients who underwent craniotomy for glioma resection at our institution between 1999 and 2014. Patients in 2 cohorts-those receiving and those not receiving prophylactic AEDs-were compared on the incidence of postoperative seizures and several hospital quality metrics including length of stay, discharge status, and use of hospital resources., Results: Among 342 patients with glioma undergoing cytoreductive surgery, 301 (88%) received AED prophylaxis and 41 (12%) did not. Seventeen patients (5.6%) in the prophylaxis group developed a seizure within 14 days of surgery, compared with 1 (2.4%) in the standard group (OR = 2.2, 95% CI [0.3-17.4]). Median hospital and intensive care unit lengths of stay were similar between the cohorts. There was also no difference in the rate at which patients presented within 90 days postoperatively to the emergency department or required hospital readmission. In addition, the rate of hospital resource consumption, including electroencephalogram and computed tomography scan acquisition, and neurology consultation, was similar between both groups., Conclusion: The administration of prophylactic AEDs following glioma surgery did not influence the rate of perioperative seizures, nor did it reduce healthcare resource consumption. The role of perioperative seizure prophylaxis should be closely reexamined, and reconsideration given to this commonplace practice., (Copyright © 2016 by the Congress of Neurological Surgeons)

Purpose: We evaluate a single-institution cohort of mothers contemporaneous with the Management of Myelomeningocele Study (MOMS) trial to determine the generalizability of MOMS results and compare shunt rates., Methods: A retrospective chart review identified patients with myelomeningocele born between 2003 and 2009. We applied MOMS eligibility criteria and compared sociodemographic variables between patients at our institution who would have been eligible or ineligible and MOMS participants. Finally, we applied the original MOMS primary outcome and the revised primary outcome to our cohort., Results: Of the 78 patients, 55 (70.5%) were eligible for the MOMS trial. Mean maternal age, race, and marital status were different from both MOMS groups. Comparing our series to MOMS postnatal shows fewer female infants (44.9 vs. 63.8%, p = 0.017) and more thoracic lesions (12.8 vs. 3.8%, p = 0.038). Shunt rates in our cohort (84.6%) were higher than MOMS prenatal and similar to MOMS postnatal (44.0 and 83.7%, respectively). Fewer children met the original primary outcome than the postnatal group (84.6 vs. 97.8%, p = 0.002). There was no significant difference between our cohort and the prenatal group (84.6 vs. 72.5%, p = 0.058). When applying the revised criteria, we find the opposite: a significant difference between local and MOMS prenatal (84.6 vs. 49.5%, p < 0.001) but no difference between the local group and MOMS postnatal (84.6 vs. 87.0%, p = 0.662)., Conclusions: Mothers in our cohort differ from mothers enrolled in MOMS via several sociodemographic factors. Baseline fetal characteristics show a significantly higher functional lesion level in between our cohort and MOMS. Treatment of hydrocephalus in our series tracks almost identically with original MOMS shunt criteria. Revision of the criteria led to greater concordance between meeting criteria and receiving a shunt in MOMS patients, but changes the results in our series.

OBJECTIVE Accurate placement of ventricular catheters may result in prolonged shunt survival, but the best target for the hole-bearing segment of the catheter has not been rigorously defined. The goal of the study was to define a target within the ventricle with the lowest risk of shunt failure. METHODS Five catheter placement variables (ventricular catheter tip location, ventricular catheter tip environment, relationship to choroid plexus, catheter tip holes within ventricle, and crosses midline) were defined, assessed for interobserver agreement, and evaluated for their effect on shunt survival in univariate and multivariate analyses. De-identified subjects from the Shunt Design Trial, the Endoscopic Shunt Insertion Trial, and a Hydrocephalus Clinical Research Network study on ultrasound-guided catheter placement were combined (n = 858 subjects, all first-time shunt insertions, all patients < 18 years old). The first postoperative brain imaging study was used to determine ventricular catheter placement for each of the catheter placement variables. RESULTS Ventricular catheter tip location, environment, catheter tip holes within the ventricle, and crosses midline all achieved sufficient interobserver agreement (κ > 0.60). In the univariate survival analysis, however, only ventricular catheter tip location was useful in distinguishing a target within the ventricle with a survival advantage (frontal horn; log-rank, p = 0.0015). None of the other catheter placement variables yielded a significant survival advantage unless they were compared with catheter tips completely not in the ventricle. Cox regression analysis was performed, examining ventricular catheter tip location with age, etiology, surgeon, decade of surgery, and catheter entry site (anterior vs posterior). Only age (p < 0.001) and entry site (p = 0.005) were associated with shunt survival; ventricular catheter tip location was not (p = 0.37). Anterior entry site lowered the risk of shunt failure compared with posterior entry site by approximately one-third (HR 0.65, 95% CI 0.51-0.83). CONCLUSIONS This analysis failed to identify an ideal target within the ventricle for the ventricular catheter tip. Unexpectedly, the choice of an anterior versus posterior catheter entry site was more important in determining shunt survival than the location of the ventricular catheter tip within the ventricle. Entry site may represent a modifiable risk factor for shunt failure, but, due to inherent limitations in study design and previous clinical research on entry site, a randomized controlled trial is necessary before treatment recommendations can be made.

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., (Copyright © 2016 Elsevier Inc. All rights reserved.)

OBJECTIVE Endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) offers an alternative to shunt treatment for infantile hydrocephalus. Diagnosing treatment failure is dependent on infantile hydrocephalus metrics, including head circumference, fontanel quality, and ventricle size. However, it is not clear to what degree these metrics should be expected to change after ETV/CPC. Using these clinical metrics, the authors present and analyze the decision making in cases of ETV/CPC failure. METHODS Infantile hydrocephalus metrics, including bulging fontanel, head circumference z-score, and frontal and occipital horn ratio (FOHR), were compared between ETV/CPC failures and successes. Treatment outcome predictive values of metrics individually and in combination were calculated. RESULTS Forty-four patients (57% males, median age 1.2 months) underwent ETV/CPC for hydrocephalus; of these patients, 25 (57%) experienced failure at a median time of 51 days postoperatively. Patients experiencing failure were younger than those experiencing successful treatment (0.8 vs 3.9 months, p = 0.01). During outpatient follow-up, bulging anterior fontanel, progressive macrocephaly, and enlarging ventricles each demonstrated a positive predictive value (PPV) of no less than 71%, but a bulging anterior fontanel remained the most predictive indicator of ETV/CPC failure, with a PPV of 100%, negative predictive value of 73%, and sensitivity of 72%. The highest PPVs and specificities existed when the clinical metrics were present in combination, although sensitivities decreased expectedly. Only 48% of failures were diagnosed on the basis all 3 hydrocephalus metrics, while only 37% of successes were negative for all 3 metrics. In the remaining 57% of patients, a diagnosis of success or failure was made in the presence of discordant data. CONCLUSIONS Successful ETV/CPC for infantile hydrocephalus was evaluated in relation to fontanel status, head growth, and change in ventricular size. In most patients, a designation of failure or success was made in the setting of discordant data.

OBJECTIVE Endoscopic third ventriculostomy (ETV) is now established as a viable treatment option for a subgroup of children with hydrocephalus. Here, the authors report prospective, multicenter results from the Hydrocephalus Clinical Research Network (HCRN) to provide the most accurate determination of morbidity, complication incidence, and efficacy of ETV in children and to determine if intraoperative predictors of ETV success add substantially to preoperative predictors. METHODS All children undergoing a first ETV (without choroid plexus cauterization) at 1 of 7 HCRN centers up to June 2013 were included in the study and followed up for a minimum of 18 months. Data, including detailed intraoperative data, were prospectively collected as part of the HCRN's Core Data Project and included details of patient characteristics, ETV failure (need for repeat hydrocephalus surgery), and, in a subset of patients, postoperative complications up to the time of discharge. RESULTS Three hundred thirty-six eligible children underwent initial ETV, 18.8% of whom had undergone shunt placement prior to the ETV. The median age at ETV was 6.9 years (IQR 1.7-12.6), with 15.2% of the study cohort younger than 12 months of age. The most common etiologies were aqueductal stenosis (24.8%) and midbrain or tectal lesions (21.2%). Visible forniceal injury (16.6%) was more common than previously reported, whereas severe bleeding (1.8%), thalamic contusion (1.8%), venous injury (1.5%), hypothalamic contusion (1.5%), and major arterial injury (0.3%) were rare. The most common postoperative complications were CSF leak (4.4%), hyponatremia (3.9%), and pseudomeningocele (3.9%). New neurological deficit occurred in 1.5% cases, with 0.5% being permanent. One hundred forty-one patients had documented failure of their ETV requiring repeat hydrocephalus surgery during follow-up, 117 of them during the first 6 months postprocedure. Kaplan-Meier rates of 30-day, 90-day, 6-month, 1-year, and 2-year failure-free survival were 73.7%, 66.7%, 64.8%, 61.7%, and 57.8%, respectively. According to multivariate modeling, the preoperative ETV Success Score (ETVSS) was associated with ETV success (p < 0.001), as was the intraoperative ability to visualize a "naked" basilar artery (p = 0.023). CONCLUSIONS The authors' documented experience represents the most detailed account of ETV results in North America and provides the most accurate picture to date of ETV success and complications, based on contemporaneously collected prospective data. Serious complications with ETV are low. In addition to the ETVSS, visualization of a naked basilar artery is predictive of ETV success.

OBJECTIVE Disparities in surgical access and timing to care result from a combination of complex patient, social, and institutional factors. Due to the perception of delayed presentation for overall health care services and treatment in African American patients on the part of the senior author, this study was designed to identify and quantify these differences in access and care between African American and Caucasian children with craniosynostosis. In addition, hypotheses regarding reasons for this difference are discussed. METHODS A retrospective study was conducted of 132 children between the ages of 0 and 17 years old who previously underwent operations for craniosynostosis at a tertiary pediatric care facility between 2010 and 2013. Patient and family characteristics, age at surgical consultation and time to surgery, and distance to primary care providers and the tertiary center were recorded and analyzed. RESULTS Of the 132 patients in this cohort, 88% were Caucasian and 12% were African American. The median patient age was 5 months (interquartile range [IQR] 2-8 months). African Americans had a significantly greater age at consult compared with Caucasians (median 341 days [IQR 192-584 days] vs median 137 days [IQR 62-235 days], respectively; p = 0.0012). However, after being evaluated in consultation, there was no significant difference in time to surgery between African American and Caucasian patients (median 56 days [IQR 36-98 days] vs median 64 days [IQR 43-87 days], respectively). Using regression analysis, race and type of synostoses were found to be significantly associated with a longer wait time for surgical consultation (p = 0.01 and p = 0.04, respectively, using cutoff points of ≤ 180 days vs > 180 days). Distance traveled to primary care physicians and to the tertiary care facility did not significantly differ between groups. Other factors such as parental education, insurance type, household income, and referring physician type also showed no significant difference between racial groups. CONCLUSIONS This study identified a correlation between race and age at consultation, but no association with time to surgery, distance, or family characteristics such as household income, parental education, insurance type, and referring physician type. This finding implies that delays in early health-seeking behaviors and subsequent referral to surgical specialists from primary care providers are the main reason for this delay among African American craniofacial patients. Future studies should focus on further detail in regards to these barriers, and educational efforts should be designed for the community and the health care personnel caring for them.

Background: Traumatic brain injury (TBI) is a common condition affecting children all over the world, and it represents a global public health concern. It is unclear how geopolitical, societal, and ethnic differences may influence the nature of TBI among children., Methods: A comprehensive literature search was conducted incorporating studies with hospital-, regional-, or country-specific pediatric TBI epidemiology data published between 1995 and 2015. Incidence, age, severity, mechanism of injury, and other relevant injury characteristics were extracted and compared across diverse geographic regions., Results: Thirty articles met inclusion criteria, incorporating TBI data from more than 165,000 children on 5 continents. The worldwide incidence of pediatric TBI ranges broadly and varies greatly by country, with most reporting a range between 47 and 280 per 100,000 children. After the age of 3, male children suffered higher rates of TBI than females. A bimodal age distribution is often described, with very young children (0-2 years) and adolescents (15-18) more commonly injured. Mild TBI (Glasgow Coma Scale ≥13) constitutes more than 80% of injuries, and up to 90% of all injuries are associated with negative imaging. Only a small fraction (<10%) requires surgical intervention. Independent of country or region of origin, the vast majority of children suffering TBI achieve a good clinical outcome. Hospital admission rates vary widely, with U.S. patients more commonly admitted than those from other countries. Falls and motor vehicle collisions (MVCs) represent the majority of injury mechanisms. In Africa and Asia, pedestrians were most commonly injured in MVCs, while vehicle occupants were more likely involved among Australian, European, and U.S., Populations: For children, nonaccidental trauma was prevalent in developing and developed nations alike., Conclusions: TBI is a relatively common entity stretching across traditional geographic and demographic boundaries and affecting pediatric populations worldwide. Continued civil infrastructure development and public health policy reforms may help to reduce the societal burden of pediatric TBI., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: This study aimed to investigate the functional outcomes of infants who underwent neurotization for shoulder abduction and elbow flexion in Narakas grade 1 birth-related brachial plexus palsy (BRBPP) and compare this cohort to children who progressed past the point of needing intervention., Methods: A cohort study was conducted at a single center between 1999 and 2010. Two-hundred and eight infants were identified with BRBPP that presented for neurosurgical care as infants. Of those, 38 (18 %) received neurosurgical intervention with approximate 2-year follow-up. Only infants undergoing cranial nerve XI to suprascapular nerve neurotization for shoulder abduction (SA) weakness and medial pectoral nerve to musculocutaneous nerve neurotization for elbow flexion (EF) weakness were included. In addition, 30 infants who improved past the need for surgical intervention and had been followed for close to 24 months were identified for comparison. Descriptive statistics and exploratory analysis were performed using SAS 9.2 and JMP 9.0.2., Results: Shoulder abduction For SA, there were no differences in age at presentation between the operative (6-9 months) and non-operative (5-9 months) groups (p = 0.99). Infants in the operative cohort had significantly worse initial function (p = 0.008). At 2-year follow-up, the two groups had become similar (p = 1.0). Elbow flexion For EF, there were no differences in age at presentation between the operative (6-8 months) and non-operative (5-8.5 months) groups (p = 0.98). Infants in the operative cohort had significantly worse initial function (p = 0.002). At 2-year follow-up, those two groups had become similar (p = 0.26)., Conclusions: Infants undergoing neurotization for Narakas grade 1 brachial plexus injury had similar long-term function to those who had improved and never required surgery. The preoperative exam findings were significantly different between the intervened and non-intervened groups, while the postoperative exam findings were not.

OBJECTIVE Administrative billing data may facilitate large-scale assessments of treatment outcomes for pediatric Chiari malformation Type I (CM-I). Validated International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) code algorithms for identifying CM-I surgery are critical prerequisites for such studies but are currently only available for adults. The objective of this study was to validate two ICD-9-CM code algorithms using hospital billing data to identify pediatric patients undergoing CM-I decompression surgery. METHODS The authors retrospectively analyzed the validity of two ICD-9-CM code algorithms for identifying pediatric CM-I decompression surgery performed at 3 academic medical centers between 2001 and 2013. Algorithm 1 included any discharge diagnosis code of 348.4 (CM-I), as well as a procedure code of 01.24 (cranial decompression) or 03.09 (spinal decompression or laminectomy). Algorithm 2 restricted this group to the subset of patients with a primary discharge diagnosis of 348.4. The positive predictive value (PPV) and sensitivity of each algorithm were calculated. RESULTS Among 625 first-time admissions identified by Algorithm 1, the overall PPV for CM-I decompression was 92%. Among the 581 admissions identified by Algorithm 2, the PPV was 97%. The PPV for Algorithm 1 was lower in one center (84%) compared with the other centers (93%-94%), whereas the PPV of Algorithm 2 remained high (96%-98%) across all subgroups. The sensitivity of Algorithms 1 (91%) and 2 (89%) was very good and remained so across subgroups (82%-97%). CONCLUSIONS An ICD-9-CM algorithm requiring a primary diagnosis of CM-I has excellent PPV and very good sensitivity for identifying CM-I decompression surgery in pediatric patients. These results establish a basis for utilizing administrative billing data to assess pediatric CM-I treatment outcomes.

OBJECTIVE Chiari malformation Type I (CM-I) is a common and often debilitating pediatric neurological disease. However, efforts to guide preoperative counseling and improve outcomes research are impeded by reliance on small, single-center studies. Consequently, the objective of this study was to investigate CM-I surgical outcomes using population-level administrative billing data. METHODS The authors used Healthcare Cost and Utilization Project State Inpatient Databases (SID) to study pediatric patients undergoing surgical decompression for CM-I from 2004 to 2010 in California, Florida, and New York. They assessed the prevalence and influence of preoperative complex chronic conditions (CCC) among included patients. Outcomes included medical and surgical complications within 90 days of treatment. Multivariate logistic regression was used to identify risk factors for surgical complications. RESULTS A total of 936 pediatric CM-I surgeries were identified for the study period. Overall, 29.2% of patients were diagnosed with syringomyelia and 13.7% were diagnosed with scoliosis. Aside from syringomyelia and scoliosis, 30.3% of patients had at least 1 CCC, most commonly neuromuscular (15.2%) or congenital or genetic (8.4%) disease. Medical complications were uncommon, occurring in 2.6% of patients. By comparison, surgical complications were diagnosed in 12.7% of patients and typically included shunt-related complications (4.0%), meningitis (3.7%), and other neurosurgery-specific complications (7.4%). Major complications (e.g., stroke or myocardial infarction) occurred in 1.4% of patients. Among children with CCCs, only comorbid hydrocephalus was associated with a significantly increased risk of surgical complications (OR 4.5, 95% CI 2.5-8.1). CONCLUSIONS Approximately 1 in 8 pediatric CM-I patients experienced a surgical complication, whereas medical complications were rare. Although CCCs were common in pediatric CM-I patients, only hydrocephalus was independently associated with increased risk of surgical events. These results may inform patient counseling and guide future research efforts.

OBJECT The rate of CSF shunt failure remains unacceptably high. The Hydrocephalus Clinical Research Network (HCRN) conducted a comprehensive prospective observational study of hydrocephalus management, the aim of which was to isolate specific risk factors for shunt failure. METHODS The study followed all first-time shunt insertions in children younger than 19 years at 6 HCRN centers. The HCRN Investigator Committee selected, a priori, 21 variables to be examined, including clinical, radiographic, and shunt design variables. Shunt failure was defined as shunt revision, subsequent endoscopic third ventriculostomy, or shunt infection. Important a priori-defined risk factors as well as those significant in univariate analyses were then tested for independence using multivariate Cox proportional hazard modeling. RESULTS A total of 1036 children underwent initial CSF shunt placement between April 2008 and December 2011. Of these, 344 patients experienced shunt failure, including 265 malfunctions and 79 infections. The mean and median length of follow-up for the entire cohort was 400 days and 264 days, respectively. The Cox model found that age younger than 6 months at first shunt placement (HR 1.6 [95% CI 1.1-2.1]), a cardiac comorbidity (HR 1.4 [95% CI 1.0-2.1]), and endoscopic placement (HR 1.9 [95% CI 1.2-2.9]) were independently associated with reduced shunt survival. The following had no independent associations with shunt survival: etiology, payer, center, valve design, valve programmability, the use of ultrasound or stereotactic guidance, and surgeon experience and volume. CONCLUSIONS This is the largest prospective study reported on children with CSF shunts for hydrocephalus. It confirms that a young age and the use of the endoscope are risk factors for first shunt failure and that valve type has no impact. A new risk factor-an existing cardiac comorbidity-was also associated with shunt failure.

OBJECT In a previous report by the same research group (Kestle et al., 2011), compliance with an 11-step protocol was shown to reduce CSF shunt infection at Hydrocephalus Clinical Research Network (HCRN) centers (from 8.7% to 5.7%). Antibiotic-impregnated catheters (AICs) were not part of the protocol but were used off protocol by some surgeons. The authors therefore began using a new protocol that included AICs in an effort to reduce the infection rate further. METHODS The new protocol was implemented at HCRN centers on January 1, 2012, for all shunt procedures (excluding external ventricular drains [EVDs], ventricular reservoirs, and subgaleal shunts). Procedures performed up to September 30, 2013, were included (21 months). Compliance with the protocol and outcome events up to March 30, 2014, were recorded. The definition of infection was unchanged from the authors' previous report. RESULTS A total of 1935 procedures were performed on 1670 patients at 8 HCRN centers. The overall infection rate was 6.0% (95% CI 5.1%-7.2%). Procedure-specific infection rates varied (insertion 5.0%, revision 5.4%, insertion after EVD 8.3%, and insertion after treatment of infection 12.6%). Full compliance with the protocol occurred in 77% of procedures. The infection rate was 5.0% after compliant procedures and 8.7% after noncompliant procedures (p = 0.005). The infection rate when using this new protocol (6.0%, 95% CI 5.1%-7.2%) was similar to the infection rate observed using the authors' old protocol (5.7%, 95% CI 4.6%-7.0%). CONCLUSIONS CSF shunt procedures performed in compliance with a new infection prevention protocol at HCRN centers had a lower infection rate than noncompliant procedures. Implementation of the new protocol (including AICs) was associated with a 6.0% infection rate, similar to the infection rate of 5.7% from the authors' previously reported protocol. Based on the current data, the role of AICs compared with other infection prevention measures is unclear.

Objective: Exposure to surgical subspecialties is limited during the preclinical years of medical school. To offset this limitation, the authors created a neurosurgery elective for first- and second-year medical students. The objective was to provide each student with early exposure to neurosurgery by combining clinical experience with faculty discussions about the academic and personal realities of a career in neurosurgery., Methods: From 2012 to 2013, the authors offered a neurosurgery elective course to first- and second-year medical students. Each class consisted of the following: 1) peer-reviewed article analysis; 2) student presentation; 3) faculty academic lecture; 4) faculty personal lecture with question and answer period., Results: Thirty-five students were enrolled over a 2-year period. After completing the elective, students were more likely to: consider neurosurgery as a future career (P < 0.0001), perceive the personalities of attending physicians to be more collegial and friendly (P = 0.0002), perceive attending quality of life to be higher (P < 0.0001), and believe it was achievable to be a neurosurgeon and have a family (P < 0.0001). The elective did not alter students' perceived difficulty of training (P = 0.7105)., Conclusions: The neurosurgery elective course significantly increased student knowledge across several areas and changed perceptions about collegiality, quality of life, and family-work balance, while not altering the students' views about the difficulty of training. Adopting a neurosurgery elective geared towards preclinical medical students can significantly change attitudes about the field of neurosurgery and has potential to increase interest in pursuing a career in neurosurgery., (Copyright © 2016 Elsevier Inc. All rights reserved.)

OBJECT The goal of critical care in treating traumatic brain injury (TBI) is to reduce secondary brain injury by limiting cerebral ischemia and optimizing cerebral blood flow. The authors compared short-term outcomes as defined by discharge disposition and Glasgow Outcome Scale scores in children with TBI before and after the implementation of a protocol that standardized decision-making and interventions among neurosurgeons and pediatric intensivists. METHODS The authors performed a retrospective pre- and postprotocol study of 128 pediatric patients with severe TBI, as defined by Glasgow Coma Scale (GCS) scores < 8, admitted to a tertiary care center pediatric critical care unit between April 1, 2008, and May 31, 2014. The preprotocol group included 99 patients, and the postprotocol group included 29 patients. The primary outcome of interest was discharge disposition before and after protocol implementation, which took place on April 1, 2013. Ordered logistic regression was used to assess outcomes while accounting for injury severity and clinical parameters. Favorable discharge disposition included discharge home. Unfavorable discharge disposition included discharge to an inpatient facility or death. RESULTS Demographics were similar between the treatment periods, as was injury severity as assessed by GCS score (mean 5.43 preprotocol, mean 5.28 postprotocol; p = 0.67). The ordered logistic regression model demonstrated an odds ratio of 4.0 of increasingly favorable outcome in the postprotocol cohort (p = 0.007). Prior to protocol implementation, 63 patients (64%) had unfavorable discharge disposition and 36 patients (36%) had favorable discharge disposition. After protocol implementation, 9 patients (31%) had unfavorable disposition, while 20 patients (69%) had favorable disposition (p = 0.002). In the preprotocol group, 31 patients (31%) died while 6 patients (21%) died after protocol implementation (p = 0.04). CONCLUSIONS Discharge disposition and mortality rates in pediatric patients with severe TBI improved after implementation of a standardized protocol among caregivers based on best-practice guidelines.

OBJECT The purpose of this study was to design and validate a patient-reported health-related quality of life (HRQOL) instrument for pediatric Chiari Type I malformation (CM-I), the Chiari Health Index for Pediatrics (CHIP). METHODS The CHIP has 45 items with 4 components making up 2 domain scores, physical (pain frequency, pain severity, nonpain symptoms) and psychosocial; physical and psychosocial scores are combined to create an overall HRQOL score. Increasing scores (0 to 1) represent increasing HRQOL. Fifty-five patients with CM-I (mean age 12 ± 4 years, 53% male) were enrolled and completed the CHIP and Health Utilities Index Mark 3 (HUI3). Twenty-five healthy controls (mean age 11.9 ± 4 years, 40% male) also completed the CHIP. CHIP scores were compared between these groups via the Mann-Whitney U-test. For CHIP discriminative function, subscore versus presence of CM-I was compared via receiver operating characteristic curve analysis. CHIP scores in the CM-I group were stratified by symptomatology (asymptomatic, headaches, and paresthesias) and compared via Kruskal-Wallis test with Mann-Whitney U-test with Bonferroni correction (p < 0.0167). CHIP was compared with HUI3 (Health Utilities Index Mark 3) via univariate and multivariate linear regression. RESULTS CHIP physical and psychosocial subscores were, respectively, 24% and 18% lower in CM-I patients than in controls (p < 0.001); the overall HRQOL score was 23% lower as well (p < 0.001). The area under the curve (AUC) for CHIP physical subscore versus presence of CM-I was 0.809. CHIP physical subscore varied significantly with symptomatology (p = 0.001) and HUI3 pain-related quality of life (R(2) = 0.311, p < 0.001). The AUC for CHIP psychosocial subscore versus presence of CM-I was 0.754. CHIP psychosocial subscore varied significantly with HUI3 cognitive- (R(2) = 0.324, p < 0.001) and emotion-related (R(2) = 0.155, p = 0.003) quality of life. The AUC for CHIP HRQOL versus presence of CM-I was 0.820. Overall CHIP HRQOL score varied significantly with symptomatology (p = 0.001) and HUI3 multiattribute composite HRQOL score (R(2) = 0.440, p < 0.001). CONCLUSIONS The CHIP is a patient-reported, CM-I-specific HRQOL instrument, with construct validity in assessing pain-, cognitive-, and emotion-related quality of life, as well as symptomatic features unique to CM-I. It holds promise as a discriminative HRQOL index in CM-I outcomes assessment.

Object: The Management of Myelomeningocele Study (MOMS) was a multicenter randomized trial comparing the safety and efficacy of prenatal and postnatal closure of myelomeningocele. The trial was stopped early because of the demonstrated efficacy of prenatal surgery, and outcomes on 158 of 183 pregnancies were reported. Here, the authors update the 1-year outcomes for the complete trial, analyze the primary and related outcomes, and evaluate whether specific prerandomization risk factors are associated with prenatal surgery benefit., Methods: The primary outcome was a composite of fetal loss or any of the following: infant death, CSF shunt placement, or meeting the prespecified criteria for shunt placement. Primary outcome, actual shunt placement, and shunt revision rates for prenatal versus postnatal repair were compared. The shunt criteria were reassessed to determine which were most concordant with practice, and a new composite outcome was created from the primary outcome by replacing the original criteria for CSF shunt placement with the revised criteria. The authors used logistic regression to estimate whether there were interactions between the type of surgery and known prenatal risk factors (lesion level, gestational age, degree of hindbrain herniation, and ventricle size) for shunt placement, and to determine which factors were associated with shunting among those infants who underwent prenatal surgery., Results: Ninety-one women were randomized to prenatal surgery and 92 to postnatal repair. The primary outcome occurred in 73% of infants in the prenatal surgery group and in 98% in the postnatal group (p < 0.0001). Actual rates of shunt placement were only 44% and 84% in the 2 groups, respectively (p < 0.0001). The authors revised the most commonly met criterion to require overt clinical signs of increased intracranial pressure, defined as split sutures, bulging fontanelle, or sunsetting eyes, in addition to increasing head circumference or hydrocephalus. Using these modified criteria, only 3 patients in each group met criteria but did not receive a shunt. For the revised composite outcome, there was a difference between the prenatal and postnatal surgery groups: 49.5% versus 87.0% (p < 0.0001). There was also a significant reduction in the number of children who had a shunt placed and then required a revision by 1 year of age in the prenatal group (15.4% vs 40.2%, relative risk 0.38 [95% CI 0.22-0.66]). In the prenatal surgery group, 20% of those with ventricle size < 10 mm at initial screening, 45.2% with ventricle size of 10 up to 15 mm, and 79.0% with ventricle size ≥ 15 mm received a shunt, whereas in the postnatal group, 79.4%, 86.0%, and 87.5%, respectively, received a shunt (p = 0.02). Lesion level and degree of hindbrain herniation appeared to have no effect on the eventual need for shunting (p = 0.19 and p = 0.13, respectively). Similar results were obtained for the revised outcome., Conclusions: Larger ventricles at initial screening are associated with an increased need for shunting among those undergoing fetal surgery for myelomeningocele. During prenatal counseling, care should be exercised in recommending prenatal surgery when the ventricles are 15 mm or larger because prenatal surgery does not appear to improve outcome in this group. The revised criteria may be useful as guidelines for treating hydrocephalus in this group., Competing Interests: The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

OBJECT Craniofacial reconstruction surgery (CFR) is often associated with significant blood loss, coagulopathy, and perioperative blood transfusion. Due to transfusion risks, many different approaches have been used to decrease allogeneic blood transfusion for these patients during the perioperative period. Protocols have decreased blood administration during the perioperative period for many types of surgeries. The object of this study was to determine if a protocol involving blood-sparing surgical techniques and a transfusion algorithm decreased intraoperative blood transfusion and blood loss. METHODS A protocol using transfusion algorithms and implementation of blood-sparing surgical techniques for CFR was implemented at Vanderbilt University on January 1, 2013. Following Institutional Review Board approval, blood loss and transfusion data were gathered retrospectively on all children undergoing primary open CFR, using the protocol, for the calendar year 2013. This postprotocol cohort was compared with a preprotocol cohort, which consisted of all children undergoing primary open CFR during the previous calendar year, 2012. RESULTS There were 41 patients in the preprotocol and 39 in the postprotocol cohort. There was no statistical difference between the demographics of the 2 groups. When compared with the preprotocol cohort, intraoperative packed red blood cell transfusion volume decreased from 36.9 ± 21.2 ml/kg to 19.2 ± 10.9 ml/kg (p = 0.0001), whereas fresh-frozen plasma transfusion decreased from 26.8 ± 25.4 ml/kg to 1.5 ± 5.7 ml/kg (p < 0.0001) following implementation of the protocol. Furthermore, estimated blood loss decreased from 64.2 ± 32.4 ml/kg to 52.3 ± 33.3 ml/kg (p = 0.015). Use of fresh-frozen plasma in the postoperative period also decreased when compared with the period before implementation of the protocol. There was no significant difference in morbidity and mortality between the 2 groups. CONCLUSIONS The results of this study suggested that using a multidisciplinary protocol consisting of transfusion algorithms and implementation of blood-sparing surgical techniques during major CFR in pediatric patients is associated with reduced intraoperative administration of blood product, without shifting the transfusion burden to the postoperative period.

Purpose: The bidirectional Glenn (BDG) procedure involves the anastomosis of the superior vena cava (SVC) to the pulmonary artery, increasing central venous pressure (CVP). We hypothesize that this increase in CVP triggers an acute neurologic insult, leading to ventriculomegaly., Methods: In this retrospective analysis in a tertiary care children's hospital, we identified 167 patients who underwent the BDG procedure between August 2006 and July 2013. Within this initial cohort, 24 patients had head imaging (CT, MRI, or ultrasound) performed both before and after the BDG., Results: From head imaging available from these 24 patients, we measured the frontal-occipital horn ratio (FOR), a well-validated measure of lateral ventricle size. Using central venous catheter data, we assessed postoperative CVP at 12, 24, and 48 h. Paired t tests and linear regression were used to evaluate our cohort. Median age at surgery was 4.9 months. Paired analysis revealed that median FOR significantly increased between preoperative (median 0.38, IQR 0.37-0.41) and postoperative (median 0.42, IQR 0.40-0.45) head images (p = 0.005). Increasing change in FOR was associated with increased 12-h (R(2) = 0.369, p = 0.003) but not 24- or 48-h postoperative CVP., Conclusions: To our knowledge, our study is the first to demonstrate ventriculomegaly developing after the BDG. Physiologically, increasing CVP after the BDG was associated with greater change in lateral ventricle size. This supports the contention that increasing CVP produced during the BDG may damage the developing brain. This study has informed a prospective evaluation of a link between the BDG procedure and neurologic outcomes.

Background: The use of administrative billing data may enable large-scale assessments of treatment outcomes for Chiari Malformation type I (CM-1). However, to utilize such data sets, validated International Classification of Diseases, Ninth Revision (ICD-9-CM) code algorithms for identifying CM-1 surgery are needed., Objective: To validate 2 ICD-9-CM code algorithms identifying patients undergoing CM-1 decompression surgery., Methods: We retrospectively analyzed the validity of 2 ICD-9-CM code algorithms for identifying adult CM-1 decompression surgery performed at 2 academic medical centers between 2001 and 2013. Algorithm 1 included any discharge diagnosis code of 348.4 (CM-1), as well as a procedure code of 01.24 (cranial decompression) or 03.09 (spinal decompression, or laminectomy). Algorithm 2 restricted this group to patients with a primary diagnosis of 348.4. The positive predictive value (PPV) and sensitivity of each algorithm were calculated., Results: Among 340 first-time admissions identified by Algorithm 1, the overall PPV for CM-1 decompression was 65%. Among the 214 admissions identified by Algorithm 2, the overall PPV was 99.5%. The PPV for Algorithm 1 was lower in the Vanderbilt (59%) cohort, males (40%), and patients treated between 2009 and 2013 (57%), whereas the PPV of Algorithm 2 remained high (≥99%) across subgroups. The sensitivity of Algorithms 1 (86%) and 2 (83%) were above 75% in all subgroups., Conclusion: ICD-9-CM code Algorithm 2 has excellent PPV and good sensitivity to identify adult CM-1 decompression surgery. These results lay the foundation for studying CM-1 treatment outcomes by using large administrative databases.

OBJECT Osseous anomalies of the craniocervical junction are hypothesized to precipitate the hindbrain herniation observed in Chiari I malformation (CM-I). Previous work by Tubbs et al. showed that posterior angulation of the odontoid process is more prevalent in children with CM-I than in healthy controls. The present study is an external validation of that report. The goals of our study were 3-fold: 1) to externally validate the results of Tubbs et al. in a different patient population; 2) to compare how morphometric parameters vary with age, sex, and symptomatology; and 3) to develop a correlative model for tonsillar ectopia in CM-I based on these measurements. METHODS The authors performed a retrospective review of 119 patients who underwent posterior fossa decompression with duraplasty at the Monroe Carell Jr. Children's Hospital at Vanderbilt University; 78 of these patients had imaging available for review. Demographic and clinical variables were collected. A neuroradiologist retrospectively evaluated preoperative MRI examinations in these 78 patients and recorded the following measurements: McRae line length; obex displacement length; odontoid process parameters (height, angle of retroflexion, and angle of retroversion); perpendicular distance to the basion-C2 line (pB-C2 line); length of cerebellar tonsillar ectopia; caudal extent of the cerebellar tonsils; and presence, location, and size of syringomyelia. Odontoid retroflexion grade was classified as Grade 0, > 90°; Grade I,85°-89°; Grade II, 80°-84°; and Grade III, < 80°. Age groups were defined as 0-6 years, 7-12 years, and 13-17 years at the time of surgery. Univariate and multivariate linear regression analyses, Kruskal-Wallis 1-way ANOVA, and Fisher's exact test were performed to assess the relationship between age, sex, and symptomatology with these craniometric variables. RESULTS The prevalence of posterior odontoid angulation was 81%, which is almost identical to that in the previous report (84%). With increasing age, the odontoid height (p < 0.001) and pB-C2 length (p < 0.001) increased, while the odontoid process became more posteriorly inclined (p = 0.010). The pB-C2 line was significantly longer in girls (p = 0.006). These measurements did not significantly correlate with symptomatology. Length of tonsillar ectopia in pediatric CM-I correlated with an enlarged foramen magnum (p = 0.023), increasing obex displacement (p = 0.020), and increasing odontoid retroflexion (p < 0.001). CONCLUSIONS Anomalous bony development of the craniocervical junction is a consistent feature of CM-I in children. The authors found that the population at their center was characterized by posterior angulation of the odontoid process in 81% of cases, similar to findings by Tubbs et al. (84%). The odontoid process appeared to lengthen and become more posteriorly inclined with age. Increased tonsillar ectopia was associated with more posterior odontoid angulation, a widened foramen magnum, and an inferiorly displaced obex.

Background: Outcomes research on Chiari malformation type 1 (CM-1) is impeded by a reliance on small, single-center cohorts., Objective: To study the complications and resource use associated with adult CM-1 surgery using administrative data., Methods: We used a recently validated International Classification of Diseases, Ninth Revision, Clinical Modification code algorithm to retrospectively study adult CM-1 surgeries from 2004 to 2010 in California, Florida, and New York using State Inpatient Databases. Outcomes included complications and resource use within 30 and 90 days of treatment. We used multivariable logistic regression to identify risk factors for morbidity and negative binomial models to determine risk-adjusted costs., Results: We identified 1947 CM-1 operations. Surgical complications were more common than medical complications at both 30 days (14.3% vs 4.4%) and 90 days (18.7% vs 5.0%) postoperatively. Certain comorbidities were associated with increased morbidity; for example, hydrocephalus increased the risk for surgical (odds ratio [OR] = 4.51) and medical (OR = 3.98) complications. Medical but not surgical complications were also more common in older patients (OR = 5.57 for oldest vs youngest age category) and male patients (OR = 3.19). Risk-adjusted hospital costs were $22530 at 30 days and $24852 at 90 days postoperatively. Risk-adjusted 90-day costs were more than twice as high for patients experiencing surgical ($46264) or medical ($65679) complications than for patients without complications ($18880)., Conclusion: Complications after CM-1 surgery are common, and surgical complications are more frequent than medical complications. Certain comorbidities and demographic characteristics are associated with increased risk for complications. Beyond harming patients, complications are also associated with substantially higher hospital costs. These results may help guide patient management and inform decision making for patients considering surgery.

Object: The clinical significance of radiological measurements of the craniocervical junction in pediatric Chiari I malformation (CM-I) is yet to be fully established across the field. The authors examined their institutional experience with the pB-C2 line (drawn perpendicular to a line drawn between the basion and the posterior aspect of the C-2 vertebral body, at the most posterior extent of the odontoid process at the dural interface). The pB-C2 line is a measure of ventral canal encroachment, and its relationship with symptomatology and syringomyelia in pediatric CM-I was assessed., Methods: The authors performed a retrospective review of 119 patients at the Monroe Carell Jr. Children's Hospital at Vanderbilt University who underwent posterior fossa decompression with duraplasty, 78 of whom had imaging for review. A neuroradiologist retrospectively evaluated preoperative and postoperative MRI examinations performed in these 78 patients, measuring the pB-C2 line length and documenting syringomyelia. The pB-C2 line length was divided into Grade 0 (<3 mm) and Grade I (≥3 mm). Statistical analysis was performed using the t-test for continuous variables and Fisher's exact test analysis for categorical variables. Multivariate logistic and linear regression analyses were performed to assess the relationship between pB-C2 line grade and clinical variables found significant on univariate analysis, controlling for age and sex., Results: The mean patient age was 8.5 years, and the mean follow-up duration was 2.4 years. The mean pB-C2 line length was 3.5 mm (SD 2 mm), ranging from 0 to 10 mm. Overall, 65.4% of patients had a Grade I pB-C2 line. Patients with Grade I pB-C2 lines were 51% more likely to have a syrinx than those with Grade 0 pB-C2 lines (RR 1.513 [95% CI 1.024-2.90], p=0.021) and, when present, had greater syrinx reduction (3.6 mm vs 0.2 mm, p=0.002). Although there was no preoperative difference in headache incidence, postoperatively patients with Grade I pB-C2 lines were 69% more likely to have headache reduction than those with Grade 0 pB-C2 lines (RR 1.686 [95% CI 1.035-2.747], p=0.009). After controlling for age and sex, pB-C2 line grade remained an independent correlate of headache improvement and syrinx reduction., Conclusions: Ventral canal encroachment may explain the symptomatology of select patients with CM-I. The clinical findings presented suggest that patients with Grade I pB-C lines2, with increased ventral canal obstruction, may experience a higher likelihood of syrinx reduction and headache resolution from decompressive surgery with duraplasty than those with Grade 0 pB-C2 lines.

Introduction: In 2013, our institution established a multidisciplinary pediatric neurovascular conference for coordination of care. Here, we review our initial experience., Methods: Clinical and demographic data were obtained from medical records for patients presented to the pediatric neurovascular conference from April 2013 to July 2014. Patient descriptive characteristics were described by mean and standard deviation for continuous measures and by number and percent for categorical measures. Patients were secondarily stratified by lesion/disease type, and descriptive statistics were used to measure demographic and clinical variables., Results: The pediatric neurovascular conference met 26 times in the study period. Overall, 75 children were presented to the conference over a 15-month period. The mean age was 9.8 (standard deviation, 6.3) years. There were 42 (56%) male patients. These 75 children were presented a total of 112 times. There were 28 (37%) patients with history of stroke. Complex vascular lesions were the most frequently discussed entity; of 62 children (83%) with a diagnosed vascular lesion, brain arteriovenous malformation (29%), cavernous malformation (15%), and moyamoya (11%) were most common. Most discussions were for review of imaging (35%), treatment plan formulation (27%), the need for additional imaging (25%), or diagnosis (13%). Standardized care protocols for arteriovenous malformation and moyamoya were developed., Conclusion: A multidisciplinary conference among a diverse group of providers guides complex care decisions, helps standardize care protocols, promotes provider collaboration, and supports continuity of care in pediatric neurovascular disease., (Copyright © 2015 Elsevier Inc. All rights reserved.)