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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 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.

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

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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

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.

* Abbreviation: SEMLS — : single-event multilevel surgery Single-event multilevel surgery (SEMLS) refers to the surgical management of children and adult patients with cerebral palsy and spastic diplegia to address muscle imbalance, contractures, and musculoskeletal deformities secondary to untreated or inadequately treated spasticity.1–3 Spasticity is a movement disorder in which there is injury to upper motor neurons comprising the pyramidal tract. The upstream inhibitory influence of the central nervous system is interrupted, causing the local reflex arc in the spinal cord to become disinhibited and leading to continuous muscle contraction. The spasticity is characterized by a velocity-dependent increase in muscle tone with hyperreflexia. Surgical management consists of concurrent orthopedic procedures targeting the musculoskeletal system at ≥2 levels (eg, feet, ankles, knees, and hips). Procedures include tendon lengthening to correct contractures, tendon transfers for muscle imbalance, rotational osteotomies for torsional deformities, and stabilization of the hip and foot. The advantages of SEMLS include a single hospital … Address correspondence to Andrew Jea, MD, MHA, FAAP, Section of Pediatric Neurosurgery, Indiana University School of Medicine, Riley Hospital for Children, 705 Riley Hospital Dr, 1134, Indianapolis, IN 46202. E-mail: ajea{at}goodmancampbell.com

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.

OBJECTIVE The emphasis on health-related quality of life (HRQOL) outcomes is increasing, along with an emphasis on evidence-based medicine. However, there is a notable paucity of validated HRQOL instruments for the pediatric population. Furthermore, no standardization or consensus currently exists concerning which HRQOL outcome measures ought to be used in pediatric neurosurgery. The authors wished to identify HRQOL outcomes used in pediatric neurosurgery research over the past 10 years, their frequency, and usage trends. METHODS Three top pediatric neurosurgical journals were reviewed for the decade from 2005 to 2014 for clinical studies of pediatric neurosurgical procedures that report HRQOL outcomes. Similar studies in the peer-reviewed journal Pediatrics were also used as a benchmark. Publication year, level of evidence, and HRQOL outcomes were collected for each article. RESULTS A total of 31 HRQOL studies were published in the pediatric neurosurgical literature over the study period. By comparison, there were 55 such articles in Pediatrics. The number of publications using HRQOL instruments showed a significant positive trend over time for Pediatrics (B = 0.62, p = 0.02) but did not increase significantly over time for the 3 neurosurgical journals (B = 0.12, p = 0.5). The authors identified a total of 46 different HRQOL instruments used across all journals. Within the neurosurgical journals, the Hydrocephalus Outcome Questionnaire (HOQ) (24%) was the most frequently used, followed by the Health Utilities Index (HUI) (16%), the Pediatric Quality of Life Inventory (PedsQL) (12%), and the 36-Item Short Form Health Survey (SF-36) (12%). Of the 55 articles identified in Pediatrics, 22 (40%) used a version of the PedsQL. No neurosurgical study reached above Level 4 on the Oxford Centre for Evidence-Based Medicine (OCEBM) system. However, multiple studies from Pediatrics achieved OCEBM Level 3, several were categorized as Level 2, and one reached Level 1. CONCLUSIONS The frequency of studies using HRQOL outcomes in pediatric neurosurgical research has not increased over the past 10 years. Within pediatric neurosurgery, high-quality studies and standardization are lacking, as compared with contemporary studies in Pediatrics. In general, although the HOQ, HUI, PedsQL, and SF-36 instruments are emerging as standards in pediatric neurosurgery, even greater standardization across the specialty is needed, along with the design and implementation of more rigorous studies.

The surgical management of high-grade spondylolisthesis in adolescents remains a controversial issue. Because the basic procedure, posterolateral fusion, is associated with a significant rate of pseudarthrosis and listhesis progression, there is a pressing need for alternative surgical techniques. In the present report, the authors describe the case of an adolescent patient with significant low-back pain who was found to have Grade IV spondylolisthesis at L5–S1 that was treated with transsacral transdiscal screw fixation. Bilateral pedicle screws were placed starting from the top of the S-1 pedicle, across the L5–S1 intervertebral disc space, and into the L-5 body. At 14 months after surgery, the patient had considerable improvement in his pain and radiographic fusion across L5–S1. The authors conclude that transsacral transdiscal pedicle screws may serve as an efficacious and safe option for the correction of high-grade spondylolisthesis in adolescent patients.

OBJECTIVE The complication profile of epidural triamcinolone acetonide use during lumbar decompression surgery is not known. However, isolated reports of increased risk of delayed CSF leakage with the use of triamcinolone acetonide in adult spinal surgery patients have been published. The purpose of this study was to determine the safety of epidural triamcinolone acetonide use in conjunction with lumbar decompression surgery in pediatric patients. METHODS The medical records of all patients who underwent lumbar decompression surgery with or without discectomy between July 1, 2007, and July 31, 2015, were retrospectively reviewed. RESULTS During the study period, 58 patients underwent 59 spine procedures at Texas Children's Hospital. There were 33 female and 25 male patients. The mean age at surgery was 16.5 years (range 12–24 years). Patients were followed for an average of 38.2 months (range 4–97 months). Triamcinolone acetonide was used in 28 (of 35 total) cases of discectomy; there were no cases of delayed symptomatic CSF leaks (0%) in the minimally invasive and open discectomies. On the other hand, triamcinolone acetonide was used in 14 (of 24 total) cases of multilevel laminectomy, among which there were 10 delayed CSF leaks (71.4%) requiring treatment. The use of triamcinolone acetonide in patients who underwent multilevel laminectomy was significantly associated with an increased risk of delayed CSF leaks or pseudomeningoceles (Fisher's exact test, p < 0.001). CONCLUSIONS There was an unacceptable incidence of delayed postoperative CSF leaks when epidural triamcinolone acetonide was used in patients who underwent multilevel laminectomy.

Background Trigeminal neuralgia (TN) is a well-recognized facial pain syndrome. Discrete forms with disparate pain symptoms include classic and atypical. However, atypical facial pain includes neuralgiform pain along a spectrum. Most cases of TN are diagnosed in the adult population. Case reports and series of children have presented TN as a similar entity, with treatment similar to that for adults. We reviewed the pertinent data and present 2 pediatric TN cases successfully treated with microvascular decompression (MVD). Case Description Two pediatric patients (age 12 and 15 years) with TN refractory to previous medical therapy were identified. Both patients were deemed appropriate surgical candidates and underwent MVD to manage their TN. TN compression was arterial in both cases and involved portions of the anterior inferior cerebellar artery. Patient 1 was pain free 6 months after the procedure. Patient 2 was pain free immediately after the procedure and had been weaned off preoperative symptomatic management at the latest follow-up visit. The most recent follow-up examination was 12 and 8 months for patients 1 and 2, respectively, with both experiencing continued freedom from pain. Conclusions Few studies have reported on the effectiveness of MVD in the pediatric population for the management of TN. The supporting data and our 2 cases have demonstrated that MVD is effective for pediatric patients to treat their TN. Furthermore, the side effects appear to be minimal, with excellent pain relief after MVD in this patient population.

Tethered cord syndrome is a constellation of signs and symptoms involving sensory and motor dysfunction of the lower extremities, bladder, and bowel. The pathophysiology may be from a mechanical stretch of the distal spinal cord causing deleterious changes in blood supply and metabolism. The standard of care for tethered cord syndrome has been tethered cord release. However, drawbacks of traditional tethered cord release include neurological injury, cerebrospinal fluid leak, and intradural scarring and retethering of the spinal cord (secondary tethering). As such, spinal column shortening was described to avoid these risks.1 We applied spinal column shortening to children and transitional adults with secondary tethered cord syndrome.1 As shown in cadaveric biomechanical studies, the objective of surgery is to shorten by the spinal column by 1.5 to 2.5 cm, to obtain the equivalent relief of traction on the spinal cord and nerve roots as direct circumferential untethering.2 In this video, we present the case of an 11-yr-old boy with a history of VATERL (vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities) and tethered cord syndromes manifest as bladder dysfunction. Our patient had segmentation and formation anomalies of the lumbosacral junction, imperforate anus, and leg length discrepancy. He had already undergone multiple tethered cord releases with no durable improvement in bladder function. After discussion of the advantages and disadvantages of spinal column shortening with the patient and his father, we decided to proceed with this treatment alternative. At 9 mo after surgery, the patient has satisfactory clinical and radiographic outcomes. Informed consent was obtained from the parents of the patient.

OBJECTIVEComplications after complex tethered spinal cord (cTSC) surgery include infections and cerebrospinal fluid (CSF) leaks. With little empirical evidence to guide management, there is variability in the interventions undertaken to limit complications. Expert-based best practices may improve the care of patients undergoing cTSC surgery. Here, authors conducted a study to identify consensus-driven best practices.METHODSThe Delphi method was employed to identify consensual best practices. A literature review regarding cTSC surgery together with a survey of current practices was distributed to 17 board-certified pediatric neurosurgeons. Thirty statements were then formulated and distributed to the group. Results of the second survey were discussed during an in-person meeting leading to further consensus, which was defined as ≥ 80% agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree).RESULTSSeventeen consensus-driven best practices were identified, with all participants willing to incorporate them into their practice. There were four preoperative interventions: (1, 2) asymptomatic AND symptomatic patients should be referred to urology preoperatively, (3, 4) routine preoperative urine cultures are not necessary for asymptomatic AND symptomatic patients. There were nine intraoperative interventions: (5) patients should receive perioperative cefazolin or an equivalent alternative in the event of allergy, (6) chlorhexidine-based skin preparation is the preferred regimen, (7) saline irrigation should be used intermittently throughout the case, (8) antibiotic-containing irrigation should be used following dural closure, (9) a nonlocking running suture technique should be used for dural closure, (10) dural graft overlay should be used when unable to obtain primary dural closure, (11) an expansile dural graft should be incorporated in cases of lipomyelomeningocele in which primary dural closure does not permit free flow of CSF, (12) paraxial muscles should be closed as a layer separate from the fascia, (13) routine placement of postoperative drains is not necessary. There were three postoperative interventions: (14) postoperative antibiotics are an option and, if given, should be discontinued within 24 hours; (15) patients should remain flat for at least 24 hours postoperatively; (16) routine use of abdominal binders or other compressive devices postoperatively is not necessary. One intervention was prioritized for additional study: (17) further study of additional gram-negative perioperative coverage is needed.CONCLUSIONSA modified Delphi technique was used to develop consensus-driven best practices for decreasing wound complications after cTSC surgery. Further study is required to determine if implementation of these practices will lead to reduced complications. Discussion through the course of this study resulted in the initiation of a multicenter study of gram-negative surgical site infections in cTSC surgery.

OBJECTIVEThe long-term effects of surgical fusion on the growing subaxial cervical spine are largely unknown. Recent cross-sectional studies have demonstrated that there is continued growth of the cervical spine through the teenage years. The purpose of this multicenter study was to determine the effects of rigid instrumentation and fusion on the growing subaxial cervical spine by investigating vertical growth, cervical alignment, cervical curvature, and adjacent-segment instability over time.METHODSA total of 15 centers participated in this multi-institutional retrospective study. Cases involving children less than 16 years of age who underwent rigid instrumentation and fusion of the subaxial cervical spine (C-2 and T-1 inclusive) with at least 1 year of clinical and radiographic follow-up were investigated. Charts were reviewed for clinical data. Postoperative and most recent radiographs, CT, and MR images were used to measure vertical growth and assess alignment and stability.RESULTSEighty-one patients were included in the study, with a mean follow-up of 33 months. Ninety-five percent of patients had complete clinical resolution or significant improvement in symptoms. Postoperative cervical kyphosis was seen in only 4 patients (5%), and none developed a swan-neck deformity, unintended adjacent-level fusion, or instability. Of patients with at least 2 years of follow-up, 62% demonstrated growth across the fusion construct. On average, vertical growth was 79% (4-level constructs), 83% (3-level constructs), or 100% (2-level constructs) of expected growth. When comparing the group with continued vertical growth to the one without growth, there were no statistically significant differences in terms of age, sex, underlying etiology, surgical approach, or number of levels fused.CONCLUSIONSContinued vertical growth of the subaxial spine occurs in nearly two-thirds of children after rigid instrumentation and fusion of the subaxial spine. Failure of continued vertical growth is not associated with the patient’s age, sex, underlying etiology, number of levels fused, or surgical approach. Further studies are needed to understand this dichotomy and determine the long-term biomechanical effects of surgery on the growing pediatric cervical spine.

Severe lower limb spasticity can hinder motor tasks and negatively impacts the quality of life in patients with cerebral palsy. Selective dorsal rhizotomy is a well-established neurosurgical intervention aimed at reducing muscle spasticity in patients with such neuromuscular conditions. Long-term outcomes of selective dorsal rhizotomy have been promising among the authors' institutional series of patients. In this case, we demonstrate the use of L1-S1 osteoplastic laminoplasty and L1-S1 selective dorsal rhizotomy in a 5-year-old male patient with cerebral palsy and spastic lower extremity diplegia. Favorable selection criteria for this case included disabling lower extremity diplegia, young age, good core strength, no cognitive delay, and strong rehabilitation potential. The patient's preoperative functional status was noncommunity ambulator (Gross Motor Function Classification System Level III) with walker use and good dynamic balance. Prior to the procedure, he demonstrated an overall decreased muscle strength in bilateral lower extremities with bilateral hamstring spasticity (Ashworth 3) and bilateral heel cord spasticity (Ashworth 4). Rhizotomy was performed with identification and selective sectioning of dorsal nerve roots with abnormal stimulation patterns. Fibers with unsustained discharge of appropriate muscles were identified and spared. No intraoperative or postoperative complications were encountered. The patient had minimal back pain and surgical morbidity postoperatively. Following the procedure and highly structured inpatient and outpatient rehabilitation therapies, the patient exhibited significant improvement in gait velocity (84%) and gait cadence (66%) at 5 months. Additionally, the patient demonstrated greater independence of activities of daily living and improvements in mobility by Pediatric Evaluation Disability Index. Patient consent was obtained from the parent.

OBJECT Currently there is no standardized tool for assessment of neurosurgical resident performance in the operating room. In light of enhanced requirements issued by the Accreditation Council for Graduate Medical Education’s Milestone Project and the Matrix Curriculum Project from the Society of Neurological Surgeons, the implementation of such a tool seems essential for objective evaluation of resident competence. Beyond compliance with governing body guidelines, objective assessment tools may be useful to direct early intervention for trainees performing below the level of their peers so that they may be given more hands-on teaching, while strong residents can be encouraged by faculty members to progress to conducting operations more independently with passive supervision. The aims of this study were to implement a validated assessment tool for evaluation of operative skills in pediatric neurosurgery and determine its feasibility and reliability. METHODS All neurosurgery residents completing their pediatric rotation over a 6-month period from January 1, 2014, to June 30, 2014, at the authors’ institution were enrolled in this study. For each procedure, residents were evaluated by means of a form, with one copy being completed by the resident and a separate copy being completed by the attending surgeon. The evaluation form was based on the validated Objective Structured Assessment of Technical Skills for Surgery (OSATS) and used a 5-point Likert-type scale with 7 categories: respect for tissue; time and motion; instrument handling; knowledge of instruments; flow of operation; use of assistants; and knowledge of specific procedure. Data were then stratified by faculty versus resident (self-) assessment; postgraduate year level; and difficulty of procedure. Descriptive statistics (means and SDs) were calculated, and the results were compared using the Wilcoxon signed-rank test and Student t-test. A p value < 0.05 was considered statistically significant. RESULTS Six faculty members, 1 fellow, and 8 residents completed evaluations for 299 procedures, including 32 ventriculoperitoneal (VP) shunt revisions, 23 VP shunt placements, 19 endoscopic third ventriculostomies, and 18 craniotomies for tumor resection. There was no significant difference between faculty and resident self-assessment scores overall or in any of the 7 domains scores for each of the involved residents. On self-assessment, senior residents scored themselves significantly higher (p < 0.02) than junior residents overall and in all domains except for “time and motion.” Faculty members scored senior residents significantly higher than junior residents only for the “knowledge of instruments” domain (p = 0.05). When procedure difficulty was considered, senior residents’ scores from faculty members were significantly higher (p = 0.04) than the scores given to junior residents for expert procedures only. Senior residents’ self-evaluation scores were significantly higher than those of junior residents for both expert (p = 0.03) and novice (p = 0.006) procedures. CONCLUSIONS OSATS is a feasible and reliable assessment tool for the comprehensive evaluation of neurosurgery resident performance in the operating room. The authors plan to use this tool to assess resident operative skill development and to improve direct resident feedback.

OBJECT The purpose of this study focusing on fusion rate was to determine the efficacy of recombinant human bone morphogenetic protein–2 (rhBMP-2) use in posterior instrumented fusions of the craniocervical junction in the pediatric population. The authors previously reported the short-term (mean follow-up 11 months) safety and efficacy of rhBMP-2 use in the pediatric age group. The present study reports on their long-term results (minimum of 12 months' follow-up) and focuses on efficacy. METHODS The authors performed a retrospective review of 83 consecutive pediatric patients who had undergone posterior occipitocervical or atlantoaxial spine fusion at Texas Children's Hospital or Riley Children's Hospital during the period from October 2007 to October 2012. Forty-nine patients were excluded from further analysis because of death, loss to follow-up, or lack of CT evaluation of fusion at 12 or more months after surgery. Fusion was determined by postoperative CT scan at a minimum of 12 months after surgery. The fusion was graded and classified by a board-certified fellowship-trained pediatric neuroradiologist. Other factors, such as patient age, diagnosis, number of vertebral levels fused, use of allograft or autograft, dosage of bone morphogenetic protein (BMP), and use of postoperative orthosis, were recorded. RESULTS Thirty-four patients had a CT scan at least 12 months after surgery. The average age of the patients at surgery was 8 years, 1 month (range 10 months–17 years). The mean follow-up was 27.7 months (range 12–81 months). There were 37 fusion procedures in 34 patients. Solid fusion (CT Grade 4 or 4−) was achieved in 89.2% of attempts (33 of 37), while incomplete fusion or failure of fusion was seen in 10.8%. Based on logistic regression analysis, there was no significant association between solid fusion and age, sex, BMP dose, type of graft material, use of postoperative orthosis, or number of levels fused. Three of 34 patients (8.8%) required revision surgery. CONCLUSIONS Despite the large number of adult studies reporting positive effects of BMP on bone fusion, our long-term outcomes using rhBMP-2 in the pediatric population suggest that rates of fusion failure are higher than observed in contemporary adult and pediatric reports of occipitocervical and atlantoaxial spine fusions.

OBJECT Grade II spinal cord ependymomas occurring in pediatric patients are exceptionally rare neoplasms. In this paper the authors use a national cancer database to determine patient demographics, treatment patterns, and associated outcomes of this cohort. METHODS The Surveillance Epidemiology and End Results (SEER) database was used to analyze subjects younger than 18 years with histologically confirmed diagnoses of Grade II spinal cord ependymoma from the years 1973 to 2008. Descriptive data on the demographic characteristics of this cohort and the associated treatment patterns are shown. The Kaplan-Meier method was used to estimate overall survival at 1, 2, 5, and 10 years. RESULTS This cohort comprised 64 pediatric subjects with Grade II spinal ependymoma. The median age was 13 years, nearly half of the patients were male, and most were white (84%). The median follow-up was 9.2 years. Overall survival at 5 and 10 years was 86% and 83%, respectively. Gross-total resection was achieved in 57% of subjects, and radiation therapy was administered to 36%. Radiation therapy was administered to 78% of subjects after subtotal resection but only to 19% of patients after gross-total resection; this difference was significant (p < 0.001). In a multivariate regression model analyzing sex, age at diagnosis, year of diagnosis, radiotherapy, and extent of resection, female sex was found to be an independent predictor of decreased mortality (HR 0.15 [95% CI 0.02–0.94], p = 0.04). CONCLUSIONS These data show long-term outcomes for pediatric patients with Grade II spinal ependymoma. Radiotherapy was more likely to be administered in cases of subtotal resection than in cases of gross-total resection. Female sex is associated with decreased mortality, while other demographic or treatment modalities are not.

OBJECT The authors have previously reported on their early experience with sublaminar polyester bands in spine surgery. In this paper, the authors describe the use of sublaminar polyester bands in long-segment posterior instrumented spinal fusions from the upper thoracic spine to the ilium in 21 children and transitional adults with progressive neuromuscular scoliosis. Transitional adults were patients older than 18 years of age with a spinal disorder of pediatric onset, such as spina bifida. This dedicated study represents the first reported use of polyester bands in spine surgery for neuromuscular scoliosis in this patient population in the US. METHODS The authors retrospectively reviewed the demographics and procedural data of patients who underwent posterior instrumented fusion using sublaminar polyester bands for neuromuscular scoliosis. RESULTS Twenty-one pediatric and adult transitional patients, ranging in age from 10 to 20 years (mean 14 years), underwent posterior instrumented fusion for progressive neuromuscular scoliosis. The average coronal Cobb angle measured 66° before surgery (range 37°–125°). Immediately after surgery, the mean coronal Cobb angle was 40° (range 13°–85°). At last follow-up, the average coronal Cobb angle was maintained at 42° (range 5°–112°). Regarding sagittal parameters, thoracic kyphosis was restored by 8%, and lumbar lordosis improved by 20% after surgery. Mean follow-up duration was 17 months (range 2–54 months). One patient with an aborted procedure due to loss of intraoperative evoked potentials was excluded from the analysis of radiographic outcomes. Mean surgical time was 7 hours 43 minutes (range 3 hours 59 minutes to 10 hours 23 minutes). All patients received either a 12- or 24-mg dose of recombinant human bone morphogenetic protein–2. Average estimated blood loss was 976 ml (range 300–2700 ml). Complications directly related to the use of sublaminar instrumentation included transient proprioceptive deficit (1 patient) and prolonged paraparesis (1 patient). Other complications noted in this series included disengagement of the rod from an iliac screw (1 patient), proximal junctional kyphosis (1 patient), noninfected wound drainage (2 patients), and perioperative death (1 patient). The lessons learned from these complications are discussed. CONCLUSIONS Pedicle screws, laminar/pedicle/transverse process hooks, and sublaminar metal wires have been incorporated into posterior spinal constructs and widely reported and used in the thoracic and lumbar spines and sacrum with varying success. This report demonstrates the satisfactory radiological outcomes of hybrid posterior spinal constructs in pediatric and adult neuromuscular scoliosis that include sublaminar polyester bands that promise the technical ease of passing sublaminar instrumentation with the immediate biomechanical rigidity of pedicle screws and hooks. However, the high neurological complication rate associated with this technique (2/21, or 10%) tempers the acceptable radiographic outcomes.

Spondyloptosis refers to complete dislocation of a vertebral body onto another. The L5–S1 level is frequently affected. As this condition is rare, few published reports describing its clinical features and surgical outcomes exist, especially in the pediatric patient population. The authors report the presentation, pathological findings, and radiographic studies of a 2-year-old girl who presented to Texas Children's Hospital with a history since birth of progressive spastic paraparesis. Preoperative CT and MRI showed severe spinal cord compression associated with T11–12 spondyloptosis. The patient underwent a single-stage posterior approach for complete resection of the dysplastic vertebral bodies at the apex of the spinal deformity with reconstruction and stabilization of the vertebral column using a titanium expandable cage and pedicle screws. At the 12-month follow-up, the patient remained neurologically stable without any radiographic evidence of instrumentation failure or loss of alignment. To the best of the authors' knowledge, there have been only 2 other children with congenital thoracolumbar spondyloptosis treated with the above-described strategy. The authors describe their case and review the literature to discuss the aggregate clinical features, surgical strategies, and operative outcomes for congenital thoracolumbar spondyloptosis.

BACKGROUND: Malignant epithelioid glioneuronal tumor (MEGNT) is a rare high-grade brain tumor that exhibits both glial and neuronal differentiation and is not included in the current World Health Organization (WHO) classification. Limited case series describe a highly aggressive tumor with poor survival and have shed little light on MEGNT biology. AIM: The objective of our study was to molecularly characterize a cohort of MEGNTs in order to provide insight into tumor biology and identify potential therapeutic targets. METHODS: An integrated genomic strategy, including exome, transcriptome, and targeted mutation panel sequencing as well as copy number array analysis, was used to evaluate tumor samples obtained from 8 children with MEGNT. Clinical information was obtained by chart review. RESULTS: Outcomes were dismal for these patients despite aggressive surgery (gross total resection x 6) followed by involved field radiation therapy (XRT), with a median event-free survival of 6 months and 4 of 8 patients dead of disease. Frequent and potentially targetable genetic alterations were detected in the MAPK pathway in 4 of 8 cases (BRAF V600E x 3, NTRK2-BEND5) and in PI3K/MTOR pathway genes in 3 of 8 cases (TSC1 x 2, PTEN). Additional aberrations related to the MAPK pathway were identified in 4 patients, including high copy gains in MET and KIT and 2 novel fusions (SOS1-MAP4K3 and MAP3K11-GFAP) which were validated by targeted sequencing and require further functional characterization. Other alterations in known high-grade glioma genes were also detected, including homozygous CDKN2A/B loss (n=3), TP53 mutation (n=2), and amplification events involving MYCN, CDK6, MET, and KIT. CONCLUSION: MEGNT is an aggressive tumor with poor response to conventional therapies. The frequency of potentially-targetable molecular alterations identified in this study indicates an opportunity to test the use of novel targeted agents for children with these tumors.

OBJECTIVETethered cord syndrome is the clinical manifestation of an abnormal stretch on the spinal cord, presumably causing mechanical injury, a compromised blood supply, and altered spinal cord metabolism. Tethered cord release is the standard treatment for tethered cord syndrome. However, direct untethering of the spinal cord carries potential risks, such as new neurological deficits from spinal cord injury, a CSF leak from opening the dura, and retethering of the spinal cord from normal scar formation after surgery. To avoid these risks, the authors applied spinal column shortening to children and transitional adults with primary and secondary tethered cord syndrome and report treatment outcomes. The authors' aim with this study was to determine the safety and efficacy of spinal column shortening for tethered cord syndrome by analyzing their experience with this surgical technique.METHODSThe authors retrospectively reviewed the demographic and procedural data of children and young adults who had undergone spinal column shortening for primary or secondary tethered cord syndrome.RESULTSSeven patients with tethered cord syndrome caused by myelomeningocele, lipomyelomeningocele, and transitional spinal lipoma were treated with spinal column shortening. One patient with less than 24 months of follow-up was excluded from further analysis. There were 3 males and 4 females; the average age at the time was surgery was 16 years (range 8–30 years). Clinical presentations for our patients included pain (in 5 patients), weakness (in 4 patients), and bowel/bladder dysfunction (in 4 patients). Spinal column osteotomy was most commonly performed at the L-1 level, with fusion between T-12 and L-2 using a pedicle screw-rod construct. Pedicle subtraction osteotomy was performed in 6 patients, and vertebral column resection was performed in 1 patient. The average follow-up period was 31 months (range 26–37 months). Computed tomography–based radiographic outcomes showed solid fusion and no instrumentation failure in all cases by the most recent follow-up. Five of 7 patients (71%) reported improvement in preoperative symptoms during the follow-up period. The mean differences in initial and most recent Scoliosis Research Society Outcomes Questionnaire and Oswestry Disability Index scores were 0.26 and –13%, respectively; minimum clinically important difference in SRS-22 and ODI were assumed to be 0.4% and –12.8%, respectively.CONCLUSIONSSpinal column shortening seems to represent a safe and efficacious alternative to traditional untethering of the spinal cord for tethered cord syndrome.

In this issue of the Journal of Neurosurgery: Spine, Professor Atul Goel submits a single-institution, singlesurgeon retrospective analysis of 65 adult and pediatric patients with “complex” Chiari malformations (CMs), that is, CM Type I associated with complex bony anomalies of the craniocervical junction and brainstem compression.4 Professor Goel is respected as a clinical pioneer and thought leader for disorders at the craniocervical junction. He asserts that the basic pathophysiological mechanism for CM with or without basilar invagination and with or without syringomyelia is C1–2 instability. This assertion challenges conventional theories. Bollo et al.1 recently thoroughly described this entity in the pediatric neurosurgical literature; the authors identified basilar invagination, CM 1.5, and a clivoaxial angle < 125° as patient risk factors necessitating reduction and occipitocervical fusion in addition to mandatory suboccipital decompression. In the present study Goel speculates that CM and syringomyelia are “Nature’s protective mechanism” of the body and compares CM to an “air bag.” This speculation, at best, is unsupported and unsubstantiated by evidence in the study and the literature and, at worst, sounds flowery and sophomoric. This literary metaphor seems out of place in a scientific journal. Professor Goel attempts to demonstrate in his series a new paradigm for treating symptomatic CM, that is, C1–2 posterior instrumented fusion alone using C-1 lateral mass screws and C-2 pars or pedicle screws—without any attempt at CM decompression and without occipitocervical fusion. Notably, in his series, there was one death (1.5%) from vertebral artery injury (out of 3 total vertebral artery injuries; approximately 5% rate of injury) while exposing entry points for the C-1 lateral mass screws. Professor Goel managed these patients with a C1–2 posterior instrumented fusion. As part of the preparation for placing the C-1 lateral mass screws, anomalous vertebral arteries needed to be “dissected” away from the C-1 facet process. Why? This seems completely unnecessary and adds significant risk to the procedure (vertebral artery injury or spasm). The C-1 arch is already fused to the occiput in more than a few examples provided as figures; therefore, fusing from the occiput (with occipital screws and plates) to C-2 would be a biomechanically equivalent construct, without the loss of additional range of motion at the craniocervical junction and without having to manipulate the vertebral arteries. Related to the above point, basilar invagination and/or platybasia are typically regarded as signs of craniocervical junction instability, and an occipitocervical construct is usually indicated. Moreover, in a subgroup of patients (Group A) in Professor Goel’s study, cervical traction was applied intraoperatively “to reduce basilar invagination and restore craniovertebral alignment.” How does a C1–2 fusion alone maintain this reduction? Again, occipitocervical fusion seems necessary to maintain this reduction. Mortality rates for CM decompression surgery with or without duraplasty range from 0% to 0.9% in large series.5,6 Vertebral artery injury rates range from 0% to 0.7% for posterior fossa decompression with and without duraplasty for the surgical treatment of CM.2 Professor Goel’s mortality rate and vertebral artery injury rate using this new surgical approach for CM seem alarmingly high, unjustified, and lie outside of published benchmarks for the surgical treatment of CM using suboccipital craniectomy plus or minus duraplasty. While the patient-reported outcomes seem very satisfactory (63 [97%] of 65) on health-related quality of life (HRQOL) outcome surveys (Japanese Orthopaedic Asso

OBJECTIVE There is limited literature on patient-reported outcomes (PROs) and health-related quality of life (HRQOL) outcomes in pediatric patients undergoing surgery for craniovertebral junction pathology. The aim of the present study was to assess surgical and quality of life outcomes in children who had undergone occipitocervical or atlantoaxial fusion. METHODS The authors retrospectively reviewed the demographics, procedural data, and outcomes of 77 consecutive pediatric patients who underwent posterior occipitocervical or atlantoaxial fusion between 2008 and 2015 at Texas Children's Hospital. Outcome measures (collected at last follow-up) included mortality, neurological improvement, complications, Scoliosis Research Society Outcomes Measure–22 (SRS-22) score, SF-36 score, Neck Disability Index (NDI), and Pediatric Quality of Life Inventory (PedsQL). Multivariate linear regression analysis was performed to identify factors affecting PROs and HRQOL scores at follow-up. RESULTS The average age in this series was 10.6 ± 4.5 years. The median follow-up was 13.9 months (range 0.5–121.5 months). Sixty-three patients (81.8%) were treated with occipitocervical fusion, and 14 patients (18.1%) were treated with atlantoaxial fusion. The American Spinal Injury Association (ASIA) grade at discharge was unchanged in 73 patients (94.8%). The average PRO metrics at the time of last follow-up were as follows: SRS-22 score, 4.2 ± 0.8; NDI, 3.0 ± 2.6; the parent's PedsQL (ParentPedsQL) score, 69.6 ± 22.7, and child's PedsQL score, 75.5 ± 18.7. Multivariate linear regression analysis revealed that older age at surgery was significantly associated with lower SRS-22 scores at follow-up (B = −0.06, p = 0.03), and the presence of comorbidities was associated with poorer ParentPedsQL scores at follow-up (B = −19.68, p = 0.03). CONCLUSIONS This study indicates that occipitocervical and atlantoaxial fusions in children preserve neurological function and are associated with acceptable PROs and ParentPedsQL scores, considering the serious nature and potential for morbidity in this patient population. However, longer follow-up and disease-specific scales are necessary to fully elucidate the impact of occipitocervical and atlantoaxial fusions on children.