The role of occipital condyle and atlas anomalies on occipital cervical fusion outcomes in Chiari malformation type I with syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia. The authors also investigated the predictive contribution of these anomalies to the occurrence of OCF following PFD (PFD+OCF). METHODS: The authors analyzed the prevalence of condylar hypoplasia and atlas arch anomalies for patients in the Park-Reeves Syringomyelia Research Consortium database who underwent PFD+OCF. Condylar hypoplasia was defined by an atlanto-occipital joint axis angle (AOJAA) ≥ 130°. Atlas assimilation and arch anomalies were identified on presurgical radiographic imaging. This PFD+OCF cohort was compared with a control cohort of patients who underwent PFD alone. The control group was matched to the PFD+OCF cohort according to age, sex, and duration of symptoms at a 2:1 ratio. RESULTS: Clinical features and radiographic atlanto-occipital joint parameters were compared between 19 patients in the PFD+OCF cohort and 38 patients in the PFD-only cohort. Demographic data were not significantly different between cohorts (p > 0.05). The mean AOJAA was significantly higher in the PFD+OCF group than in the PFD group (144° ± 12° vs 127° ± 6°, p < 0.0001). In the PFD+OCF group, atlas assimilation and atlas arch anomalies were identified in 10 (53%) and 5 (26%) patients, respectively. These anomalies were absent (n = 0) in the PFD group (p < 0.001). Multivariate regression analysis identified the following 3 CVJ radiographic variables that were predictive of OCF occurrence after PFD: AOJAA ≥ 130° (p = 0.01), clivoaxial angle < 125° (p = 0.02), and occipital condyle-C2 sagittal vertical alignment (C-C2SVA) ≥ 5 mm (p = 0.01). A predictive model based on these 3 factors accurately predicted OCF following PFD (C-statistic 0.95). CONCLUSIONS: The authors' results indicate that the occipital condyle-atlas joint complex might affect the biomechanical integrity of the CVJ in children with CM-I and syringomyelia. They describe the role of the AOJAA metric as an independent predictive factor for occurrence of OCF following PFD. Preoperative identification of these skeletal abnormalities may be used to guide surgical planning and treatment of patients with complex CM-I and coexistent osseous pathology.

Disparities in Indications and Outcomes Reporting for Spinal Column Shortening for Tethered Cord Syndrome: The Need for a Standardized Approach.

STUDY DESIGN: Systematic review. OBJECTIVE: To identify commonly reported indications and outcomes in spinal column shortening (SCS) procedures. SUMMARY OF BACKGROUND DATA: SCS is a surgical procedure used in patients with tethered cord syndrome (TCS)-characterized by abnormal attachment of neural components to surrounding tissues-to shorten the vertebral column, release tension on the spinal cord/neural elements, and alleviate associated symptoms. METHODS: PubMed and EMBASE searches captured SCS literature published between 1950 and 2023. Prospective/retrospective cohort studies and case series were included without age limit or required follow-up period. Review articles without new patient presentations, meta-analyses, systematic reviews, conference abstracts, and letters were excluded. Studies included adult and pediatric patients. RESULTS: The 29 identified studies represented 278 patients (age 5-76 y). In 24.1% of studies, patients underwent primary TCS intervention via SCS. In 41.4% of studies, patients underwent SCS after failed previous primary detethering (24.1% of studies were mixed and 10.3% were unspecified). The most commonly reported non-genitourinary/bowel surgical indications were back pain (55.2%), lower-extremity pain (48.3%), lower-extremity weakness (48.3%), lower-extremity numbness (34.5%), and lower-extremity motor dysfunction (34.5%). Genitourinary/bowel symptoms were most often described as nonspecific bladder dysfunction (58.6%), bladder incontinence (34.5%), and bowel dysfunction (31.0%). After SCS, non-genitourinary/bowel outcomes included lower-extremity pain (44.8%), back pain (31.0%), and lower-extremity sensory and motor function (both 31.0%). Bladder dysfunction (79.3%), bowel dysfunction (34.5%), and bladder incontinence (13.8%) were commonly reported genitourinary/bowel outcomes. In total, 40 presenting surgical indication categories and 33 unique outcome measures were reported across studies. Seventeen of the 278 patients (6.1%) experienced a complication. CONCLUSION: The SCS surgical literature displays variability in operative indications and postoperative outcomes. The lack of common reporting mechanisms impedes higher-level analysis. A standardized outcomes measurement tool, encompassing both patient-reported outcome measures and objective metrics, is necessary. LEVEL OF EVIDENCE: Level 4.

Morphological and ultrastructural investigation of the posterior atlanto-occipital membrane: Comparing children with Chiari malformation type I and controls.

INTRODUCTION: The fibrous posterior atlanto-occipital membrane (PAOM) at the craniocervical junction is typically removed during decompression surgery for Chiari malformation type I (CM-I); however, its importance and ultrastructural architecture have not been investigated in children. We hypothesized that there are structural differences in the PAOM of patients with CM-I and those without. METHODS: In this prospective study, blinded pathological analysis was performed on PAOM specimens from children who had surgery for CM-I and children who had surgery for posterior fossa tumors (controls). Clinical and radiographic data were collected. Statistical analysis included comparisons between the CM-I and control cohorts and correlations with imaging measures. RESULTS: A total of 35 children (mean age at surgery 10.7 years; 94.3% white) with viable specimens for evaluation were enrolled: 24 with CM-I and 11 controls. There were no statistical demographic differences between the two cohorts. Four children had a family history of CM-I and five had a syndromic condition. The cohorts had similar measurements of tonsillar descent, syringomyelia, basion to C2, and condylar-to-C2 vertical axis (all p>0.05). The clival-axial angle was lower in patients with CM-I (138.1 vs. 149.3 degrees, p = 0.016). Morphologically, the PAOM demonstrated statistically higher proportions of disorganized architecture in patients with CM-I (75.0% vs. 36.4%, p = 0.012). There were no differences in PAOM fat, elastin, or collagen percentages overall and no differences in imaging or ultrastructural findings between male and female patients. Posterior fossa volume was lower in children with CM-I (163,234 mm3 vs. 218,305 mm3, p<0.001), a difference that persisted after normalizing for patient height (129.9 vs. 160.9, p = 0.028). CONCLUSIONS: In patients with CM-I, the PAOM demonstrates disorganized architecture compared with that of control patients. This likely represents an anatomic adaptation in the presence of CM-I rather than a pathologic contribution.

Disparities in indications and outcomes reporting for pediatric tethered cord surgery: The need for a standardized outcome assessment tool.

PURPOSE: Tethered cord syndrome (TCS) is characterized by abnormal attachment of the spinal cord neural elements to surrounding tissues. The most common symptoms include pain, motor or sensory dysfunction, and urologic deficits. Although TCS is common in children, there is a significant heterogeneity in outcomes reporting. We systematically reviewed surgical indications and postoperative outcomes to assess the need for a grading/classification system. METHODS: PubMed and EMBASE searches identified pediatric TCS literature published between 1950 and 2023. Studies reporting surgical interventions, ≥ 6-month follow-up, and ≥ 5 patients were included. RESULTS: Fifty-five studies representing 3798 patients were included. The most commonly reported non-urologic symptoms were nonspecific lower-extremity motor disturbances (36.4% of studies), lower-extremity/back pain (32.7%), nonspecific lower-extremity sensory disturbances (29.1%), gait abnormalities (29.1%), and nonspecific bowel dysfunction/fecal incontinence (25.5%). Urologic symptoms were most commonly reported as nonspecific complaints (40.0%). After detethering surgery, retethering was the most widely reported non-urologic outcome (40.0%), followed by other nonspecific findings: motor deficits (32.7%), lower-extremity/back/perianal pain (18.2%), gait/ambulation function (18.2%), sensory deficits (12.7%), and bowel deficits/fecal incontinence (12.7%). Commonly reported urologic outcomes included nonspecific bladder/urinary deficits (27.3%), bladder capacity (20.0%), bladder compliance (18.2%), urinary incontinence/enuresis/neurogenic bladder (18.2%), and nonspecific urodynamics/urodynamics score change (16.4%). CONCLUSION: TCS surgical literature is highly variable regarding surgical indications and reporting of postsurgical outcomes. The lack of common data elements and consistent quantitative measures inhibits higher-level analysis. The development and validation of a standardized outcomes measurement tool-ideally encompassing both patient-reported outcome and objective measures-would significantly benefit future TCS research and surgical management.

Ten-Year Analysis of Complications Related to Simple Basilar Skull Fractures in Children Presenting to a Trauma Center.

OBJECTIVES: Head trauma is a common presenting complaint among children requiring urgent medical attention, accounting for more than 600,000 emergency department (ED) visits annually, 4% to 30% of which identify skull fractures among the patient's injuries. Previous literature shows that children with basilar skull fractures (BSFs) are usually admitted for observation. We studied whether children with an isolated BSF have complications precluding them from safe discharge home from the ED. METHODS: We performed a retrospective review of ED patients aged 0 to 18 years given a simple BSF diagnosis (defined by nondisplaced fracture, with normal neurologic examination, Glasgow Coma Score of 15, no intracranial hemorrhage, no pneumocephalus) during a 10-year period to identify complications associated with their injury. Complications were defined as death, vascular injury, delayed intracranial hemorrhage, sinus thrombosis, or meningitis. We also considered hospital length of stay (LOS) longer than 24 hours or any return visit within 3 weeks of the original injury. RESULTS: Of the 174 patients included in the analysis, there were no deaths, cases of meningitis, vascular injury, nor delayed bleeding events. Thirty (17.2%) patients required a hospital LOS longer than 24 hours and 9 (5.2%) returned to the hospital within 3 weeks of discharge. Of those with LOS longer than 24 hours, 22 (12.6%) patients needed subspecialty consultation or intravenous fluids, 3 (1.7%) had cerebrospinal fluid leak, and 2 (1.2%) had a concern for facial nerve abnormality. On the return visits, only 1 (0.6%) patient required readmission for intravenous fluids because of nausea and vomiting. CONCLUSIONS: Our findings suggest that patients with uncomplicated BSFs can be safely discharged from the ED if the patient has reliable follow-up, is tolerating oral fluids, has no evidence of cerebrospinal fluid leak, and has been evaluated by appropriate subspecialists before discharge.

Combined endovascular and skull base surgical management of pediatric craniocervical pathology: a case series.

OBJECTIVE: Pathological bony abnormalities of the craniocervical region in children sometimes require surgical intervention as part of their management. Rarely, abnormal skeletal or vascular anatomy can render traditional surgical techniques ineffective because of the risk of injury to the vertebral artery. To mitigate these risks, a combined endovascular and skull base approach was devised. The authors describe their experience using vertebral artery sacrifice as an adjunctive surgical method to reduce the risk of inadvertent vertebral artery injury during surgical correction of pediatric craniocervical deformity. METHODS: Three patients underwent vertebral artery sacrifice for structural craniocervical pathologies (1 male, 2 females; ages 12, 14, and 3 years). One patient presented with basilar invagination odontogenic brainstem compression, and the other 2 patients presented with congenital cervical fusion. All patients underwent endovascular left vertebral artery sacrifice after passing balloon test occlusion. RESULTS: No adverse effects from the vertebral artery sacrifice were observed. At the last follow-ups (35, 30, and 32 months), all 3 patients had a satisfactory outcome with no adverse effects as a result of their sacrificed artery. CONCLUSIONS: Endovascular vertebral artery sacrifice followed by skull base approaches can be used to effectively and safely treat craniocervical pathology from a variety of pediatric skeletal abnormalities.

Pediatric cervical spine clearance: A 10-year evaluation of multidetector computed tomography at a level 1 pediatric trauma center.

INTRODUCTION: Efficient and accurate evaluation of the pediatric cervical spine (c-spine) for both injury identification and posttraumatic clearance remains a challenge. We aimed to determine the sensitivity of multidetector computed tomography (MDCT) for identification of cervical spine injuries (CSIs) in pediatric blunt trauma patients. METHODS: A retrospective cohort study was conducted at a level 1 pediatric trauma center from 2012 to 2021. All pediatric trauma patients age younger than 18 years who underwent c-spine imaging (plain radiograph, MDCT, and/or magnetic resonance imaging [MRI]) were included. All patients with abnormal MRIs but normal MDCTs were reviewed by a pediatric spine surgeon to assess specific injury characteristics. RESULTS: A total of 4,477 patients underwent c-spine imaging, and 60 (1.3%) were diagnosed with a clinically significant CSI that required surgery or a halo. These patients were older, more likely to be intubated, have a Glasgow Coma Scale score of <14, and more likely to be transferred in from a referring hospital. One patient with a fracture on radiography and neurologic symptoms got an MRI and no MDCT before operative repair. All other patients who underwent surgery including halo placement for a clinically significant CSI had their injury diagnosed by MDCT, representing a sensitivity of 100%. There were 17 patients with abnormal MRIs and normal MDCTs; none underwent surgery or halo placement. Imaging from these patients was reviewed by a pediatric spine surgeon, and no unstable injuries were identified. CONCLUSION: Multidetector computed tomography appears to have 100% sensitivity for detecting clinically significant CSIs in pediatric trauma patients, regardless of age or mental status. Forthcoming prospective data will be useful to confirm these results and inform recommendations for whether pediatric c-spine clearance can be safely performed based on the results of a normal MDCT alone. LEVEL OF EVIDENCE: Diagnostic Tests or Criteria; Level IV.

Hanging and Strangulation Injuries: An Institutional Review From a Level 1 Pediatric Trauma Center.

BACKGROUND: We sought to define the incidence and outcomes of pediatric hanging and strangulation injuries to inform best practices for trauma triage and management. METHODS: A retrospective review was conducted that included all patients who presented after hanging or strangulation to a Level I Pediatric Trauma Center from 2011 through 2021. Patient demographics, injury characteristics, and clinical outcomes were collected. All imaging modalities of the head and neck were reviewed to determine if a bony fracture or vascular injury was present. RESULTS: Over the 11-year study period, 128 patients met inclusion criteria. The median age of the cohort was 13 years [IQR: 8.5-15], most patients were male (60.9%), and the median GCS was 11 [3, 15]. There were 96 cases (75%) that were intentional injuries. 76 patients (59.4%) received imaging in the form of plain radiographs, CT, or MRI of the neck and cervical spine. No fractures were identified and there were 0 clinically significant cervical spine injuries. CT angiograms of the neck identified no cerebral vascular injuries. Mortality was high (32%), and 25% of patients with nonaccidental injuries had a documented prior suicide attempt. CONCLUSION: We identified no cervical spine fractures and no blunt cerebral vascular injuries after a hanging or strangulation in over 10 years at a Level 1 Pediatric Trauma Center. Use of CT and CT angiography of the neck and cervical spine should be minimized in this patient population without high clinical index of suspicion and/or significant mechanism. LEVEL OF EVIDENCE: IV.

Building consensus for the medical management of children with moderate and severe acute spinal cord injury: a modified Delphi study.

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.

A likely HOXC4 predisposition variant for Chiari malformations.

OBJECTIVE: Inherited variants predisposing patients to type 1 or 1.5 Chiari malformation (CM) have been hypothesized but have proven difficult to confirm. The authors used a unique high-risk pedigree population resource and approach to identify rare candidate variants that likely predispose individuals to CM and protein structure prediction tools to identify pathogenicity mechanisms. METHODS: By using the Utah Population Database, the authors identified pedigrees with significantly increased numbers of members with CM diagnosis. From a separate DNA biorepository of 451 samples from CM patients and families, 32 CM patients belonging to 1 or more of 24 high-risk Chiari pedigrees were identified. Two high-risk pedigrees had 3 CM-affected relatives, and 22 pedigrees had 2 CM-affected relatives. To identify rare candidate predisposition gene variants, whole-exome sequence data from these 32 CM patients belonging to 24 CM-affected related pairs from high-risk pedigrees were analyzed. The I-TASSER package for protein structure prediction was used to predict the structures of both the wild-type and mutant proteins found here. RESULTS: Sequence analysis of the 24 affected relative pairs identified 38 rare candidate Chiari predisposition gene variants that were shared by at least 1 CM-affected pair from a high-risk pedigree. The authors found a candidate variant in HOXC4 that was shared by 2 CM-affected patients in 2 independent pedigrees. All 4 of these CM cases, 2 in each pedigree, exhibited a specific craniocervical bony phenotype defined by a clivoaxial angle less than 125°. The protein structure prediction results suggested that the mutation considered here may reduce the binding affinity of HOXC4 to DNA. CONCLUSIONS: Analysis of unique and powerful Utah genetic resources allowed identification of 38 strong candidate CM predisposition gene variants. These variants should be pursued in independent populations. One of the candidates, a rare HOXC4 variant, was identified in 2 high-risk CM pedigrees, with this variant possibly predisposing patients to a Chiari phenotype with craniocervical kyphosis.

Combined transnasal/transoral endoscopic odontoid resection in pediatric patients: Otolaryngologic considerations to airway management, endoscopic exposure, and complication management.

BACKGROUND: Anterior brainstem compression from odontoid pathology can occur in patients with craniocervical disorders. Occasionally, odontoid resection is required. In adults, odontoid resection has evolved toward transnasal-only endoscopic techniques. Pediatric patients, however, pose special challenges due to abnormal anatomy and smaller working spaces. A combined transnasal/transoral endoscopic odontoid resection (TN/TO EOR) can overcome this limitation. We present a case series with emphasis on otolaryngologic considerations to airway management, endoscopic approach, and management of complications. METHODS: A single center, retrospective review of patients aged ≤18 undergoing combined transnasal/transoral endoscopic odontoid resection between 2011 and 2022 is presented. Clinical and surgical variables consisting of diagnosis, intubation approach, other airway procedures performed, symptoms, complications, blood loss, and time to extubation, return to oral feeding, and discharge were recorded. RESULTS: 19 patients aged 10.7 ± 4.3 (range: 3-18) were included. Diagnoses included congenital syndrome (n = 6), complex Chiari malformation (n = 11), and congenital syndrome with Chiari (n = 2). Patients commonly required indirect videolaryngoscopy for intubation, with or without fiberoptic endoscopic assistance. Seven underwent adenoidectomy, two underwent adenotonsillectomy, and one required adenoidectomy with midline palatal split and inferior turbinate outfracture. Four patients had undergone prior adenotonsillectomy. Presenting symptoms included extremity weakness (n = 9), dysphagia (n = 8), velopharyngeal insufficiency (n = 4), sleep disturbance (n = 5), and headaches (n = 8). Four patients had complications, including one re-operation for residual odontoid, one flap dehiscence, one cerebrospinal fluid (CSF) leak repaired primarily, and one complicated course including temporary spinal cord injury. Blood loss was 50 ± 43 cc (median 30). Time to extubation was 1.1 ± 2.1 days (median 0; one patient underwent tracheotomy for respiratory failure), time to oral intake was 2.9 ± 3.7 days (median 1), and time to discharge was 7.1 ± 7.5 days (median 4). CONCLUSIONS: A combined transnasal/transoral approach can be successfully used in pediatric patients to overcome difficult endoscopic access. Although complications exist, early extubation and return to oral intake occurs in the vast majority of cases. For pediatric TN/TO EOR, the otolaryngologist plays a key role in preoperative assessment, airway management, endoscopic exposure, and complication management.

Complex Chiari Malformations: Diagnosis, Evaluation, and Treatment.

The term "complex Chiari malformation" (CCM) refers to a subset of clinical and radiographic findings that describe a subpopulation of Chiari patients with craniocervical kyphosis and secondary brainstem compression. These patients are at a greater risk for unsuccessful surgical treatment with standard Chiari surgical decompressive procedures and may require craniocervical fusion and/or odontoid resection. This article reviews concepts related to the diagnosis, management, and treatment of CCM and discusses possible directions for future research.

Developing consensus for the management of pediatric cervical spine disorders and stabilization: a modified Delphi study.

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.

The Fate of the Cervical Collar: An Observational Pilot Study Investigating Follow-up Care After Emergency Department Discharge in Children With Mild Traumatic Neck Injuries.

OBJECTIVES: After evaluation and treatment of minor traumatic cervical spine injury (CSI), many children are discharged home in a rigid cervical orthosis (RCO). This study investigated their adherence to RCO treatment recommendations. The feasibility of telehealth cervical spine clearance was also explored. METHODS: This was a prospective observational study of children 3 to 18 years old with mild CSI evaluated at a level I pediatric trauma center from December 1, 2019, through July 31, 2021. Before emergency department discharge, patients received RCO use instructions and recommendation for follow-up with in-person neurosurgery clinic visit, neurosurgery telehealth visit, or in-person primary care provider visit. The family was responsible for arranging follow-up. Primary outcomes included compliance with follow-up and collar use. RESULTS: Ninety-eight children (mean age, 11.3 ± 4.1 years) were included. Overall, follow-up contact was available for 51 patients (52%). At 1-week follow-up with 36 children, 64% were collar compliant, 13 had no pain (38% remained in RCO), 14 had mild pain without limitations, 8 had pain with some limitations, and 1 had significant pain. At 2-week follow-up with 31 children, 9 (29%) were collar compliant, 23 had no pain, 7 had mild pain without limitations, and 1 with significant persistent pain was found to have an odontoid fracture requiring C1-2 fusion. Patients/families often discontinued the use of the collar without follow-up (47%). Approximately half utilized a recommended clinical follow-up option for clearance, most often in neurosurgery clinic or using a neurosurgery telehealth visit. The mean time to follow-up was 11.34 ± 4.9 days (range, 3-25 days), and mean collar compliance lasted 9.8 ± 5.7 days (range, 1-25 days). No child experienced any short-term complications related to RCO use. CONCLUSIONS: In this pilot study, a substantial portion of children with mild CSIs discharged from the emergency department with an RCO did not adhere to compliance or follow-up recommendations. Persistent pain requires further evaluation.

Initial experience with Pipeline embolization of intracranial pseudoaneurysms in pediatric patients.

OBJECTIVE: Flow-diverting devices have been used successfully for the treatment of complex intracranial vascular injuries in adults, but the role of these devices in treating iatrogenic and traumatic intracranial vascular injuries in children remains unclear. The authors present their experience using the Pipeline embolization device (PED) for treating intracranial pseudoaneurysms in children. METHODS: This single-center retrospective cohort study included pediatric patients with traumatic and iatrogenic injuries to the intracranial vasculature that were treated with the PED between 2015 and 2021. Demographic data, indications for treatment, the number and sizes of PEDs used, follow-up imaging, and clinical outcomes were analyzed. RESULTS: Six patients with a median age of 12 years (range 7-16 years) underwent PED placement to treat intracranial pseudoaneurysms. There were 3 patients with hemorrhagic presentation, 2 with ischemia, and 1 in whom a growing pseudoaneurysm was found on angiography. Injured vessels included the anterior cerebral artery (n = 2), the supraclinoid internal carotid artery (ICA, n = 2), the cavernous ICA (n = 1), and the distal cervical ICA (n = 1). All 6 pseudoaneurysms were successfully treated with PED deployment. One patient required re-treatment with a second PED within a week because of concern for a growing pseudoaneurysm. One patient experienced parent vessel occlusion without neurological sequelae. CONCLUSIONS: Use of the PED is feasible for the management of iatrogenic and traumatic pseudoaneurysms of the intracranial vasculature in children, even in the setting of hemorrhagic presentation.

High prevalence of gram-negative and multiorganism surgical site infections after pediatric complex tethered spinal cord surgery: a multicenter study.

OBJECTIVE: Complex tethered spinal cord (cTSC) release in children is often complicated by surgical site infection (SSI). Children undergoing this surgery share many similarities with patients undergoing correction for neuromuscular scoliosis, where high rates of gram-negative and polymicrobial infections have been reported. Similar organisms isolated from SSIs after cTSC release were recently demonstrated in a single-center pilot study. The purpose of this investigation was to determine if these findings are reproducible across a larger, multicenter study. METHODS: A multicenter, retrospective chart review including 7 centers was conducted to identify all cases of SSI following cTSC release during a 10-year study period from 2007 to 2017. Demographic information along with specific microbial culture data and antibiotic sensitivities for each cultured organism were collected. RESULTS: A total of 44 SSIs were identified from a total of 655 cases, with 78 individual organisms isolated. There was an overall SSI rate of 6.7%, with 43% polymicrobial and 66% containing at least one gram-negative organism. Half of SSIs included an organism that was resistant to cefazolin, whereas only 32% of SSIs were completely susceptible to cefazolin. CONCLUSIONS: In this study, gram-negative and polymicrobial infections were responsible for the majority of SSIs following cTSC surgery, with approximately half resistant to cefazolin. Broader gram-negative antibiotic prophylaxis should be considered for this patient population.

Complications and outcomes of posterior fossa decompression with duraplasty versus without duraplasty for pediatric patients with Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: The aim of this study was to determine differences in complications and outcomes between posterior fossa decompression with duraplasty (PFDD) and without duraplasty (PFD) for the treatment of pediatric Chiari malformation type I (CM1) and syringomyelia (SM). METHODS: The authors used retrospective and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM1-SM who received PFD or PFDD and had at least 1 year of follow-up data. Preoperative, treatment, and postoperative characteristics were recorded and compared between groups. RESULTS: A total of 692 patients met the inclusion criteria for this database study. PFD was performed in 117 (16.9%) and PFDD in 575 (83.1%) patients. The mean age at surgery was 9.86 years, and the mean follow-up time was 2.73 years. There were no significant differences in presenting signs or symptoms between groups, although the preoperative syrinx size was smaller in the PFD group. The PFD group had a shorter mean operating room time (p < 0.0001), fewer patients with > 50 mL of blood loss (p = 0.04), and shorter hospital stays (p = 0.0001). There were 4 intraoperative complications, all within the PFDD group (0.7%, p > 0.99). Patients undergoing PFDD had a 6-month complication rate of 24.3%, compared with 13.7% in the PFD group (p = 0.01). There were no differences between groups for postoperative complications beyond 6 months (p = 0.33). PFD patients were more likely to require revision surgery (17.9% vs 8.3%, p = 0.002). PFDD was associated with greater improvements in headaches (89.6% vs 80.8%, p = 0.04) and back pain (86.5% vs 59.1%, p = 0.01). There were no differences between groups for improvement in neurological examination findings. PFDD was associated with greater reduction in anteroposterior syrinx size (43.7% vs 26.9%, p = 0.0001) and syrinx length (18.9% vs 5.6%, p = 0.04) compared with PFD. CONCLUSIONS: PFD was associated with reduced operative time and blood loss, shorter hospital stays, and fewer postoperative complications within 6 months. However, PFDD was associated with better symptom improvement and reduction in syrinx size and lower rates of revision decompression. The two surgeries have low intraoperative complication rates and comparable complication rates beyond 6 months.

FEBio finite element model of a pediatric cervical spine.

OBJECTIVE: The underlying biomechanical differences between the pediatric and adult cervical spine are incompletely understood. Computational spine modeling can address that knowledge gap. Using a computational method known as finite element modeling, the authors describe the creation and evaluation of a complete pediatric cervical spine model. METHODS: Using a thin-slice CT scan of the cervical spine from a 5-year-old boy, a 3D model was created for finite element analysis. The material properties and boundary and loading conditions were created and model analysis performed using open-source software. Because the precise material properties of the pediatric cervical spine are not known, a published parametric approach of scaling adult properties by 50%, 25%, and 10% was used. Each scaled finite element model (FEM) underwent two types of simulations for pediatric cadaver testing (axial tension and cardinal ranges of motion [ROMs]) to assess axial stiffness, ROM, and facet joint force (FJF). The authors evaluated the axial stiffness and flexion-extension ROM predicted by the model using previously published experimental measurements obtained from pediatric cadaveric tissues. RESULTS: In the axial tension simulation, the model with 50% adult ligamentous and annulus material properties predicted an axial stiffness of 49 N/mm, which corresponded with previously published data from similarly aged cadavers (46.1 ± 9.6 N/mm). In the flexion-extension simulation, the same 50% model predicted an ROM that was within the range of the similarly aged cohort of cadavers. The subaxial FJFs predicted by the model in extension, lateral bending, and axial rotation were in the range of 1-4 N and, as expected, tended to increase as the ligament and disc material properties decreased. CONCLUSIONS: A pediatric cervical spine FEM was created that accurately predicts axial tension and flexion-extension ROM when ligamentous and annulus material properties are reduced to 50% of published adult properties. This model shows promise for use in surgical simulation procedures and as a normal comparison for disease-specific FEMs.

Socioeconomic and demographic factors in the diagnosis and treatment of Chiari malformation type I and syringomyelia.

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.