Rare and de novo coding variants in chromodomain genes in Chiari I malformation.

Chiari I malformation (CM1), the displacement of the cerebellum through the foramen magnum into the spinal canal, is one of the most common pediatric neurological conditions. Individuals with CM1 can present with neurological symptoms, including severe headaches and sensory or motor deficits, often as a consequence of brainstem compression or syringomyelia (SM). We conducted whole-exome sequencing (WES) on 668 CM1 probands and 232 family members and performed gene-burden and de novo enrichment analyses. A significant enrichment of rare and de novo non-synonymous variants in chromodomain (CHD) genes was observed among individuals with CM1 (combined p = 2.4 × 10-10), including 3 de novo loss-of-function variants in CHD8 (LOF enrichment p = 1.9 × 10-10) and a significant burden of rare transmitted variants in CHD3 (p = 1.8 × 10-6). Overall, individuals with CM1 were found to have significantly increased head circumference (p = 2.6 × 10-9), with many harboring CHD rare variants having macrocephaly. Finally, haploinsufficiency for chd8 in zebrafish led to macrocephaly and posterior hindbrain displacement reminiscent of CM1. These results implicate chromodomain genes and excessive brain growth in CM1 pathogenesis.

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.

Pediatric spinal instrumentation.

This article reviews the evolution of spinal instrumentation in the pediatric age group, starting with the cervical spine and atlantoaxial area and ending with the lower spine. The congenital and the acquired conditions which require instrumentation are described. The technical details regarding pediatric instrumentation are alluded to, and finally an attempt is made to predict the future of spinal instrumentation in this age group.

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.

Evaluating the utility and quality of large administrative databases in pediatric spinal neurosurgery research.

PURPOSE: The purpose of this study was to assess the quality of articles utilizing large administrative databases to answer questions related to pediatric spinal neurosurgery by quantifying their adherence to standard reporting guidelines. METHODS: A systematic literature search was conducted with search terms including "pediatric" and "neurosurgery," associated neurosurgical diagnoses, and the names of known databases. Study abstracts were reviewed to identify clinical studies involving pediatric populations, spine-related pathology or procedures, and large administrative databases. Included studies were graded using the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) criteria. RESULTS: A total of 28 papers of the initial 1496 identified met inclusion criteria. These papers involved 10 databases and had a mean study period of 11.46 ± 12.27 years. The subjects of these research papers were undergoing treatment of scoliosis (n = 5), spinal cord injury (n = 5), spinal cord tumors (n = 9), and spine surgery in general (n = 9). The mean STROBE score was 19.41 ± 2.02 (out of 22). CONCLUSION: Large administrative databases are commonly used within pediatric spine-related neurosurgical research to cover a broad spectrum of research questions and study topics. The heterogeneity of research to this point encourages the continued use of large databases to better understand treatment and diagnostic trends, perioperative and long-term outcomes, and rare pathologies within pediatric spinal neurosurgery.

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

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

Complex Chiari malformation: using craniovertebral junction metrics to guide treatment.

The complex Chiari, characterized by abnormal craniocervical bony anatomy in addition to Chiari tonsillar herniation, is a relatively recent addition to the concepts surrounding the Chiari literature. The primary findings of complex Chiari include craniocervical kyphosis and retroflexed odontoid, both of which can be described with radiographic measurements. This manuscript will outline the background literature regarding Chiari craniocervical morphometrics and supply an algorithm for the general management of complex Chiari patients.

Increased complications without neurological benefit are associated with prophylactic spinal cord untethering prior to scoliosis surgery in children with myelomeningocele.

PURPOSE: Children with myelomeningocele (MMC) are at increased risk of developing neuromuscular scoliosis and spinal cord re-tethering (Childs Nerv Syst 12:748-754, 1996; Neurosurg Focus 16:2, 2004; Neurosurg Focus 29:1, 2010). Some centers perform prophylactic untethering on asymptomatic MMC patients prior to scoliosis surgery because of concern that additional traction on the cord may place the patient at greater risk of neurologic deterioration peri-operatively. However, prophylactic untethering may not be justified if it carries increased surgical risks. The purpose of this study was to determine if prophylactic untethering is necessary in asymptomatic children with MMC undergoing scoliosis surgery. METHODS: A multidisciplinary, retrospective cohort study from seven children's hospitals was performed including asymptomatic children with MMC < 21 years old, managed with or without prophylactic untethering prior to scoliosis surgery. Patients were divided into three groups for analysis: (1) untethering at the time of scoliosis surgery (concomitant untethering), (2) untethering within 3 months of scoliosis surgery (prior untethering), and (3) no prophylactic untethering. Baseline data, intra-operative reports, and 90-day post-operative outcomes were analyzed to assess for differences in neurologic outcomes, surgical complications, and overall length of stay. RESULTS: A total of 208 patients were included for analysis (mean age 9.4 years, 52% girls). No patient in any of the groups exhibited worsened motor or sensory function at 90 days post-operatively. However, comparing the prophylactic untethering groups with the group that was not untethered, there was an increased risk of surgical site infection (SSI) (31.3% concomitant, 28.6% prior untethering vs. 12.3% no untethering; p = 0.0104), return to the OR (43.8% concomitant, 23.8% prior untethering vs. 17.4% no untethering; p = 0.0047), need for blood transfusion (51.6% concomitant, 57.1% prior untethering vs. 33.8% no untethering; p = 0.04), and increased mean length of stay (LOS) (13.4 days concomitant, 10.6 days prior untethering vs. 6.8 days no untethering; p < 0.0001). In multivariable logistic regression analysis, prophylactic untethering was independently associated with increased adjusted relative risks of surgical site infection (aRR = 2.65, 95% CI 1.17-5.02), unplanned re-operation (aRR = 2.17, 95% CI 1.02-4.65), and any complication (aRR = 2.25, 95% CI 1.07-4.74). CONCLUSION: In this study, asymptomatic children with myelomeningocele who underwent scoliosis surgery developed no neurologic injuries regardless of prophylactic untethering. However, those who underwent prophylactic untethering were more likely to experience SSIs, return to the OR, need a blood transfusion, and have increased LOS than children not undergoing untethering. Based on these data, prophylactic untethering in asymptomatic MMC patients prior to scoliosis surgery does not provide any neurological benefit and is associated with increased surgical risks.

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

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

Primary osseous tumors of the pediatric spinal column: review of pathology and surgical decision making.

Spinal column tumors are rare in children and young adults, accounting for only 1% of all spine and spinal cord tumors combined. They often present diagnostic and therapeutic challenges. In this article, the authors review the current management of primary osseous tumors of the pediatric spinal column and highlight diagnosis, management, and surgical decision making.

Unilateral fixation for treatment of occipitocervical instability in children with congenital vertebral anomalies of the craniocervical junction.

OBJECT Patients with occipitocervical (OC) instability from congenital vertebral anomalies (CVAs) of the craniocervical junction (CCJ) often have bony abnormalities that make instrumentation placement difficult. Within this patient population, some bilateral instrumentation constructs either fail or are not feasible, and a unilateral construct must be used. The authors describe the surgical management and outcomes of this disorder in patients in whom unilateral fixation constructs were used to treat OC instability. METHODS From a database of OC fusion procedures, the authors identified patients who underwent unilateral fixation for the management of OC instability. Patient characteristics, surgical details, and radiographic outcomes were reviewed. In each patient, CT scans were performed at least 4 months after surgery to evaluate for fusion. RESULTS Eight patients with CVAs of the CCJ underwent unilateral fixation for the treatment of OC instability. For 4 patients, the procedure occurred after a bilateral OC construct failed or infection forced hardware removal. For the remainder, it was the primary procedure. Two patients required reoperation for hardware revision and 1 developed nonunion requiring revision of the bone graft. Ultimately, 7 patients demonstrated osseous fusion on CT scans and 1 had a stable fibrous union. CONCLUSIONS These findings demonstrate that a unilateral OC fixation is effective for the treatment of OC instability in children with CVAs of the CCJ in whom bilateral screw placement fails or is not feasible.

Association between structural rib autograft and the rate of arthrodesis in children undergoing occiput-C2 instrumentation and fusion.

OBJECTIVE: The purpose of this study was to identify factors associated with fusion success among pediatric patients undergoing occiput-C2 rigid instrumentation and fusion. METHODS: The Pediatric Spine Study Group registry was queried to identify patients ≤ 21 years of age who underwent occiput-C2 posterior spinal rigid instrumentation and fusion and had a 2-year minimum clinical and radiographic (postoperative lateral cervical radiograph or CT scan) follow-up. Fusion failure was defined clinically if a patient underwent hardware revision surgery > 30 days after the index procedure or radiographically by the presence of hardware failure or screw haloing on the most recent follow-up imaging study. Univariate comparisons and multivariable logistic regression analyses were subsequently performed. RESULTS: Seventy-six patients met inclusion criteria. The median age at surgery was 9 years (range 1.5-17.2 years), and 51% of the cohort was male. Overall, 75% of patients had syndromic (n = 41) or congenital (n = 15) etiologies, with the most frequent diagnoses of Down syndrome (28%), Chiari malformation (13%), and Klippel-Feil syndrome (12%). Data were available to determine if there was a fusion failure in 97% (74/76) of patients. Overall, 38% (28/74) of patients had fusion failure (95% CI 27%-50%). Univariate analysis demonstrated that use of a rigid cervical collar postoperatively (p = 0.04) and structural rib autograft (p = 0.02) were associated with successful fusion. Multivariable logistic regression analysis determined that patients who had rib autograft used in surgery had a 73% decrease in the odds of fusion failure (OR 0.27, 95% CI 0.09-0.82; p = 0.02). Age, etiology including Down syndrome, instrumentation type, unilateral instrumentation, use of recombinant human bone morphogenetic protein, and other variables did not influence the risk for fusion failure. CONCLUSIONS: In this multicenter, multidisciplinary, international registry of children undergoing occiput-C2 instrumentation and fusion, fusion failure was seen in 38% of patients, a higher rate than previously reported in the literature. The authors' data suggest that postoperative immobilization in a rigid cervical collar may be beneficial, and the use of structural rib autograft should be considered, as rib autograft was associated with a 75% higher chance of successful fusion.

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.

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.

Building consensus: development of a Best Practice Guideline (BPG) for surgical site infection (SSI) prevention in high-risk pediatric spine surgery.

BACKGROUND: Perioperative surgical site infection (SSI) after pediatric spine fusion is a recognized complication with rates between 0.5% and 1.6% in adolescent idiopathic scoliosis and up to 22% in "high risk" patients. Significant variation in the approach to infection prophylaxis has been well documented. The purpose of this initiative is to develop a consensus-based "Best Practice" Guideline (BPG), informed by both the available evidence in the literature and expert opinion, for high-risk pediatric patients undergoing spine fusion. For the purpose of this effort, high risk was defined as anything other than a primary fusion in a patient with idiopathic scoliosis without significant comorbidities. The ultimate goal of this initiative is to decrease the wide variability in SSI prevention strategies in this area, ultimately leading to improved patient outcomes and reduced health care costs. METHODS: An expert panel composed of 20 pediatric spine surgeons and 3 infectious disease specialists from North America, selected for their extensive experience in the field of pediatric spine surgery, was developed. Using the Delphi process and iterative rounds using a nominal group technique, participants in this panel were as follows: (1) surveyed for current practices; (2) presented with a detailed systematic review of the relevant literature; (3) given the opportunity to voice opinion collectively; and (4) asked to vote regarding preferences privately. Round 1 was conducted using an electronic survey. Initial results were compiled and discussed face-to-face. Round 2 was conducted using the Audience Response System, allowing participants to vote for (strongly support or support) or against inclusion of each intervention. Agreement >80% was considered consensus. Interventions without consensus were discussed and revised, if feasible. Repeat voting for consensus was performed. RESULTS: Consensus was reached to support 14 SSI prevention strategies and all participants agreed to implement the BPG in their practices. All agreed to participate in further studies assessing implementation and effectiveness of the BPG. The final consensus driven BPG for high-risk pediatric spine surgery patients includes: (1) patients should have a chlorhexidine skin wash the night before surgery; (2) patients should have preoperative urine cultures obtained; (3) patients should receive a preoperative Patient Education Sheet; (4) patients should have a preoperative nutritional assessment; (5) if removing hair, clipping is preferred to shaving; (6) patients should receive perioperative intravenous cefazolin; (7) patients should receive perioperative intravenous prophylaxis for gram-negative bacilli; (8) adherence to perioperative antimicrobial regimens should be monitored; (9) operating room access should be limited during scoliosis surgery (whenever practical); (10) UV lights need NOT be used in the operating room; (11) patients should have intraoperative wound irrigation; (12) vancomycin powder should be used in the bone graft and/or the surgical site; (13) impervious dressings are preferred postoperatively; (14) postoperative dressing changes should be minimized before discharge to the extent possible. CONCLUSIONS: In conclusion, we present a consensus-based BPG consisting of 14 recommendations for the prevention of SSIs after spine surgery in high-risk pediatric patients. This can serve as a tool to reduce the variability in practice in this area and help guide research priorities in the future. Pending such data, it is the unsubstantiated opinion of the authors of the current paper that adherence to recommendations in the BPG will not only decrease variability in practice but also result in fewer SSI in high-risk children undergoing spinal fusion. LEVEL OF EVIDENCE: Not applicable.