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OBJECTIVE Quality improvement (QI) tools are increasingly being used to calibrate healthcare quality. Achieving healthcare quality is essential, as there is a movement toward value-based healthcare delivery. Visual management, such as a living Pareto chart, is a strategy for improvement within the QI framework. The authors herein hypothesized that transparency of data through a living Pareto chart is a powerful way to improve patient outcomes and gain clinical efficiency. METHODS The authors retrospectively reviewed patient outcomes and complications; cerebrospinal fluid (CSF) leaks; shunt, baclofen, and other surgical site infections; readmission rates; and same- or next-day appointments in a cohort of patients at the Riley Hospital for Children from November 1, 2016, to May 31, 2020. Similarly, they reviewed neurosurgical outcomes and complications at a second institution, the Oklahoma Children’s Hospital, where a living Pareto chart was utilized from February 1, 2021, to March 31, 2022. The discrete frequency and rates per month of outcomes and complications were graphed on scatterplots, Pearson correlation coefficients were calculated to measure the strength of the relationship between event frequency and time, and best-fit lines illustrated the relationship between those points through the least-squares method. RESULTS At both the Riley Hospital for Children and Oklahoma Children’s Hospital, the use of a living Pareto chart to display data transparently was associated with decreasing infections, and it was associated with decreasing readmissions at Riley. On the other hand, it encouraged same- or next-day clinic appointments to be offered to patients and families. Interestingly, CSF leaks were not mitigated with data transparency alone. CONCLUSIONS Transparency is a driver of change in patient, provider, and institutional behaviors. It is an essential element of QI and patient safety, as well as building a culture of trust. Readmissions, infections, and same- or next-day appointments were influenced by the living Pareto chart; however, CSF leaks remained recalcitrant to data transparency. Other QI strategies may be necessary to positively affect the occurrence of CSF leaks in neurosurgery.

OBJECTIVE The focus of this modified Delphi study was to investigate and build consensus regarding the medical management of children with moderate and severe acute spinal cord injury (SCI) during their initial inpatient hospitalization. This impetus for the study was based on the AANS/CNS guidelines for pediatric SCI published in 2013, which indicated that there was no consensus provided in the literature describing the medical management of pediatric patients with SCIs. METHODS An international, multidisciplinary group of 19 physicians, including pediatric neurosurgeons, orthopedic surgeons, and intensivists, were asked to participate. The authors chose to include both complete and incomplete injuries with traumatic as well as iatrogenic etiologies (e.g., spinal deformity surgery, spinal traction, intradural spinal surgery, etc.) due to the overall low incidence of pediatric SCI, potentially similar pathophysiology, and scarce literature exploring whether different etiologies of SCI should be managed differently. An initial survey of current practices was administered, and based on the responses, a follow-up survey of potential consensus statements was distributed. Consensus was defined as ≥ 80% of participants reaching agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree). A final meeting was held virtually to generate final consensus statements. RESULTS Following the final Delphi round, 35 statements reached consensus after modification and consolidation of previous statements. Statements were categorized into the following eight sections: inpatient care unit, spinal immobilization, pharmacological management, cardiopulmonary management, venous thromboembolism prophylaxis, genitourinary management, gastrointestinal/nutritional management, and pressure ulcer prophylaxis. All participants stated that they would be willing or somewhat willing to change their practices based on consensus guidelines. CONCLUSIONS General management strategies were similar for both iatrogenic (e.g., spinal deformity, traction, etc.) and traumatic SCIs. Steroids were recommended only for injury after intradural surgery, not after acute traumatic or iatrogenic extradural surgery. Consensus was reached that mean arterial pressure ranges are preferred for blood pressure targets following SCI, with goals between 80 and 90 mm Hg for children at least 6 years of age. Further multicenter study of steroid use following acute neuromonitoring changes was recommended.

OBJECTIVE The goal of this study was to assess the social determinants that influence access and outcomes for pediatric neurosurgical care for patients with Chiari malformation type I (CM-I) and syringomyelia (SM). METHODS The authors used retro- and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM-I and SM who received surgical treatment and had at least 1 year of follow-up data. Race, ethnicity, and insurance status were used as comparators for preoperative, treatment, and postoperative characteristics and outcomes. RESULTS A total of 637 patients met inclusion criteria, and race or ethnicity data were available for 603 (94.7%) patients. A total of 463 (76.8%) were non-Hispanic White (NHW) and 140 (23.2%) were non-White. The non-White patients were older at diagnosis (p = 0.002) and were more likely to have an individualized education plan (p < 0.01). More non-White than NHW patients presented with cerebellar and cranial nerve deficits (i.e., gait ataxia [p = 0.028], nystagmus [p = 0.002], dysconjugate gaze [p = 0.03], hearing loss [p = 0.003], gait instability [p = 0.003], tremor [p = 0.021], or dysmetria [p < 0.001]). Non-White patients had higher rates of skull malformation (p = 0.004), platybasia (p = 0.002), and basilar invagination (p = 0.036). Non-White patients were more likely to be treated at low-volume centers than at high-volume centers (38.7% vs 15.2%; p < 0.01). Non-White patients were older at the time of surgery (p = 0.001) and had longer operative times (p < 0.001), higher estimated blood loss (p < 0.001), and a longer hospital stay (p = 0.04). There were no major group differences in terms of treatments performed or complications. The majority of subjects used private insurance (440, 71.5%), whereas 175 (28.5%) were using Medicaid or self-pay. Private insurance was used in 42.2% of non-White patients compared to 79.8% of NHW patients (p < 0.01). There were no major differences in presentation, treatment, or outcome between insurance groups. In multivariate modeling, non-White patients were more likely to present at an older age after controlling for sex and insurance status (p < 0.01). Non-White and male patients had a longer duration of symptoms before reaching diagnosis (p = 0.033 and 0.004, respectively). CONCLUSIONS Socioeconomic and demographic factors appear to influence the presentation and management of patients with CM-I and SM. Race is associated with age and timing of diagnosis as well as operating room time, estimated blood loss, and length of hospital stay. This exploration of socioeconomic and demographic barriers to care will be useful in understanding how to improve access to pediatric neurosurgical care for patients with CM-I and SM.

Pediatric low-grade gliomas (LGGs) are found in approximately one to three percent of patients with childhood epilepsy. Epilepsy in these patients is often medically refractory and therefore represents a unique cohort with significant morbidity from concomitant pathology. Similar studies in adult patients with low-grade gliomas have identified predictors of seizure freedom including gross-total resection, preoperative seizure control on antiepileptic medication and duration of seizures of less than one year. This study aims to identify similar predictors of seizure freedom in operatively managed pediatric LGGs.A retrospective chart review was performed for patients diagnosed with World Health Organization (WHO) Grade I and II gliomas in patients ≤18 years old at a single institution (Indiana University School of Medicine at Riley Hospital for Children in Indianapolis, IN) from 2007-2017. Infratentorial and purely intraventricular lesions were excluded. WHO classification and histologic diagnosis were based on surgical pathology. Tumor grade, location, laterality, seizure status at presentation, and AED requirements pre- and post-operatively were recorded. Chi-squared analyses for independence were performed controlling for age at presentation, resection extent, seizure type, and Engel Class for seizure freedom post-operatively.Forty-two patients met the inclusion criteria. Preoperative seizures were observed in 23 patients (55%). Presentation with preoperative seizures was highly associated with continued seizure burden post-operatively, independent of the extent of surgical resection. Supratentorial location and the administration of prophylactic pre- and post-operative AEDs were associated with Engel Class I seizure freedom. Temporal location was not significantly associated with medically refractory epilepsy compared with extra-temporal locations.In our cohort of pediatric LGGs, we find that patients that did not initially present with seizures and those who were treated with prophylactic pre- and post-operative AEDs, were more likely to achieve Engel Class I seizure freedom post-operatively. Tumors located in the temporal location were not significantly associated with a higher seizure burden than other supratentorial, extra-temporal tumors. Neither extent of resection nor electrocorticography-guided resection correlated with improved seizure freedom outcomes during glioma resection.

BACKGROUND “Torcular pseudomass,” or redundant soft tissue in the torcular region, is not an infrequent incidental finding on advanced imaging of the brain in infants and young children. It was recently codified among pediatric neuroradiologists; however, its report in the pediatric neurosurgical community has not previously been elucidated. OBSERVATIONS The authors present a case of a 14-month-old child who presented with fever and a first-time seizure. Computed tomography of the head suggested an epidural abscess; however, magnetic resonance imaging characteristics of the lesion were consistent with torcular pseudomass, a normal variant. At the 3-month follow-up, the child was continuing to do well and had not had another seizure. There have been no indications for surgical intervention or additional radiographic surveillance. LESSONS The differential diagnosis for torcular pseudomass includes dural venous sinus thrombosis, dermoid cysts, occipital encephalocele, eosinophilic granuloma, and primary and metastatic tumors, such as neuroblastoma. The management of each of these disorders in the differential diagnosis may be much more invasive than continued observation in the case of torcular pseudomass. Therefore, it is important for pediatric neurosurgeons to become familiar with this developmental anomaly of the dura and occipital skull.

OBJECTIVE Cervical spine disorders in children are relatively uncommon; therefore, paradigms for surgical and nonsurgical clinical management are not well established. The purpose of this study was to bring together an international, multidisciplinary group of pediatric cervical spine experts to build consensus via a modified Delphi approach regarding the clinical management of children with cervical spine disorders and those undergoing cervical spine stabilization surgery. METHODS A modified Delphi method was used to identify consensus statements for the management of children with cervical spine disorders requiring stabilization. A survey of current practices, supplemented by a literature review, was electronically distributed to 17 neurosurgeons and orthopedic surgeons experienced with the clinical management of pediatric cervical spine disorders. Subsequently, 52 summary statements were formulated and distributed to the group. Statements that reached near consensus or that were of particular interest were then discussed during an in-person meeting to attain further consensus. Consensus was defined as ≥ 80% agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree). RESULTS Forty-five consensus-driven statements were identified, with all participants willing to incorporate them into their practice. For children with cervical spine disorders and/or stabilization, consensus statements were divided into the following categories: A) preoperative planning (12 statements); B) radiographic thresholds of instability (4); C) intraoperative/perioperative management (15); D) postoperative care (11); and E) nonoperative management (3). Several important statements reaching consensus included the following recommendations: 1) to obtain pre-positioning baseline signals with intraoperative neuromonitoring; 2) to use rigid instrumentation when technically feasible; 3) to provide postoperative external immobilization for 6–12 weeks with a rigid cervical collar rather than halo vest immobilization; and 4) to continue clinical postoperative follow-up at least until anatomical cervical spine maturity was reached. In addition, preoperative radiographic thresholds for instability that reached consensus included the following: 1) translational motion ≥ 5 mm at C1–2 (excluding patients with Down syndrome) or ≥ 4 mm in the subaxial spine; 2) dynamic angulation in the subaxial spine ≥ 10°; and 3) abnormal motion and T2 signal change on MRI seen at the same level. CONCLUSIONS In this study, the authors have demonstrated that a multidisciplinary, international group of pediatric cervical spine experts was able to reach consensus on 45 statements regarding the management of pediatric cervical spine disorders and stabilization. Further study is required to determine if implementation of these practices can lead to reduced complications and improved outcomes for children.

OBJECTIVE Tethered cord syndrome (TCS) is a clinical and radiographic diagnosis of pathological stretching of the spinal cord leading to progressive loss of neurological function. The gold standard treatment for TCS is a tethered cord release. However, detethering involves significant risks of spinal cord injury and high rates of retethering. To mitigate these risks, the concept of spinal column shortening (SCS) to decrease spinal cord tension has become an alternative to detethering. In this study, the authors applied SCS to a pediatric and emerging adult population affected by secondary TCS. METHODS A retrospective review of a prospective database at the authors’ tertiary pediatric institution was performed. The Pediatric Quality of Life Inventory, patient- and parent-reported outcomes, and urodynamics were used to evaluate the outcomes of TCS treated with SCS. RESULTS A total of 41 patients with secondary TCS were treated with SCS. The average age at the time of surgery was 15.9 years (range 5–55 years). Preoperative symptoms evaluated included pain (33 patients), weakness (30 patients), and bladder/bowel dysfunction (39 patients). The most common level of spinal column osteotomy was T12, with spinal fusion between T10 and L2. The mean follow-up time was 22.6 months (range 8–45 months). For patients with at least 12 months of follow-up, subjective clinical improvements were reported in 21/23 (91.3%) of those with preoperative pain (p < 0.01); in 16/24 (66.7%) of patients with weakness (p < 0.01), and in 15/29 (51.7%) of those with bladder/bowel dysfunction (p < 0.01). The median differences in initial and most recent Pediatric Quality of Life Inventory results were +5 for patient-reported scores (n = 19, p = 0.04) and +5 for parent-reported scores (n = 19, p = 0.08). Formal urodynamics performed at a median of 3.5 months after surgery documented stable to improved bladder function in 16/17 patients, with a median improvement in one classification category (n = 17, p = 0.01). CONCLUSIONS SCS continues to represent a safe and efficacious alternative to traditional spinal cord untethering for TCS in children and emerging adults, as documented by objective formal urodynamics and patient- and parent-reported outcomes.

OBJECTIVE Hypothermia in adult surgical patients has been correlated with an increase in the occurrence of surgical site wound infections, increased bleeding, slower recovery from anesthetics, prolonged hospitalization, and increased healthcare costs. Pediatric surgical patients are at potentially increased risk for hypothermia because of their smaller body size, limited stores of subcutaneous fat, and less effective regulatory capacity. This risk is exacerbated during pediatric spinal surgery by lower preoperative temperature, increased surface exposure to cold during induction and positioning, and prolonged surgical procedure times. The purpose of this quality improvement initiative was to reduce the duration of hypothermia for pediatric patients undergoing spine surgery. METHODS Demographic and clinical data were collected on 162 patients who underwent spinal deformity surgery between October 1, 2017, and July 31, 2019. Data points included patient age, gender, diagnosis, surgical procedure, and temperature readings throughout different phases of perioperative care. Temperatures were obtained upon arrival to the day of surgery, upon presentation to the operating room, during prone positioning, at incision, and at the end of the procedure. Twelve patients were analyzed prior to implementation of a protocol, while 150 patients composed the post-protocol group. RESULTS Using descriptive statistics, the authors found that the average body temperature at the time of incision was 34.0°C prior to the adoption of a preoperative warming protocol, and 35.3°C following a preoperative warming protocol (p = 0.001). There were no complications, such as burns, hyperthermia, or arrhythmias, related to preoperative warming of patients. CONCLUSIONS The placement of a warming blanket on the bed prior to patient arrival and actively targeting normothermia reduced the incidence and duration of hypothermia in pediatric patients undergoing spine surgery with no adverse events.

OBJECTIVE CSF shunts are the most common procedures performed in the pediatric neurosurgical population. Despite attempts in multiple studies, a superior shunt valve has never been shown. Because of this, the authors aim was to examine the impact of shunt valve standardization at their institution to determine if there is a difference in surgical cost, operative time, or short-term postoperative shunt failure. METHODS A retrospective analysis at the authors’ institution was performed for all new CSF diversion shunts, as well as shunt revisions requiring a new valve, or a new valve and at least a new proximal or distal catheter over a 1-year period (January 1, 2016, to December 31, 2016). After a period of transition, neurosurgeons were encouraged to use only one type of fixed-differential-pressure valve and one type of programmable valve when performing shunt surgeries. These patients who underwent “standardized” shunt surgery over a 1-year period (January 1, 2018, to December 31, 2018) were then compared to patients in the prestandardization epoch. All patients were followed for a 12-month period after surgery. Demographic information, surgical cost, operative time, and postoperative shunt failure data were collected in all patients in the study. RESULTS The authors analyzed 87 shunt surgeries in patients prior to standardization and 94 shunt surgeries in patients after standardization. The rate of violation of the standardized shunt valve policy after implementation was 5.3% (5 of 94 procedures). When comparing the prestandardization group to those who received the standardized valve, operative costs were less ($1821.04 vs $1333.75, p = 0.0034). There was no difference in operative times between groups (78 minutes vs 81 minutes, p = 0.5501). There was no difference in total number of shunt failures between the two groups at 12 months after surgery (p = 0.0859). The rate of postoperative infection was consistent with the literature at 8%. CONCLUSIONS In accordance with quality improvement principles, the reduction of unexplained clinical variance invariably leads to a decrease in cost and, more importantly, increased value. In this study, the implementation of a standardized shunt valve decreased operative cost. There were no differences in postoperative shunt failures at 12 months after surgery and no differences in length of surgery. Standardizing shunt valves in the treatment of pediatric hydrocephalus seems to be cost-effective and safe.

OBJECTIVE COVID-19 has not only impacted healthcare systems directly via hospitalizations and resource utilization, but also indirectly via adaptations in healthcare practice, such as the evolution of the academic environment and the rise of telemedicine and virtual education. This void in clinical responsibilities has been filled with academic productivity in various fields. In this study the authors investigate the influence of COVID-19 on the academic focus within pediatric neurosurgery. METHODS All data were obtained from the Journal of Neurosurgery: Pediatrics (JNS Peds). The number of submissions for each month from January 2017 to December 2021 was collected. Data including number of publications, publication level of evidence (LOE), and COVID-19–related articles were collected and verified. Each publication was categorized by manuscript and LOE according to adaptations from the Canadian Task Force on Periodic Health Examination. Publication groups were categorized as pre–COVID-19 (January 2017–February 2020), peri–COVID-19 (March 2020–July 2020), and post–COVID-19 (August 2020–December 2021). Statistical analysis was performed to compare pre–COVID-19, peri–COVID-19, and post–COVID-19 academic volume and quality. RESULTS During the study time period, a total of 3116 submissions and 997 publications were identified for JNS Peds. Only 2 articles specifically related to COVID-19 and its impact on pediatric neurosurgery were identified, both published in 2021. When analyzing submission volume, a statistically significant increase was seen during the shutdown relative to pre–COVID-19 and post-shutdown time periods, and a significant decrease was seen post-shutdown relative to pre–COVID-19. LOE changed significantly as well. When comparing pre–COVID-19 versus post–COVID-19 articles, a statistically significant increase was identified only in level 4 publications. When analyzing pre–COVID-19 versus post–COVID-19 (2020) and post–COVID-19 (2021), a statistically significant decrease in level 3 and increases in levels 4 and 5 were identified during post–COVID-19 (2020), with a rebound increase in level 3 and a decrease in level 5 during post–COVID-19 (2021). CONCLUSIONS There was a significant increase in manuscript submission during the initial pandemic period. However, there was no change during subsequent spikes in COVID-19–related hospitalizations. Coincident with the initial surge in academic productivity, despite steady publication volume, was an inverse decline in quality as assessed by LOE.

BACKGROUND: Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology. OBJECTIVE: To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD. METHODS: The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD. Comparisons were made between subjects who received PFD alone and those with PFD + OCF or PFD + OCF/VD. RESULTS: All 637 patients underwent PFD, 505 (79.2%) with and 132 (20.8%) without duraplasty. A total of 12 subjects went on to have OCF at some point in their management (PFD + OCF), whereas 4 had OCF and VD (PFD + OCF/VD). Of those with complete data, a history of platybasia (3/10, P = .011), Klippel-Feil (2/10, P = .015), and basilar invagination (3/12, P

OBJECTIVE Neurocutaneous syndromes have variable multisystem involvement. The multiorgan involvement, potential pathologies, and various treatment options necessitate collaboration and open discussion to ensure optimal treatment in any given patient. These disorders provide quintessential examples of chronic medical conditions that require a lifelong, multidisciplinary approach. The objectives of this study were to 1) perform a systematic review, thoroughly assessing different multidisciplinary clinic layouts utilized in centers worldwide; and 2) characterize an institutional experience with the management of these conditions, focusing on the patient demographics, clinical presentation, complications, and therapeutic strategies seen in a patient population. METHODS A systematic review of studies involving multidisciplinary clinics and their reported structure was performed according to PRISMA guidelines using the PubMed database. Then a retrospective chart review of patients enrolled in the Oklahoma Children’s Hospital Neurocutaneous Syndromes Clinic was conducted. RESULTS A search of the PubMed database yielded 251 unique results. Of these, 15 papers were included in the analysis, which identified 16 clinics that treated more than 2000 patients worldwide. The majority of these clinics treated patients with neurofibromatosis (13/16). The remaining clinics treated patients with von Hippel–Lindau syndrome (n = 1), tuberous sclerosis complex (n = 1), and multiple neurocutaneous syndromes (n = 1). The most commonly represented subspecialties in these clinics were genetics (15/16) and neurology (13/16). Five clinics (31%) solely saw pediatric patients, 10 clinics saw a combination of children and adults, and the final clinic had separate pediatric and adult clinics. The retrospective chart review of the Neurocutaneous Syndromes Clinic demonstrated that 164 patients were enrolled and seen in the clinic from April 2013 to December 2021. Diagnoses were made based on clinical findings or results of genetic testing; 115 (70%) had neurofibromatosis type 1, 9 (5.5%) had neurofibromatosis type 2, 35 (21%) had tuberous sclerosis complex, 2 (1%) had von Hippel–Lindau syndrome, 2 (1%) had Gorlin syndrome, and the remaining patient (0.6%) had Aarskog-Scott syndrome. Patient demographics, clinical presentation, complications, and therapeutic strategies are also discussed. CONCLUSIONS To the best of the authors’ knowledge, this is the first detailed description of a comprehensive pediatric neurocutaneous clinic in the US that serves patients with multiple syndromes. There is currently heterogeneity between described multidisciplinary clinic structures and practices. More detailed accounts of clinic compositions and practices along with patient data and outcomes are needed in order to establish the most comprehensive and efficient multidisciplinary approach for neurocutaneous syndromes.

Background Pediatric hydrocephalus is a devastating and costly disease. The mainstay of treatment is still surgical shunting of cerebrospinal fluid (CSF). These shunts fail at a high rate and impose a significant burden on patients, their families and society. The relationship between clinical decision making and shunt failure is poorly understood and multifaceted, but catheter occlusion remains the most frequent cause of shunt complications. In order to investigate factors that affect shunt failure, we have established the Wayne State University (WSU) shunt biobank. Methods To date, four hospital centers have contributed various components of failed shunts and CSF from patients diagnosed with hydrocephalus before adulthood. The hardware samples are transported in paraformaldehyde and transferred to phosphate-buffered saline with sodium azide upon deposit into the biobank. Once in the bank, they are then available for study. Informed consent is obtained by the local center before corresponding clinical data are entered into a REDCap database. Data such as hydrocephalus etiology and details of shunt revision history. All data are entered under a coded identifier. Results 293 shunt samples were collected from 228 pediatric patients starting from May 2015 to September 2019. We saw a significant difference in the number of revisions per patient between centers (Kruskal–Wallis H test, p value Conclusion The retrospective analysis identified that differences exist between currently enrolled centers, although further work is needed before clinically actionable recommendations can be made. Moreover, the variables collected from this chart review explain a meaningful amount of variance in the number of revision surgeries. Future work will expand on the contribution of different site-specific and patient-specific factors to identify potential cause and effect relationships.

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. Clinical and radiographic data were collected retrospectively for children screened for BCVI with computed tomography angiography at 5 academic pediatric trauma centers. 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. 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.

OBJECTIVEThe purpose of this study is to determine if the preoperative Pediatric Quality of Life Inventory (PedsQL) score is predictive of short- and intermediate-term PedsQL outcomes following Chiari decompression surgery. The utility of preoperative patient-reported outcomes (PROs) in predicting pain, opioid consumption, and long-term PROs has been demonstrated in adult spine surgery. To the best of the authors’ knowledge, however, there is currently no widely accepted tool to predict short-, intermediate-, or long-term outcomes after pediatric Chiari decompression surgery.METHODSA prospectively maintained database was retrospectively reviewed. Patients who had undergone first-time decompression for symptomatic Chiari malformation were identified and grouped according to their preoperative PedsQL scores: mild disability (score 80–100), moderate disability (score 60–79), and severe disability (score < 60). PedsQL scores at the 6-week, 3-month, and/or 6-month follow-ups were collected. Preoperative PedsQL subgroups were tested for an association with demographic and perioperative characteristics using one-way ANOVA or chi-square analysis. Preoperative PedsQL subgroups were tested for an association with improvements in short- and intermediate-term PedsQL scores using one-way ANOVA and a paired Wilcoxon signed-rank test controlling for statistically different demographic characteristics when appropriate.RESULTSA total of 87 patients were included in this analysis. According to their preoperative PedsQL scores, 28% of patients had mild disability, 40% had moderate disability, and 32% had severe disability. There was a significant difference in the prevalence of comorbidities (p = 0.009) and the presenting symptoms of headaches (p = 0.032) and myelopathy (p = 0.047) among the subgroups; however, in terms of other demographic or operative factors, there was no significant difference. Patients with greater preoperative disability demonstrated statistically significantly lower PedsQL scores at all postoperative time points, except in terms of the parent-reported PedsQL at 6 months after surgery (p = 0.195). Patients with severe disability demonstrated statistically significantly greater improvements (compared to preoperative scores) in PedsQL scores at all time points after surgery, except in terms of the 6-week and 6-month PROs and the 6-month parent-reported outcomes (p = 0.068, 0.483, and 0.076, respectively).CONCLUSIONSPatients with severe disability, as assessed by the PedsQL, had lower absolute PedsQL scores at all time points after surgery but greater improvement in short- and intermediate-term PROs. The authors conclude that the PedsQL is an efficient and accurate tool that can quickly assess patient disability in the preoperative period and predict both short- and intermediate-term surgical outcomes.

Pure arterial malformations of the brain are rare vascular lesions. We present a 10-year-old healthy boy who presented with an incidental finding of pure arterial malformation. Our case seems to represent the second description of pure arterial malformation discovered incidentally in a child. We review the clinical presentation, angiographic findings, and management of our case in the context of other reported pediatric cases.

OBJECTIVE. The purpose of our study was to assess whether linear ventricular dimensions-specifically, the frontal occipital horn ratio (FOHR) and frontal temporal horn ratio (FTHR) obtained from ultrasound (US)-are reliable measures of ventriculomegaly in infants. Our hypothesis was that these US measures would show good correlation with linear ventricular indexes and ventricular volumes obtained from MRI. MATERIALS AND METHODS. We retrospectively identified 90 infants (age ≤ 6 months corrected gestational age) with ventriculomegaly from 2014 to 2017 who had a total of 100 sets of US and MRI studies performed in a 3-day period. FOHR and FTHR were independently measured on US and MRI by two pediatric radiologists and two pediatric neuroradiologists, respectively. Ventricular and brain volumes were segmented from the MR images, and the ventricle-to-intracranial volume ratio was calculated. MRI served as the reference standard. Intraclass correlation coefficients and Bland-Altman analyses were generated to evaluate interobserver and US-MRI concordance. We assessed correlation of the FOHR and FTHR with the ventricle-to-intracranial volume ratio. RESULTS. Bland-Altman plots of the FOHR and FTHR between US and MRI showed excellent concordance with a bias of 0.05 (95% CI, -0.04 to 0.14) and 0.03 (95% CI, -0.06 to 0.13), respectively. There was good-to-excellent interobserver concordance for FOHR and FTHR on head US or MRI (r = 0.86-0.96). There was good correlation between ventricle-to-intracranial volume ratios and US- and MRI-derived FOHRs and FTHRs (r = 0.79-0.87). CONCLUSION. FOHR and FTHR obtained from US in infants with ventriculomegaly have excellent interobserver concordance, are concordant with MRI-derived linear ratios, and correlate with MRI-derived ventricular volumes. Therefore, US-derived FOHR and FTHR are reliable indexes for clinical follow-up of infantile ventriculomegaly.

OBJECTIVE Tethered cord release (TCR) is the gold standard treatment for tethered cord syndrome (TCS); however, there are significant shortcomings including high rates of retethering, especially in complex and recurrent cases. Spinal column shortening (SCS) is an alternative treatment for TCS intended to avoid these shortcomings. Early studies were limited to case reports and smaller case series; however, in recent years, larger case series and small cohort studies have been conducted. Given the increase in available data, a repeat systematic review and meta-analysis is warranted to assess the safety and efficacy of SCS for TCS. METHODS The authors conducted a systematic review using MEDLINE (OVID), Embase (Elsevier), and Web of Science records dating from 1944 to July 2021 to identify all articles investigating SCS for TCS. They performed standard and individual patient data (IPD) meta-analyses, with 2 independent reviewers using PRISMA-IPD guidelines. Primary outcomes were improvement of preoperative clinical symptoms of pain, motor weakness, and bladder and bowel dysfunction, and also surgical complication rate. Secondary outcomes included urodynamic improvement and health-related quality-of-life outcomes determined using patient-reported outcome tools. Individual study quality assessment was performed using a standardized assessment tool for case reports/series, and publication bias was assessed using funnel plot analyses. RESULTS The review yielded 15 studies with 191 cases of TCS treated with SCS. IPD were available in 11 studies with 89 cases. The average age at time of surgery was 28.0 years (range 5–76 years). The average follow-up time was 33.2 months (range 7–132 months). Improvement was observed at last follow-up in 60 of 70 (85.7%) patients with preoperative pain, in 38 of 60 (60.3%) patients with preoperative weakness, and in 36 of 76 (47.4%) patients with preoperative bladder or bowel dysfunction. Complications of CSF leak, new neurological deficit, wound infection, or reoperation occurred in 4 of 89 (4.5%) patients. CONCLUSIONS SCS may be considered a safe and efficacious treatment option for TCS in children and adults (level C evidence; class IIb recommendation), especially for recurrent and complex cases. Current evidence is likely to be affected by selection and publication bias. Prospective comparative studies of SCS and TCR for TCS are recommended to determine long-term duration of outcomes, long-term safety in skeletally immature children, and exact indications of SCS versus traditional TCR.

Complaints related to the neck are common following mild pediatric trauma. Although significant cervical spine injuries are most often seen and evaluated in the emergency room or inpatient setting, the primary care provider is faced with the evaluation of lower acuity complaints. We provide a review to assist with the efficient evaluation of these patients to facilitate decisions regarding return to play, the need for imaging, and need for referral to subspecialty providers.

The transoral transpharyngeal approach is the standard approach to resect the odontoid process and decompress the cervicomedullary spinal cord. There are some significant risks associated with this approach, however, including infection, CSF leak, prolonged intubation or tracheostomy, need for nasogastric tube feeding, extended hospitalization, and possible effects of phonation. Other ventral approaches, such as transmandibular and circumglossal, endoscopic transcervical, and endoscopic transnasal, are also viable alternatives but are technically challenging or may still traverse the nasopharyngeal cavity. Far-lateral and posterior extradural approaches to the craniocervical junction require extensive soft-tissue dissection. Recently, a posterior transdural approach was used to resect retro-odontoid cysts in 3 adult patients. The authors present the case of a 12-year-old girl with Down syndrome and significant spinal cord compression due to basilar invagination and a retro-flexed odontoid process. A posterior transdural odontoidectomy prior to occiptocervical fusion was performed. At 12 months after surgery, the authors report satisfactory clinical and radiographic outcomes with this approach.

Chordomas are histologically benign tumors with local aggressive behavior. They arise from embryological remnants of the notochord at the clivus, mobile spine, and sacrum. Chordomas are rare tumors in the pediatric age group. Their surgical management is difficult, given their propensity for inaccessible anatomical regions, and proximity to critical neurovascular structures. While en bloc resection with surgical margins has been advocated as the preferred approach for chordomas, tumor characteristics and violation of adjacent anatomical boundaries may not allow for safe en bloc resection of the tumor. Here, the authors present the case of a C1 chordoma in a 5-year-old boy with epidural and prevertebral extension. The patient’s treatment consisted of a far-lateral approach for resection of the tumor and C1 arch, followed by circumferential reconstruction of the craniocervical junction with an expandable cage spanning the skull base to C2, and posterior occipitocervical spinal instrumentation. At 42 months after surgery, the patient remains neurologically intact with stable oncological status, and no evidence of craniocervical junction instrumentation failure.

Background Upper airway obstruction leading to dyspnea and dysphagia after occipitocervical fusion is a rare complication that has significant morbidity. Objective To estimate the frequency of postoperative dyspnea and dysphagia in children after occipitocervical fusion and to identify variables associated with its occurrence. Methods We retrospectively reviewed outcomes from all pediatric occipitocervical fusions at our institution between 2007 and 2014. Pre- and postoperative computed tomography (CT) scans were compared to determine differences in the clivoaxial (OC2) angles. Results Sixty-seven pediatric patients underwent occipitocervical fusions. Median age was 9.6 yr (range 6 mo-18 yr). Fifty-six of 62 patients (90.3%) with at least 1 yr of follow-up had successful fusions. Eleven had pre-existing symptoms or otherwise compromised examination (eg, severe traumatic brain injury). None of 15 patients placed in extension (>2 degrees) relative to preoperative CT in Situ position developed new dyspnea or dysphagia. Nine of forty patients (23%) kept in Situ or flexed position developed new symptoms of dyspnea or dysphagia. Dysphagia in patients fused in the in Situ position was milder and resolved within a few weeks. No patient under age 5 (n = 20) developed symptoms of dyspnea or dysphagia regardless of head position. There were 3 cases of infection, 1 clinically silent vertebral artery injury, and 3 deaths at last follow-up. Conclusion Positioning of the child's head prior to occipitocervical fusion has considerable impact on outcomes, especially in older children. Careful measurements of the OC2 angle during surgery to ensure optimal head positioning in Situ or slightly extended position may prevent postoperative dysphagia or dyspnea.

OBJECTIVEThe indication for and timing of surgery for epilepsy associated with low-grade mixed neuronal-glial tumors may be controversial. The purpose of this study was to evaluate the effect of resection and associated variables on epilepsy and on progression-free survival (PFS).METHODSA retrospective chart review of patients treated between 1992 and 2016 was conducted to identify individuals with epilepsy and low-grade gliomas or neuronal-glial tumors who underwent resective surgery. Data analyzed included age at epilepsy onset, age at surgery, extent of resection, use of electrocorticography, the number of antiepileptic drugs (AEDs) before and after surgery, the presence of dysplasia, Engel class, histological findings, and PFS. The institutional review board protocol was specifically approved to conduct this study.RESULTSA total of 107 patients were identified. The median follow-up was 4.9 years. The most common pathology was dysembryoplastic neuroepithelial tumor (36.4%), followed by ganglioglioma (31.8%). Eighty-four percent of patients had Engel class I outcomes following surgery. Gross-total resection was associated with a higher likelihood of an Engel class I outcome (90%) as compared to subtotal resection (58%) (p = 0.0005). Surgery reduced the AED burden, with 40% of patients requiring no AEDs after surgery (p < 0.0001). Children with neurodevelopmental comorbidities (n = 5) uniformly did not experience seizure improvement following resection (0% vs 83% overall; p < 0.0001). Electrocorticography was used in 33% of cases and did not significantly increase class I outcomes. PFS was 90% at 5 years. Eleven percent of tumors recurred, with subtotal resection more likely to result in recurrence (hazard ratio 5.3, p = 0.02). Histological subtype showed no significant impact on recurrence.CONCLUSIONSGross-total resection was strongly associated with Engel class I outcome and longer PFS. Further studies are needed to elucidate the suitable time for surgery and to identify factors associated with oncological transformation.

OBJECTIVEThe evolution of pediatric spinal instrumentation has progressed in the last 70 years since the popularization of the Harrington rod showing the feasibility of placing spinal instrumentation into the pediatric spine. Although lacking in pediatric-specific spinal instrumentation, when possible, adult instrumentation techniques and tools have been adapted for the pediatric spine. A new generation of pediatric neurosurgeons with interest in complex spine disorder has pushed the field forward, while keeping the special nuances of the growing immature spine in mind. The authors sought to review their own experience with various types of spinal instrumentation in the pediatric spine and document the state of the art for pediatric spine surgery.METHODSThe authors retrospectively reviewed patients in their practice who underwent complex spine surgery. Patient demographics, operative data, and perioperative complications were recorded. At the same time, the authors surveyed the literature for spinal instrumentation techniques that have been utilized in the pediatric spine. The authors chronicle the past and present of pediatric spinal instrumentation, and speculate about its future.RESULTSThe medical records of the first 361 patients who underwent 384 procedures involving spinal instrumentation from July 1, 2007, to May 31, 2018, were analyzed. The mean age at surgery was 12 years and 6 months (range 3 months to 21 years and 4 months). The types of spinal instrumentation utilized included occipital screws (94 cases); C1 lateral mass screws (115 cases); C2 pars/translaminar screws (143 cases); subaxial cervical lateral mass screws (95 cases); thoracic and lumbar spine traditional-trajectory and cortical-trajectory pedicle screws (234 cases); thoracic and lumbar sublaminar, subtransverse, and subcostal polyester bands (65 cases); S1 pedicle screws (103 cases); and S2 alar-iliac/iliac screws (56 cases). Complications related to spinal instrumentation included hardware-related skin breakdown (1.8%), infection (1.8%), proximal junctional kyphosis (1.0%), pseudarthroses (1.0%), screw malpositioning (0.5%), CSF leak (0.5%), hardware failure (0.5%), graft migration (0.3%), nerve root injury (0.3%), and vertebral artery injury (0.3%).CONCLUSIONSPediatric neurosurgeons with an interest in complex spine disorders in children should develop a comprehensive armamentarium of safe techniques for placing rigid and nonrigid spinal instrumentation even in the smallest of children, with low complication rates. The authors’ review provides some benchmarks and outcomes for comparison, and furnishes a historical perspective of the past and future of pediatric spine surgery.

OBJECTIVECranial defects can result from trauma, infection, congenital malformations, and iatrogenic causes and represent a surgical challenge. The current standard of care is cranioplasty, with either autologous or allogeneic material. In either case, the intrinsic vascularity of the surrounding tissues allows for bone healing. The objective of this study was to determine if mechanotransductive gene manipulation would yield non–weight-bearing bone regeneration in a critical size calvarial defect in mice.METHODSA mouse model of Sost deletion in Sost knockout (KO) mice was created in which the osteocytes do not express sclerostin. A critical size calvarial defect (4 mm in diameter) was surgically created in the parietal bone in 8-week-old wild-type (n = 8) and Sost KO (n = 8) male mice. The defects were left undisturbed (no implant or scaffold) to simulate a traumatic calvariectomy model. Eight weeks later, the animals were examined at necropsy by planimetry, histological analysis of new bone growth, and micro-CT scanning of bone thickness.RESULTSDefects created in wild-type mice did not fill with bone over the study period of 2 months. Genetic downregulation of sclerostin yielded animals that were able to regenerate 40% of the initial critical size defect area 8 weeks after surgery. A thin layer of bone covered a significant portion of the original defect in all Sost KO animals. A statistically significant increase in bone volume (p < 0.05) was measured in Sost KO mice using radiodensitometric analysis. Immunohistochemical analysis also confirmed that this bone regeneration occurred through the Wnt pathway and originated from the edge of the defect; BMP signaling did not appear to be affected by sclerostin.CONCLUSIONSMechanical loading is an important mechanism of bone formation in the cranial skeleton and is poorly understood. This is partially due to the fact that it is difficult to load bone in the craniomaxillofacial skeleton. This study suggests that modulation of the Wnt pathway, as is able to be done with monoclonal antibodies, is a potentially efficacious method for bone regeneration that requires further study.

Background Implantation of ventricular catheters (VCs) to drain cerebrospinal fluid (CSF) is a standard approach to treat hydrocephalus. VCs fail frequently due to tissue obstructing the lumen via the drainage holes. Mechanisms driving obstruction are poorly understood. This study aimed to characterize the histological features of VC obstructions and identify links to clinical factors. Methods 343 VCs with relevant clinical data were collected from five centers. Each hole on the VCs was classified by degree of tissue obstruction after macroscopic analysis. A subgroup of 54 samples was analyzed using immunofluorescent labelling, histology and immunohistochemistry. Results 61.5% of the 343 VCs analyzed had tissue aggregates occluding at least one hole (n = 211) however the vast majority of the holes (70%) showed no tissue aggregates. Mean age at which patients with occluded VCs had their first surgeries (3.25 yrs) was lower than in patients with non-occluded VCs (5.29 yrs, p Conclusion Our data show that age of the first surgery and length of time a VC is implanted are factors that influence the degree of VC obstruction. The tissue aggregates obstructing VCs are composed predominantly of astrocytes and macrophages; microglia have a relatively small presence.

Supplemental Digital Content is available in the text., Introduction: There has been a proliferation in the development of indwelling neuromodulatory devices with varied safety recommendations, making it difficult for providers to remain up-to-date. This deficit presents an opportunity for significant improvement in patient safety. Methods: We performed a search for monopolar electrocautery and magnetic resonance imaging safety recommendations for several indwelling neuromodulatory devices. We developed a questionnaire followed by an educational compendium and a posttest for 50 care providers. Results: Overall, there was a poor performance on the pretest (mean 39%, SD 19%) but significant improvement on the posttest (mean 71%, SD 16%), P < 0.0001. We placed the educational compendium that included all manufacturer recommendations in the operating room for easy reference. A 2.4 times decrease in the case start times of vagus nerve stimulator cases is evidence of its effectiveness. Conclusions: The authors highlight the lack of knowledge about manufacturer safety recommendations for indwelling neurosurgical devices, which led to the creation of operating room supplements and educational devices.

OBJECTIVE Cervical traction in pediatric patients is an uncommon but invaluable technique in the management of cervical trauma and deformity. Despite its utility, little empirical evidence exists to guide its implementation, with most practitioners employing custom or modified adult protocols. Expert-based best practices may improve the care of children undergoing cervical traction. In this study, the authors aimed to build consensus and establish best practices for the use of pediatric cervical traction in order to enhance its utilization, safety, and efficacy. METHODS A modified Delphi method was employed to try to identify areas of consensus regarding the utilization and implementation of pediatric cervical spine traction. A literature review of pediatric cervical traction was distributed electronically along with a survey of current practices to a group of 20 board-certified pediatric neurosurgeons and orthopedic surgeons with expertise in the pediatric cervical spine. Sixty statements were then formulated and distributed to the group. The results of the second survey were discussed during an in-person meeting leading to further consensus. Consensus was defined as ≥ 80% agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree). RESULTS After the initial round, consensus was achieved with 40 statements regarding the following topics: goals, indications, and contraindications of traction (12), pretraction imaging (6), practical application and initiation of various traction techniques (8), protocols in trauma and deformity patients (8), and management of traction-related complications (6). Following the second round, an additional 9 statements reached consensus related to goals/indications/contraindications of traction (4), related to initiation of traction (4), and related to complication management (1). All participants were willing to incorporate the consensus statements into their practice. CONCLUSIONS In an attempt to improve and standardize the use of cervical traction in pediatric patients, the authors have identified 49 best-practice recommendations, which were generated by reaching consensus among a multidisciplinary group of pediatric spine experts using a modified Delphi technique. Further study is required to determine if implementation of these practices can lead to reduced complications and improved outcomes for children.

Background: Trigeminal schwannomas are rare tumors of the trigeminal nerve. Depending on the location, from which they arise along the trigeminal nerve, these tumors can present with a variety of symptoms that include, but are not limited to, changes in facial sensation, weakness of the masticatory muscles, and facial pain. Case Description: We present a case of a 16-year-old boy with an atypical presentation of a large trigeminal schwannoma: painless malocclusion and unilateral masticatory weakness. This case is the first documented instance; to the best of our knowledge, in which a trigeminal schwannoma has led to underbite malocclusion; it is the 19th documented case of unilateral trigeminal motor neuropathy of any etiology. We discuss this case as a unique presentation of this pathology, and the relevant anatomy implicated in clinical examination aid in further understanding trigeminal nerve pathology. Conclusion: We believe our patient’s underbite malocclusion occurred secondary to his trigeminal schwannoma, resulting in associated atrophy and weakness of the muscles innervated by the mandibular branch of the trigeminal nerve. Furthermore, understanding the trigeminal nerve anatomy is crucial in localizing lesions of the trigeminal nerve.

OBJECTIVE Scoliosis is common in patients with Chiari malformation type I (CM-I)–associated syringomyelia. While it is known that treatment with posterior fossa decompression (PFD) may reduce the progression of scoliosis, it is unknown if decompression with duraplasty is superior to extradural decompression. METHODS A large multicenter retrospective and prospective registry of 1257 pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for patients with scoliosis who underwent PFD with or without duraplasty. RESULTS In total, 422 patients who underwent PFD had a clinical diagnosis of scoliosis. Of these patients, 346 underwent duraplasty, 51 received extradural decompression alone, and 25 were excluded because no data were available on the type of PFD. The mean clinical follow-up was 2.6 years. Overall, there was no difference in subsequent occurrence of fusion or proportion of patients with curve progression between those with and those without a duraplasty. However, after controlling for age, sex, preoperative curve magnitude, syrinx length, syrinx width, and holocord syrinx, extradural decompression was associated with curve progression > 10°, but not increased occurrence of fusion. Older age at PFD and larger preoperative curve magnitude were independently associated with subsequent occurrence of fusion. Greater syrinx reduction after PFD of either type was associated with decreased occurrence of fusion. CONCLUSIONS In patients with CM-I, syrinx, and scoliosis undergoing PFD, there was no difference in subsequent occurrence of surgical correction of scoliosis between those receiving a duraplasty and those with an extradural decompression. However, after controlling for preoperative factors including age, syrinx characteristics, and curve magnitude, patients treated with duraplasty were less likely to have curve progression than patients treated with extradural decompression. Further study is needed to evaluate the role of duraplasty in curve stabilization after PFD.

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 children10 years of age. Our goal was to compare radiographic and clinical outcomes based on medical treatment modality for BCVI in children10 years.Clinical and radiographic data were collected retrospectively for children screened for BCVI with computed tomography angiography at 5 academic pediatric trauma centers.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.There were no observed differences in the rate of hemorrhagic complications between anticoagulation and antiplatelet therapy for BCVI in children10 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.

Background Pediatric hydrocephalus is a devastating and costly disease. The mainstay of treatment is still surgical shunting of cerebrospinal fluid (CSF). These shunts fail at a high rate and impose a significant burden on patients, their families and society. The relationship between clinical decision making and shunt failure is poorly understood and multifaceted, but catheter occlusion remains the most frequent cause of shunt complications. In order to investigate factors that affect shunt failure, we have established the Wayne State University (WSU) shunt biobank.Methods To date, six hospital centers have contributed various components of failed shunts and CSF from patients diagnosed with hydrocephalus at a young age. The hardware samples are transported in paraformaldehyde and transferred to phosphate-buffered saline with sodium azide upon deposit into the biobank. Once in the bank, they are then available for study. Informed consent is obtained by the local center before corresponding clinical data are entered into a REDCap database. All data are entered under a coded identifier. Collaborators may then correlate biologic findings against the clinical database.Results 295 shunt samples were collected from 228 patients starting from May 2015 to September 2019. Patients with multiple samples in the bank provide a unique opportunity to study longitudinal changes in disease. With the clinical data alone, we saw a significant difference in the number of revisions per patient between centers (Kruskal-Wallis H test, p-value= 0.000022). There was no significant difference between the distributions of hydrocephalus etiologies between centers, and the leading etiology at all centers was post-hemorrhagic hydrocephalus.Conclusion Hospital center and patient recruitment for the biobank is ongoing. The biobank will yield insights for future collaborators, allow centers to benchmark their performance, and offer a unique-longitudinal perspective on the pathology of this lifelong condition. Future work will expand on the contribution of different site-specific and patient-specific factors to identify potential cause and effect relationships.

OBJECTIVEThe revelation of normative radiographic measurements for the developing pediatric spine is incomplete. The purpose of this analysis was to determine the normal range of asymmetry of the lateral atlantodental interval (LADI) and define age- and sex-related differences.METHODSA total of 3072 children aged 0–18 years who underwent CT scanning of the cervical spine were identified at Riley Hospital for Children between 2005 and 2017. Patients were stratified by sex and age (in years) into 36 cohorts. Following this stratification, patients within each group were randomly selected for inclusion until 15 patients in each group had been measured (quota sampling). A total of 540 patients were included for study. Right and left linear measurements were performed in the CT axial plane at the C-1 midlateral mass level.RESULTSThe overall mean difference between the right and left LADI was 0.09 ± 1.23 mm (range -6.05 to 4.87 mm). The magnitude of this asymmetry remained statistically insignificant across age groups (p = 0.278) and sex (p = 0.889). The intraclass correlation coefficient was 0.805 (95% CI 0.779–0.829).CONCLUSIONSAsymmetry of the LADI is not unusual in asymptomatic children. There is no appreciable difference in magnitude of this asymmetry across age ranges and sex. Measurement of LADI asymmetry shows “good” reliability and is easy to perform. Pediatric neurosurgeons, emergency department physicians, and radiologists should be aware of normative values of asymmetry when interpreting CT scans of the cervical spine. This may prevent unnecessary further workup with dynamic CT or MRI.

Background and Hypothesis: Chiari malformation type I (CM-I) is a common pediatric disorder in which the cerebellar tonsils herniate the foramen magnum with associated spinal symptoms. The purpose of this study is to determine if the preoperative Pediatric Quality of Life Inventory (PedsQL) score is predictive of intermediate-term PedsQL outcomes following Chiari decompression surgery. The utility of preoperative patient-reported outcomes (PROs) to postoperative PROs has been previously demonstrated in adult spine surgery. However, to our knowledge, there is currently no widely accepted tool to predict short-, intermediate-, or long-term outcomes after pediatric Chiari decompression surgery. Project Methods: A prospectively maintained database was retrospectively reviewed. Patients who underwent first-time decompression for symptomatic Chiari malformation were identified and grouped by their preoperative PedsQL scores: mild disability (score 80–100), moderate disability (score 60–80); and severe disability (score < 60). PedsQL outcomes were collected at 6-week, 3-month, and 6-month follow-up. Preoperative PedsQL subgroups were tested for an association with demographic, perioperative characteristics, and improvements between groups by one-way ANOVA, chi-square, or Wilcoxon signed rank test, where appropriate. Results: The postoperative patient- and parent-reported PedsQL values were significantly different between all three groups at 6-weeks, 3-months, and 6-months after surgery (p < 0.05), except at the 6-month timepoint for parent-reported outcomes (p = 0.111). Patients with higher preoperative disability demonstrated statistically significant greater improvements (compared to preoperative score) in parent-reported PedsQL at all timepoints after surgery, except at the 6-month parent-reported outcomes (p = 0.133). Conclusion: Patients with worse preoperative disability, as assessed by PedsQL, experienced lower absolute PedsQL scores at all timepoints after surgery but had greater improvement in short- and intermediate-term PROs. We conclude that PedsQL is an efficient and accurate tool that can quickly assess patient disability in the preoperative period and predict both short-term and long-term surgical outcomes.

OBJECTIVEPatients with shunts often interact with providers distant from their primary hospital, making it important that the parent(s)/guardian(s) is well versed in the type of shunt implanted and symptoms of malfunction/infection. This is particularly important with magnetic-sensitive programmable valves, as the use of MRI becomes more prevalent.METHODSOver a 6-month period, primary caregivers of 148 consecutive patients who received shunts were prospectively administered questionnaires at clinic visits. Caregivers were asked to do the following: 1) identify shunt valve name, type, and setting if applicable; 2) list symptoms of shunt malfunction/infection; and 3) indicate whether they had access to references regarding shunt type/setting, booklets from the Hydrocephalus Association, and quick reference cards with symptoms of shunt malfunction/infection. One cohort of caregivers (n = 75) was asked to carry informational cards with shunt valve/setting information (group I); this cohort was compared with another subgroup of caregivers (n = 73) not carrying cards (group II).RESULTSThe mean (± SD) age of patients at implantation/revision was 3.71 ± 4.91 years, and the age at follow-up was 6.12 ± 5.4 years. The average time from surgery to administration of the questionnaire was 2.38 ± 3.22 years. There were 86 new shunt insertions and 62 revisions. One hundred twenty-eight caregivers (87%) could identify the type of valve (programmable vs nonprogrammable). On the other hand, only 72 caregivers (49%) could identify the valve name. Fifty-four of 73 (74%) caregivers of patients who had shunts with programmable valves could correctly identify the valve setting. One hundred caregivers (68%) had a copy of the Hydrocephalus Association booklet, and 103 (70%) had quick reference cards. Eighty caregivers (54%) had references on shunt type/setting. Most caregivers (127 [86%]) could name ≥ 3 signs/symptoms of shunt malfunction, with vomiting (61%), headache (49%), and sleeps more/lethargic (35%) most frequently reported. Caregivers of patients in group I were more likely to have cards with symptoms of shunt infection or malfunction (p = 0.015); have information cards regarding shunt type/setting (p < 0.001); and correctly identify valve type (p = 0.001), name (p < 0.001), and setting if programmable (p = 0.0016). There were no differences in ability to list symptoms of shunt malfunction or infection (p = 0.8812) or in access to Hydrocephalus Association booklets (p = 0.1288). There were no significant demographic differences between the groups, except that group I patients had a shorter time from surgery to last follow-up (1.66 vs 3.17 years; p = 0.0001).CONCLUSIONSEducation regarding the care of patients with shunts by providing written cards with shunt type/setting and access to reference materials seems to be effective. Developing plans for guided instruction with assessment in the clinic setting of a caregiver’s knowledge is important for patient safety.

OBJECTIVEEndoscopic third ventriculostomy (ETV) is a surgical alternative to placing a CSF shunt in certain patients with hydrocephalus. The ETV Success Score (ETVSS) is a reliable, simple method to estimate the success of the procedure by 6 months of postoperative follow-up. The highest score is 90, estimating a 90% chance of the ETV effectively treating hydrocephalus without requiring a shunt. Treatment with ETV fails in certain patients, despite their being the theoretically best candidates for the procedure. In this study the authors attempted to identify factors that further predicted success in patients with the highest ETVSSs.METHODSA retrospective review was performed of all patients treated with ETV at 3 institutions. Demographic, radiological, and clinical data were recorded. All patients by definition were older than 1 year, had obstructive hydrocephalus, and did not have a prior shunt. Failure of ETV was defined as the need for a shunt by 1 year. The ETV was considered a success if the patient did not require another surgery (either shunt placement or a repeat endoscopic procedure) by 1 year. A statistical analysis was performed to identify factors associated with success or failure.RESULTSFifty-nine patients met the entry criteria for the study. Eleven patients (18.6%) required further surgery by 1 year. All of these patients received a shunt. The presenting symptom of lethargy statistically correlated with success (p = 0.0126, odds ratio [OR] = 0.072). The preoperative radiological finding of transependymal flow (p = 0.0375, OR 0.158) correlated with success. A postoperative larger maximum width of the third ventricle correlated with failure (p = 0.0265).CONCLUSIONSThe preoperative findings of lethargy and transependymal flow statistically correlated with success. This suggests that the best candidates for ETV are those with a relatively acute elevation of intracranial pressure. Cases without these findings may represent the failures in this highly selected group.

Background Blunt cerebrovascular injury is uncommon in the pediatric population; penetrating cerebrovascular injuries are even rarer and are thus poorly understood. Objective To describe the diagnosis and management of penetrating cerebrovascular injuries and describe outcomes of available treatment modalities. Methods Clinical and radiographic data were collected retrospectively from a multicenter trauma registry for children screened for cerebrovascular injury during 2003 to 2013 at 4 academic pediatric trauma centers. Results Among 645 pediatric patients evaluated with computed tomography angiography with blunt cerebrovascular injury, 130 also had a penetrating trauma indication. Seven penetrating cerebrovascular injuries were diagnosed in 7 male patients (mean age 12.4 years, range 12-18 years). Focal neurological deficit and concomitant intracranial injury were each seen in 2 patients. There were 2 intracranial carotid artery injuries, 4 extracranial carotid artery injuries, and 1 vertebral artery injury. The majority of injuries were higher than grade I (5/7; 71%): 2 were grade I, 1 grade II, 2 grade III, and 2 grade IV. The 2 patients with grade III injuries required open surgery, and 1 patient with a grade IV injury underwent endovascular treatment. Two patients suffered immediate stroke secondary to the penetrating cerebrovascular injury. There were no delayed neurological deficits from the penetrating injuries, and no patients died as a result of the injuries. Conclusion This is the largest series of penetrating cerebrovascular trauma in the pediatric literature. Although rare, penetrating cerebrovascular injuries can be high-grade injuries that require urgent recognition and may require aggressive endovascular and/or open surgery for treatment.

OBJECTIVE The authors previously demonstrated the use of a validated Objective Structured Assessment of Technical Skills (OSATS) tool for evaluating residents' operative skills in pediatric neurosurgery. However, no benchmarks have been established for specific pediatric procedures despite an increased need for meaningful assessments that can either allow for early intervention for underperforming trainees or allow for proficient residents to progress to conducting operations independently with more passive supervision. This validated methodology and tool for assessment of operative skills for common pediatric neurosurgical procedures—external ventricular drain (EVD) placement and shunt surgery— was applied to establish its procedure-based feasibility and reliability, and to document the effect of repetition on achieving surgical skill proficiency in pediatric EVD placement and shunt surgery. METHODS A procedure-based technical skills assessment for EVD placements and shunt surgeries in pediatric neurosurgery was established through the use of task analysis. The authors enrolled all residents from 3 training programs (Baylor College of Medicine, Houston Methodist Hospital, and University of Texas–Medical Branch) who rotated through pediatric neurosurgery at Texas Children's Hospital over a 26-month period. For each EVD placement or shunt procedure performed with a resident, the faculty and resident (for self-assessment) completed an evaluation form (OSATS) based on a 5-point Likert scale with 7 categories. Data forms were then grouped according to faculty versus resident (self) assessment, length of pediatric neurosurgery rotation, postgraduate year level, and date of evaluation (“beginning of rotation,” within 1 month of start date; “end of rotation,” within 1 month of completion date; or “middle of rotation”). Descriptive statistical analyses were performed with the commercially available SPSS statistical software package. A p value < 0.05 was considered statistically significant. RESULTS Five attending evaluators (including 2 fellows who acted as attending surgeons) completed 260 evaluations. Twenty house staff completed 269 evaluations for self-assessment. Evaluations were completed in 562 EVD and shunt procedures before the surgeons left the operating room. There were statistically significant differences (p < 0.05) between overall attending (mean 4.3) and junior resident (self; mean 3.6) assessments, and between overall attending (mean 4.8) and senior resident (self; mean 4.6) assessment scores on general performance and technical skills. The learning curves produced for the residents demonstrate a stereotypical U- or V-shaped curve for acquiring skills, with a significant improvement in overall scores at the end of the rotation compared with the beginning. The improvement for junior residents (Δ score = 0.5; p = 0.002) was larger than for senior residents (Δ score = 0.2; p = 0.018). CONCLUSIONS The OSATS is an effective assessment tool as part of a comprehensive evaluation of neurosurgery residents' performance for specific pediatric procedures. The authors observed a U-shaped learning curve, contradicting the idea that developing one's surgical technique and learning a procedure represents a monotonic, cumulative process of repetitions and improvement.

BACKGROUND Pediatric blunt cerebrovascular injury (BCVI) lacks accepted treatment algorithms, and postinjury outcomes are ill defined. OBJECTIVE To compare treatment practices among pediatric trauma centers and to describe outcomes for available treatment modalities. METHODS Clinical and radiographic data were collected from a patient cohort with BCVI between 2003 and 2013 at 4 academic pediatric trauma centers. RESULTS Among 645 pediatric patients evaluated with computed tomography angiography for BCVI, 57 vascular injuries (82% carotid artery, 18% vertebral artery) were diagnosed in 52 patients. Grade I (58%) and II (23%) injuries accounted for most lesions. Severe intracranial or intra-abdominal hemorrhage precluded antithrombotic therapy in 10 patients. Among the remaining patients, primary therapy was an antiplatelet agent in 14 (33%), anticoagulation in 8 (19%), endovascular intervention in 3 (7%), open surgery in 1 (2%), and no treatment in 16 (38%). Among 27 eligible grade I injuries, 16 (59%) were not treated, and the choice to not treat varied significantly among centers (P < .001). There were no complications from medical management. Glasgow Coma Scale (GCS) score

Pediatric stroke is a relatively rare occurrence, with an annual incidence of 1.2–13 cases per 100,000. Hemorrhagic strokes account for half of these cases.[28] In adults, hemorrhagic strokes are predominantly hypertensive in etiology. However, in the pediatric population, they are frequently associated with vascular lesions such as AVMs (47%), arteriovenous fistulas, or CMs [Table 1].[21] Other causes of ICH in adults, such as amyloid angiopathy or drug-related vascular damage, are rarely seen in the pediatric population. Workup of pediatric ICH should include vascular imaging consisting of either CTA or DSA. An MRI of the brain should be obtained to detect CMs, which are angiographically occult. MR angiography may lack the sensitivity to allow for visualization of the smaller vessels that may be involved with some of these lesions.[25] In a series of 137 patients with ICH described by Hino et al., 9% were found to have an “occult” vascular lesion that was not visualized on first angiogram. The clinical index of suspicion should guide the workup further if a causative lesion cannot be identified upon initial imaging. This may include repeat DSA, which is considered the gold standard for the assessment of vascular lesions. In the setting of clinically symptomatic hemorrhage, any vascular lesion, including AVM, CM, capillary telangiectasias, or developmental venous anomalies, may present in occult fashion, though AVM is most common.[7] Table 1 Most common etiologies of spontaneous intracerebral hemorrhage in adults and children

OBJECTIVE The purpose of this study was to describe patterns of transfer, resource utilization, and clinical outcomes associated with the interhospital transfer of pediatric neurosurgical patients. METHODS All consecutive, prospectively collected requests for interhospital patient transfer to the pediatric neurosurgical service at Texas Children's Hospital were retrospectively analyzed from October 2013 to September 2014. Demographic patient information, resource utilization, and outcomes were recorded and compared across predefined strata (low [< 5%], moderate [5%–30%], and high [> 30%]) of predicted probability of mortality using the Pediatric Risk of Mortality score. RESULTS Requests for pediatric neurosurgical care comprised 400 (3.7%) of a total of 10,833 calls. Of 400 transfer admissions, 96.5%, 2.8%, and 0.8% were in the low, moderate, and high mortality risk groups, respectively. The median age was 54 months, and 45% were female. The median transit time was 125 minutes. The majority of transfers were after-hours (69.8%); nearly a third occurred during the weekend (32.3%). The median intensive care unit stay for 103 patients was 3 days (range 1–269 days). Median length of hospital stay was 2 days (range 1–269 days). Ninety patients (22.5%) were discharged from the emergency room after transfer. Seventy-seven patients (19.3%) required neurosurgical intervention after transfer, with the majority requiring a cranial procedure (66.2%); 87.3% of patients were discharged home. CONCLUSIONS This study highlights patient characteristics, resource utilization, and outcomes among pediatric neurosurgical patients. Opportunities for quality improvement were identified in diagnosing and managing isolated skull fractures and neck pain after trauma.