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

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.

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.

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

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

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.

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. A multidisciplinary, retrospective cohort study from seven children’s hospitals was performed including asymptomatic children with MMC

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.

The American College of Surgeons and the American Pediatric Surgical Association collaborate to provide pediatric hospitals with multispeciality surgical outcomes data through the Pediatric National Surgical Quality Improvement Program (NSQIP Peds). The authors used this national multicenter database to describe 30-day outcomes from craniosynostosis surgery and identify associations with perioperative events and blood transfusion.Data from NSQIP Peds were used to describe children undergoing craniosynostosis surgery. The authors examined statistical association of clinical risk factors with the defined end point outcomes of perioperative complications and blood transfusion.Five hundred seventy-two surgeries were included. By Common Procedural Terminology codes, 93 identified as single suture synostosis, the remainder as multiple or unknown suture involvement. Location of the affected suture is not captured. Mean surgical time was 196.84 minutes (SD 113.46). Mean length of stay was 4.22 days (SD 5.04). Sixty-seven percent of patients received blood transfusions. 3.15% were other perioperative occurrences, including infection, wound disruption, unplanned reintubation, stroke/hemorrhage, cardiac arrest, seizures, thromboembolism. 2.8% were readmitted; 2.45% underwent reoperation within 30 days. Duration of surgery and length of hospital stay significantly differed in the presence of blood transfusion versus none. On multivariate analysis, duration from anesthesia start to surgery start, duration from surgery end to anesthesia end, and duration of operation were risk factors for blood transfusion.Pediatric NSQIP gives a national overview of 30-day outcome metrics in craniosynostosis surgery. Perioperative adverse event rate was 3.15%. Duration of surgery and duration of anesthesia were significantly associated with blood transfusion. The authors identified opportunities for pediatric NSQIP database improvement.

Although the incidence of myelomeningocele (MMC) has declined over the past decades with folic acid supplementation and prenatal screening, neural tube defects remain the most common birth defect in the USA. A majority of affected neonates require surgical repair. To characterize US trends in the epidemiology and hospital utilization of MMC repair over the past decade, we analyzed a nationally representative database. We queried the Healthcare Cost and Utilization Project (HCUP) Kid’s Inpatient Database (KID) for all discharges with procedure code for MMC repair for the years 2000, 2003, 2006, and 2009. The cohorts from these time points were compared for their demographic and in-hospital variables. Results are reported as estimated frequencies and means with 95 % confidence intervals (CI). Sex, race, insurance status, family income level, and mortality of affected infants have not changed significantly over the decade. A majority of neonatal MMC repairs occur in larger hospital bed size and more specialized children’s hospital centers. Of patients, 52.3 to 60 % receive VPS placement during the same admission as the primary MMC repair. Total hospital costs for the MMC hospitalizations have remained relatively stable from 42,843 dollars in 2003 to 46,749 dollars in 2009 (adjusted to 2009 dollars). Demographics of children having MMC repair have not changed significantly over the past decade, while these surgeries have become more concentrated in pediatric-specialized centers. There appears to be a plateau in public health and access advances with relatively stable cost of MMC hospital care.

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 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.

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.

Intrathecal baclofen has been suggested as an effective and safe treatment for intractable spasticity and dystonia. Techniques of lumbar and intraventricular catheter placement have been previously described. The purpose of this study was to describe a technique to implant catheters for intrathecal baclofen infusion through C1–2 puncture.Four of 5 consecutively treated patients underwent successful placement of catheters for intrathecal baclofen. There were no instances of infection, CSF leak, or catheter migration seen during a follow-up period of at least 6 months; furthermore, there were no occurrences of vertebral artery or spinal cord injury. All patients had an effective stabilization or reduction of their upper-extremity, lower-extremity, or trunk tone. There were no cases of worsening hypertonia.The authors’ preliminary experience with C1–2 puncture for placement of the intrathecal baclofen catheter seems to indicate that this is a safe and efficacious technique. Lessons learned from the failed attempt at C1–2 puncture will be delineated.

STUDY DESIGN Multicenter retrospective cohort study with multivariate analysis. OBJECTIVE To determine factors predictive of posterior atlantoaxial fusion failure in pediatric patients. SUMMARY OF BACKGROUND DATA Fusion rates for pediatric posterior atlantoaxial arthrodesis have been reported to be high in single-center studies; however, factors predictive of surgical non-union have not been identified by a multicenter study. METHODS Clinical and surgical details for all patients who underwent posterior atlantoaxial fusion at seven pediatric spine centers from 1995 to 2014 were retrospectively recorded. The primary outcome was surgical failure, defined as either instrumentation failure or fusion failure seen on either plain x-ray or computed tomography scan. Multiple logistic regression analysis was undertaken to identify clinical and technical factors predictive of surgical failure. RESULTS One hundred thirty-one patients met the inclusion criteria and were included in the analysis. Successful fusion was seen in 117 (89%) of the patients. Of the 14 (11%) patients with failed fusion, the cause was instrumentation failure in 3 patients (2%) and graft failure in 11 (8%). Multivariate analysis identified Down syndrome as the single factor predictive of fusion failure (odds ratio 14.6, 95% confidence interval [3.7-64.0]). CONCLUSION This retrospective analysis of a multicenter cohort demonstrates that although posterior pediatric atlantoaxial fusion success rates are generally high, Down syndrome is a risk factor that significantly predicts the possibility of surgical failure. LEVEL OF EVIDENCE 3.

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.

Cross-sectional study using data from the Health Care Cost and Utilization Project Kids' Inpatient Database.Blood loss during spinal fusion surgery may lead to the need for transfusion. Preoperative identification of patient-related, procedure-related, or hospital-related risk factors for blood transfusion would allow for implementation of interventions designed to control excessive bleeding.Several studies have analyzed predictors associated with transfusion in spinal fusion. Identified predictors include age, female sex, anemia, comorbidities, number of fusion levels, osteotomy, and greater hospital volume. There have been few studies examining these predictors in children undergoing spinal fusion.Using Kids' Inpatient Database data, univariate and multivariate logistic regression was used to calculate unadjusted and adjusted odds ratios (aOR). P values of less than 0.05 were considered statistically significant.We identified 9538 pediatric hospitalizations (patients21 yr) with spinal fusion in 2009. Overall, 25.1% were associated with blood transfusion. The following factors were associated with transfusions: female sex (aOR 1.14, P = 0.023), black race (aOR 1.35, P = 0.005), length of hospital stay (aOR 1.03, P0.001), anterior approach/lumbar segment (aOR 2.11, P = 0.011) and posterior approach/lumbar segment (aOR 2.75, P0.001) compared with anterior approach/cervical segment, midlength fusion (aOR 1.71, P0.001), and long length fusion (aOR 2.85, P0.001) compared with short length. Higher transfusion rates were observed in patients with complications of fever and hematoma but not wound infection.This study showed significant patient-, procedure-, and hospital-related predictors of allogeneic and autologous blood transfusion in spinal fusion in the pediatric age group. Higher health care resource utilization of length of stay and additional procedures are directed toward care of this transfused subgroup. Therapies to reduce blood loss and transfusion requirement are necessary for this pediatric population.4.

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.

This study aimed to determine if partial removal of the occipital condyle provides a significant increase in visibility and "angle of attack" for treating lesions with extension ventral to the brainstem in children using CT morphometric data. Morphometric analysis was performed in 199 children using CT scans. Angle of attack was measured for both the paracondylar and transcondylar far lateral approach. Statistical analysis was performed using paired or unpaired Student's t-tests (p

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.

We present a small series consisting of eight children with unilateral facet injury of the cervical spine treated surgically. A retrospective review was performed. Injury data, radiographs, surgical data, and outcomes (Neck Disability Index (NDI), Short Form 36 (SF-36), and Visual Analog Scale for Neck Pain (VAS-NP)) were collected from seven patients. A literature review was performed for one additional case. Motor vehicle accidents (62 %, n = 5) and falls (38 %, n = 3) accounted for all injuries. The C6–7 level accounted for most of the injuries (37.5 %, n = 3). The mean NDI score with at least 3 months follow-up was 5.3 (n = 6, range, 1–12; standard deviation, 4.5), corresponding to mild disability. Of the norm-based SF-36 scale scores available (n = 6), the mean physical functioning (PF), role-physical (RP), and role-emotional (RE) scores were significantly less than the adult, age 18–24, norm-based means, with a mean difference of −6.4, −9.13, and −11.3, respectively (p value = 0.03, 0.001, and 0.01, respectively). The mean general health (GH) and vitality (VT) scores, however, were significantly greater than the adult, age 18–24, norm-based mean, with a mean difference of 7.82 and 10.3 (p = 0.04 and 0.02, respectively). VAS-NP showed a return to the “no pain” level at 3 months or more follow-up in all patients. We suggest that surgical treatment of these injuries in the pediatric age group may lead to satisfactory clinical and radiographic outcomes, but HRQoL analysis suggests that patients remain physically and emotionally disabled to some degree after surgery.

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.

It is common to evaluate children who have sustained minor head trauma with computed tomography (CT) of the head. Scalp swelling, in particular, has been associated with intracranial injury. A subset of patients, however, present in delayed fashion, often days after the head trauma, as soft tissue edema progresses and their caregiver notices scalp swelling. We explore the value of further workup in this setting.We conducted a retrospective review of a prospectively collected cohort of children ≤24 months of age presenting to the Texas Children's Hospital with scalp swelling more than 24 h following a head trauma. Cases were collected over a 2-year study period from June 1, 2014 to May 31, 2016.Seventy-six patients comprising 78 patient encounters were included in our study. The mean age at presentation was 8.8 months (range 3 days-24 months). All patients had noncontrast CT of the head as part of their evaluation by emergency medicine, as well as screening for nonaccidental trauma (NAT) by the Child Protection Team. The most common finding on CT head was a linear/nondisplaced skull fracture (SF) with associated extra-axial hemorrhage (epidural or subdural hematoma), which was found in 31/78 patient encounters (40%). Of all 78 patient encounters, 43 patients (55%) were discharged from the emergency room (ER), 17 patients (22%) were admitted for neurologic monitoring, and 18 patients (23%) were admitted solely to allow further NAT evaluation. Of those patients admitted, none experienced a neurologic decline and all had nonfocal neurologic exams on discharge. No patient returned to the ER in delayed fashion for a neurologic decline. Of all the patient encounters, no patient required surgery.Pediatric patients ≤24 months of age presenting to the ER in delayed fashion with scalp swelling after minor head trauma-who were otherwise nonfocal on examination-did not require surgical intervention and did not experience any neurologic decline. Further radiographic investigation did not alter neurosurgical management in these patients; however, it should be noted that workup for child abuse and social care may have been influenced by CT findings, suggesting the need for the future development of a clinical decision-making tool to help safely avoid CT imaging in this setting.

Neurofibrosarcoma is rare in the pediatric age group. A malignant tumor of the sacrum presents significant challenges, especially if the goals are to resect with wide and clean surgical margins and to achieve acceptable functional outcomes. The authors report a case of this rare tumor affecting the sacrum and sacral nerve roots of a 7-year-old girl and review the role of total hemisacrectomy sparing the contralateral sacral nerve roots and lumbopelvic reconstruction in the treatment of this disease. This patient is, to the best of the authors' knowledge, the youngest to be treated in this manner.

Introduction Resection of inferiorly located posterior mediastinal tumors can be complicated by their proximity to the artery of Adamkiewicz (AKA). Although uncommon, intraoperative injury to the AKA may result in paraparesis or paralysis secondary to spinal cord ischemia. The use of preoperative spinal angiography may serve as a useful adjunct to the surgeon in guiding extent of resection of the tumor to avoid injury to this critical artery. Methods After IRB approval (H-31712), three patients, from 2008 to 2011, with lower posterior mediastinal tumors were identified. Their charts were reviewed for information concerning preoperative imaging, operative details, and postoperative neurologic complications. The literature regarding imaging of the AKA, cases of injury in pediatric patients, and recommendations for treatment after its injury were reviewed. Results One patient, who did not have preoperative spinal angiography, developed transient paresis lasting 6weeks after posterior mediastinal tumor resection. Two patients underwent preoperative spinal angiography with successful localization of the AKA. In both cases, the patients subsequently underwent posterior mediastinal tumor resection without injury to the artery and without postoperative neurologic sequelae. Conclusions Preoperative spinal angiography may serve as a useful adjunct in the evaluation of children with inferior posterior mediastinal tumors in order to delineate the relationship of the artery of Adamkiewicz to the tumor for the purpose of guiding surgical resection.

The authors describe rare cases of congenital hypoplasia of the L-5 pedicles and the congenital absence of the left S-1 pedicle in 2 young girls, respectively, including the presentation, diagnosis, and treatment. Moreover, they review the literature on this clinical entity. The patients presented with intractable chronic low-back pain. Plain radiographs and 2D CT scanning revealed the presence of the aforementioned anomalies. Degenerative changes to adjacent level and contralateral facet joints were thought to be the result of overload and instability and seemed to have led to spondylolisthesis, micromotion at L-5 and S-1, and subsequent low-back pain. The pediatric patients were treated with posterior instrumented fusion with good functional outcomes at a minimum 3-month follow-up. To the best of the authors' knowledge, these are the first reports of intractable low-back pain and spondylolisthesis accompanied by hypoplastic-aplastic pedicles at the lumbosacral junction in children.

Cerebellar glioblastomas in children are rare. As a result, an optimal treatment strategy has not yet been identified. A review of the characteristics of the disease as well as the effectiveness of various therapeutic modalities would help in optimizing the treatment paradigm. We performed a detailed clinical, radiographic, and pathologic retrospective review of five patients (three boys and two girls, average age at presentation 7.2 years (range, 3–14 years)) and surveyed the literature for an additional 55 cases. Computed tomography and magnetic resonance imaging usually revealed a large lesion with minimal edema, heterogeneous contrast enhancement, and a discrete border. Subtotal tumor resection was performed in two patients and gross total resection in three patients. Immunostaining of the tumor cells with antisera to glial fibrillary acidic protein and vimentin was variably positive. Adjuvant therapy included local radiation and chemotherapy in all followed patients. Tumor recurrence was seen in two patients. Patients were followed from 2 months to 3.5 years (mean, 12 months). Two patients were dead at last follow-up with a mean survival of 9.5 months. The prognosis for pediatric patients with cerebellar glioblastomas is dismal, even when compared to adult counterparts or other malignant posterior fossa tumors in children. Cerebellar glioblastomas have a tendency to recur and disseminate despite treatment with surgery, chemotherapy, and radiation. The poor outcomes seen with this tumor suggest that the optimal treatment strategy has yet to be elucidated and much work needs to be done.

Risk factors for blunt cerebrovascular injury (BCVI) may differ between children and adults, suggesting that children at low risk for BCVI after trauma receive unnecessary computed tomography angiography (CTA) and high-dose radiation. We previously developed a score for predicting pediatric BCVI based on retrospective cohort analysis. Our objective is to externally validate this prediction score with a retrospective multi-institutional cohort. We included patients who underwent CTA for traumatic cranial injury at four pediatric Level I trauma centers. Each patient in the validation cohort was scored using the “Utah Score” and classified as high or low risk. Before analysis, we defined a misclassification rate

OBJECTIVE Due to improved nutrition and early detection, myelomeningocele repair is a relatively uncommon procedure. Although previous studies have reviewed surgical trends and predictors of outcomes, they have relied largely on single-hospital experiences or on databases centered on hospital admission data. Here, the authors report 30-day outcomes of pediatric patients undergoing postnatal myelomeningocele repair from a national prospective surgical outcomes database. They sought to investigate the association between preoperative and intraoperative factors on the occurrence of 30-day complications, readmissions, and unplanned return to operating room events. METHODS The 2013 American College of Surgeons National Surgical Quality Improvement Program Pediatric database (NSQIP-P) was queried for all patients undergoing postnatal myelomeningocele repair. Patients were subdivided on the basis of the size of the repair (< 5 cm vs > 5 cm). Preoperative variables, intraoperative characteristics, and postoperative 30-day events were tabulated from prospectively collected data. Three separate outcomes for complication, unplanned readmission, and return to the operating room were analyzed using univariate and multivariate logistic regression. Rates of associated CSF diversion operations and their timing were also analyzed. RESULTS A total of 114 patients were included; 54 had myelomeningocele repair for a defect size smaller than 5 cm, and 60 had repair for a defect size larger than 5 cm. CSF shunts were placed concurrently in 8% of the cases. There were 42 NSQIP-defined complications in 31 patients (27%); these included wound complications and infections, in addition to others. Postoperative wound complications were the most common and occurred in 27 patients (24%). Forty patients (35%) had at least one subsequent surgery within 30 days. Twenty-four patients (21%) returned to the operating room for initial shunt placement. Unplanned readmission occurred in 11% of cases. Both complication and return to operating room outcomes were statistically associated with age at repair. CONCLUSIONS The NSQIP-P allows examination of 30-day perioperative outcomes from a national prospectively collected database. In this cohort, over one-quarter of patients undergoing postnatal myelomeningocele repair experienced a complication within 30 days. The complication rate was significantly higher in patients who had surgical repair within the first 24 hours of birth than in patients who had surgery after the 1st day of life. The authors also highlight limitations of investigating myelomeningocele repair using NSQIP-P and advocate the importance of disease-specific data collection.

OBJECTIVE The Thoracolumbar Injury Classification and Severity Score (TLICS) system was developed to streamline injury assessment and guide surgical decision making. To the best of the authors' knowledge, external validation in the pediatric age group has not been undertaken prior to this report. METHODS This study evaluated the use of the TLICS in a large retrospective series of children and adolescents treated at 4 pediatric medical centers (Texas Children's Hospital, Children's Healthcare of Atlanta, Riley Children's Hospital, and Doernbecher Children's Hospital). A total of 147 patients treated for traumatic thoracic or lumbar spine trauma between February 1, 2002, and September 1, 2015, were included in this study. Clinical and radiographic data were evaluated. Injuries were classified using American Spinal Injury Association (ASIA) status, Denis classification, and TLICS. RESULTS A total of 102 patients (69%) were treated conservatively, and 45 patients (31%) were treated surgically. All patients but one in the conservative group were classified as ASIA E. In this group, 86/102 patients (84%) had Denis type compression injuries. The TLICS in the conservative group ranged from 1 to 10 (mean 1.6). Overall, 93% of patients matched TLICS conservative treatment recommendations (score ≤ 3). No patients crossed over to the surgical group in delayed fashion. In the surgical group, 26/45 (58%) were ASIA E, whereas 19/45 (42%) had neurological deficits (ASIA A, B, C, or D). One of 45 (2%) patients was classified with Denis type compression injuries; 25/45 (56%) were classified with Denis type burst injuries; 14/45 (31%) were classified with Denis type seat belt injuries; and 5/45 (11%) were classified with Denis type fracture-dislocation injuries. The TLICS ranged from 2 to 10 (mean 6.4). Eighty-two percent of patients matched TLICS surgical treatment recommendations (score ≥ 5). No patients crossed over to the conservative management group. Eight patients (8/147, 5%) had a calculated TLICS of 4, which meant they were candidates for surgery or conservative therapy by TLICS criteria. Excluding these patients, the degree of agreement between TLICS and surgeon decision was deemed to be very good (κ = 0.878). CONCLUSIONS The TLICS results and recommendations matched treatment in 96% of conservative group cases. In the surgical group, TLICS recommendations matched treatment in 93% of cases. The TLICS recommendations and surgeon decision making displayed very good concordance. The TLICS appears to be effective in the classification of thoracic and lumbar spine injuries and in guiding treatment in the pediatric age group.

Intracranial cerebral aneurysms in the pediatric population are infrequent, and those occurring in infants less than 1 year old are extremely rare. Of intracranial aneurysms in children, dissecting aneurysms are the most common type seen. While spontaneous dissecting aneurysms usually present with ischemia, hemorrhage can also occur. A retrospective review of our patients revealed that from July 1, 2007 to June 30, 2012, four infants were treated for ruptured distal dissecting intracranial aneurysms at Texas Children’s Hospital. Mycotic aneurysms and collagen vascular disorder were excluded in all four cases. All patients presented in our series presented with subarachnoid hemorrhage, and three had intraventricular hemorrhage. All patients underwent conventional catheter angiography for diagnosis. All patients in this series were managed in the acute or subacute period with surgical or endovascular trapping without distal bypass procedures. All four patients tolerated sacrifice of the parent vessels feeding these distal aneurysms well. We describe the presentation and management of these rare cases and then review the current literature on the management of these dissecting aneurysms in infants.

Background Endoscopic third ventriculostomy (ETV) has become the procedure of choice in the treatment of obstructive hydrocephalus. In certain cases, standard ETV might not be technically possible or may engender significant risk. Objective To present an alternative through the lamina terminalis (LT) by a transventricular, transforaminal approach with flexible neuroendoscopy and to discuss the indications, technique, neuroendoscopic findings, and outcomes. Methods Between 1994 and 2010, all patients who underwent endoscopic LT fenestration as an alternative to ETV were analyzed and prospectively followed up. The decision to perform an LT fenestration was made intraoperatively. Results Twenty-five patients, ranging in age from 7 months to 76 years (mean, 28.1 years), underwent endoscopic LT fenestration. Patients had obstructive hydrocephalus secondary to neurocysticercosis (11 patients), neoplasms (6 patients), congenital aqueductal stenosis (3 patients), and other (5 patients). Thirteen patients (52%) had had at least 1 ventriculoperitoneal shunt that malfunctioned; 6 patients (24%) had undergone a previous endoscopic procedure. Intraoperative findings that led to an LT fenestration were the following: ETV not feasible to perform, basal subarachnoid space not sufficient, or adhesions in the third ventricle. No perioperative complications occurred. The mean follow-up period was 63.76 months. Overall, 19 patients (76%) had resolutions of symptoms, had no evidence of ventriculomegaly, and did not require another procedure. Six (24%) required a ventriculoperitoneal shunt. Conclusion Endoscopic transventricular transforaminal LT fenestration with flexible neuroendoscopy is feasible with a low incidence of complications. It is a good alternative to standard ETV. Adequate intraoperative assessment of ETV success is necessary to identify patients who will benefit.

Study design A retrospective review of coronal spine balance after tethered cord release for children with tight filum terminale. Objective To understand the effects of untethering on coronal spine balance for these patients. Summary of background data In patients with tight filum terminale, the spinal cord is tethered by a thickened filum with a low conus medullaris but without other forms of spinal dysraphism. There have not been studies examining the effects of spinal cord untethering on coronal spinal alignment in children with tight filum terminale. Methods Forty-five consecutive pediatric patients with tight filum terminale who had undergone untethering were evaluated. Their presenting signs and symptoms, pre- and postsurgery imagings, and clinical courses were reviewed for scoliosis progression. Results Twenty-six girls and 19 boys underwent tethered cord release at a mean age of 4.5 years. The prevalence of coronal spinal malalignment, manifesting as scoliosis, before the untethering procedure was 31% (14 of 45). During the follow-up period, nine patients had coronal spinal alignment that worsened>10° (five patients eventually underwent surgical fusion), two patients had spinal alignment that improved, and five patients' curves stabilized after untethering surgery. Therefore, at the end of the follow-up period, 9 of 45 patients (20%) had worsened coronal spinal alignment. In the multivariate analysis, patients who presented with a Cobb angle greater than 35° were most likely to progress (P=0.002, odds ratio=21). There was no operative morbidity or mortality associated with scoliosis surgery. Conclusion A significant number of children with tight filum terminale were found to present with scoliosis. In patients with less severe curves, tethered cord release may halt scoliosis progression.

BACKGROUND: Previous studies using recombinant human bone morphogenetic protein-2 (rhBMP-2) in the adult lumbar spine have shown consistently good results, There have been no pediatric case series. OBJECTIVE: To determine the safety and efficacy of rhBMP-2 use in posterior instrumented fusions of the pediatric population. METHODS: A retrospective review of 19 consecutive pediatric patients who underwent posterior occiptocervical, cervical, thoracic, lumbar, or lumbosacral spine fusion from October 1, 2007, to June 30, 2008, at Texas Children's Hospital was performed. The average age was approximately 12 years old (range, 9 months to 20 years). The minimum follow-up was 17 months (average of 19 months, range: 17-25 months), with computed tomography (CT) evaluation and grading of fusion by an independent radiologist at 3 months after surgery. RESULTS: The average CT grade was 3, indicating bilateral bridging bone. No pseudoarthroses or loss of correction was identified clinically or radiographically at 3 months and latest follow-up. There was one complication of bony overgrowth and restenosis of the spinal canal necessitating reoperation, and two superficial wound infections. There were no deep wound infections. CONCLUSION: Early outcomes using rhBMP-2 in the pediatric population show that it is a safe and efficacious adjunct to posterior spine fusions of the occipitocervical, cervical, thoracic, lumbar, and lumbosacral spine. It dependably results in the development of stable bridging bone at 3 months after surgery with good maintenance of correction and stability in long-term follow-up. Lessons learned from the case of unexpected bony overgrowth are discussed.

Study Design. Technical report. Objective. To describe a minimally invasive surgical approach for the obliteration of a subarachnoid-pleural fistula in a 4-year-old child after resection of an intrathoracic ganglioneuroma. Summary of Background Data. Development of a subarachnoid-pleural fistula has been reported after thoracotomy for lung, chest wall, and spinal tumors, when an iatrogenic meningeal laceration results in establishing communication between the spinal subarachnoid space and the pleural cavity. Methods. Review of a single case in which video-assisted thorascopic surgery (VATS) was used to deposit fibrin glue and to suture a pleural allograft. Literature review was performed to document other options to treat subarachnoid-pleural cerebrospinal fluid (CSF) fistula. Results. At 10 months after VATS repair, the CSF fistula has remained closed. Conclusion. VATS technique should be considered for a safe, efficacious, and durable CSF leak repair and as an alternative to open thoracotomy in the pediatric age group.

Selective downward gaze paralysis has not previously been described as a complication after posterior fossa operations in children. The authors found downgaze palsy to be a transient complication after resection of large pediatric posterior fossa midline tumors reaching the aqueduct of Sylvius. They reviewed the cases of 2 children with large posterior fossa midline tumors who underwent resection via an inferior transventricular approach. They developed a hypothetical scheme to account for downward gaze paralysis based on anatomy and insight gained from experimental studies. The authors describe potential risk factors for developing transient selective downward gaze paralysis with the hope of making more pediatric neurosurgeons aware of this complication following removal of lesions around the mesencephalic periaqueductal gray matter. Recognition and understanding of downward gaze palsy after posterior fossa surgery should improve preoperative counseling and promote postoperative family coping.

Objective The management of upper cervical spinal instability in children continues to represent a technical challenge. Traditionally, a number of wiring techniques followed by halo orthosis have been applied; however, they have been associated with a high rate of nonunion and poor tolerance for the halo. Alternatively, C1-C2 transarticular screws and C2 pars/pedicle screws allow more rigid fixation, but they are technically demanding and associated with vertebral artery injuries. Recently, C2 translaminar screws have been added to the armamentarium of the pediatric spine surgeon as a technically simple and biomechanically efficient method of fixation. However, subaxial translaminar screws have not been described in the pediatric population. We describe our experience with axial and subaxial translaminar screws in 7 pediatric patients. Methods Seven pediatric patients with the diagnosis of upper cervical spinal instability required surgical fixation (age, 19 months-14 years; sex, 4 boys and 3 girls; follow-up, 4-21 months; etiology, trauma [3 patients], os odontoideum/os terminale [2 patients], hypoplastic dens [2 patients]). All patients underwent axial and/or subaxial translaminar screw insertion. Iliac crest bone graft was used for fusion in 4 patients; bone morphogenic protein and cancellous morselized allograft was used for fusion in 3 patients. A rigid cervical collar was applied for 12 weeks postoperatively in all cases. No intraoperative image guidance was used for insertion of the translaminar screws. Results All patients had a postoperative computed tomographic scan. Two patients underwent placement of bilateral crossing C2 translaminar screws. Two patients had subaxial translaminar screw placement at C3 and the upper thoracic spine, respectively. Hybrid constructs (a C2 translaminar screw combined with a C2 pars screw) were incorporated in 3 patients. No patients were found to have a breach of the ventral laminar cortex. All patients achieved solid fusion. One patient had a perioperative complication: prolonged dysphagia probably related to C1 lateral mass screw insertion rather than C2 translaminar screw placement. Conclusion To our knowledge, this report represents the only series of pediatric patients treated with axial and subaxial translaminar screws. This series shows that axial and subaxial translaminar screw fixation is a viable option for upper cervical spinal fusion in children. The technique is safe and results in adequate fixation with high fusion rates and minimal complications.

In the pediatric population, a strong correlation between multilevel laminectomy and postlaminectomy spinal deformities, predominantly kyphosis, has been demonstrated. This has been observed mainly in the cervical and thoracic spine. Therefore, laminoplasty was proposed as an alternative to laminectomy with a reduced incidence of spinal deformity and need for stabilization and fusion. Moreover, to the best of our knowledge, postlaminoplasty kyphosis of the thoracic spine has not been previously described in the literature. We report the case of a pediatric patient who rapidly developed severe thoracic kyphosis 3 months after resection of a symptomatic extradural spinal arachnoid cyst and multilevel en bloc thoracic laminoplasty and review the relevant literature.

Object Currently, no diagnostic or treatment standards exist for extracranial carotid artery dissection (CAD) in children after trauma. The purpose of this study was to review and describe the characteristics, diagnosis, and treatment of this rather uncommon sequelae of pediatric trauma. Methods A systematic review of the literature was performed to examine the pertinent studies of traumatic extracranial carotid artery (CA) injuries in children. Results No randomized trials were identified; however, 19 case reports or small case series consisting of 34 pediatric patients were found in the literature. The diagnosis of CAD was made in 33 of 34 patients only after the onset of ischemic symptomatology. Twenty-four of 34 patients underwent cerebral angiography to confirm diagnosis; MR angiography affirmed the diagnosis in 6 of 34 patients. There was little published experience with CA ultrasonography or CT angiography for diagnosis. Thirty of 34 patients were treated with medical therapy or observation; 2 of 4 patients treated with observation alone died. There was little experience with open surgical treatment of CAD in the pediatric population, and there were no studies on the endovascular treatment of traumatic CAD in children. The literature does not support anticoagulation therapy over antiplatelet therapy. Conclusions As a result of this review of the literature, the authors propose the algorithms for the evaluation and treatment of traumatic extracranial CADs in children. These recommendations include utilizing MR angiography as a screening tool in cases in which the clinical suspicion of CAD is high, using conventional cerebral angiography to confirm the diagnosis, implementing antiplatelet therapy as initial medical management, and reserving endovascular stenting in cases of failed medical treatment.

Background context Several relatively new screw techniques have been described that rigidly capture the posterior elements of C2. The previously described procedures of axis fixation are technically demanding and place the vertebral artery at some risk. A novel and less technically demanding technique of obtaining C-2 translaminar screws has been recently described. Although the risk of vertebral artery injury has been essentially eliminated, the authors recognize that neurologic injury from breakthrough of the inner cortex of the lamina by the drill or screw is still a possibility. Purpose Describe and illustrate a modified C2 translaminar technique and review the results of patients who have undergone the surgery. The current modification of the C2 translaminar screw technique was designed to reduce the risk of inadvertent screw placement within the spinal canal. Study design/setting A techniques paper combined with a retrospective clinical review of patients undergoing the surgery. Patient sample Patients undergoing posterior instrumented fusion surgery of the cervical spine, which incorporates C2 posterior elements using the translaminar technique. Outcome measures Radiographic analysis of the fusion construct incorporating the C2 translaminar screws. Methods We have modified the previously described technique of C-2 translaminar screw placement with the addition of “exit” cortical windows to assure bicortical, intralaminar screw placement. Results The results of the first six patients with an average follow-up of 12 months demonstrated this method to be safe and effective in fixating the axis. Conclusions We have made a simple modification of Wright's elegant technique with the addition of “exit” windows at the facet-laminar junctions. This gives us the assurance that the C2 screw has not entered the spinal canal by directly visualizing the tip of the screw exiting the outer cortices of the lamina before leaving the operating room.

The authors describe the novel use of C-1 lateral mass screws in four children 8 years of age or younger, in whom occipitocervical or atlantoaxial fusion was performed for trauma or os odontoideum. The authors retrospectively reviewed the demographics and procedural data of four children, ranging in age from 2 to 8 years, who required and underwent surgical fixation. Although C1-2 screw/rod constructs involving individual C-1 lateral mass screws and C-2 pars interarticularis or pedicle screws have been widely applied in adults, only C1-2 transarticular screw fixation has been reported in children less than 8 years of age. This report demonstrates the successful results of rigid occipitocervical and atlantoaxial fusion in four children in whom C-1 lateral mass screws were placed as part of a screw/rod construct. There was one instance of a vertebral artery injury, and the lessons learned from this complication are discussed.