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Background Previous studies of cerebrospinal fluid (CSF) shunt infection treatment have been limited in size and unable to compare patient and treatment characteristics by infecting organism. Our objective was to describe variation in patient and treatment characteristics for children with first CSF shunt infection, stratified by infecting organism subgroups outlined in the 2017 Infectious Disease Society of America’s (IDSA) guidelines. Methods We studied a prospective cohort of children Results There were 145 children whose infections were diagnosed by CSF culture and addressed by IDSA guidelines, including 47 with Staphylococcus aureus, 52 with coagulase-negative Staphylococcus, 37 with Gram-negative bacilli, and 9 with Propionibacterium acnes. No differences in many patient and treatment characteristics were seen between infecting organism subgroups, including age at initial shunt, gender, race, insurance, indication for shunt, gastrostomy, tracheostomy, ultrasound, and/or endoscope use at all surgeries before infection, or numbers of revisions before infection. A larger proportion of infections were caused by Gram-negative bacilli when antibiotic-impregnated catheters were used at initial shunt placement (12 of 23, 52%) and/or subsequent revisions (11 of 23, 48%) compared with all other infections (9 of 68 [13%] and 13 of 68 [19%], respectively). No differences in reinfection were observed between infecting organism subgroups. Conclusions The organism profile encountered at infection differs when antibiotic-impregnated catheters are used, with a higher proportion of Gram-negative bacilli. This warrants further investigation given increasing adoption of antibiotic-impregnated catheters.

OBJECTIVECSF shunt infection requires both surgical and antibiotic treatment. Surgical treatment includes either total shunt removal with external ventricular drain (EVD) placement followed by new shunt insertion, or distal shunt externalization followed by new shunt insertion once the CSF is sterile. Antibiotic treatment includes the administration of intravenous antibiotics. The Hydrocephalus Clinical Research Network (HCRN) registry provides a unique opportunity to understand reinfection following treatment for CSF shunt infection. This study examines the association of surgical and antibiotic decisions in the treatment of first CSF shunt infection with reinfection.METHODSA prospective cohort study of children undergoing treatment for first CSF infection at 7 HCRN hospitals from April 2008 to December 2012 was performed. The HCRN consensus definition was used to define CSF shunt infection and reinfection. The key surgical predictor variable was surgical approach to treatment for CSF shunt infection, and the key antibiotic treatment predictor variable was intravenous antibiotic selection and duration. Cox proportional hazards models were constructed to address the time-varying nature of the characteristics associated with shunt surgeries.RESULTSOf 233 children in the HCRN registry with an initial CSF shunt infection during the study period, 38 patients (16%) developed reinfection over a median time of 44 days (interquartile range [IQR] 19–437). The majority of initial CSF shunt infections were treated with total shunt removal and EVD placement (175 patients; 75%). The median time between infection surgeries was 15 days (IQR 10–22). For the subset of 172 infections diagnosed by CSF culture, the mean ± SD duration of antibiotic treatment was 18.7 ± 12.8 days. In all Cox proportional hazards models, neither surgical approach to infection treatment nor overall intravenous antibiotic duration was independently associated with reinfection. The only treatment decision independently associated with decreased infection risk was the use of rifampin. While this finding did not achieve statistical significance, in all 5 Cox proportional hazards models both surgical approach (other than total shunt removal at initial CSF shunt infection) and nonventriculoperitoneal shunt location were consistently associated with a higher hazard of reinfection, while the use of ultrasound was consistently associated with a lower hazard of reinfection.CONCLUSIONSNeither surgical approach to treatment nor antibiotic duration was associated with reinfection risk. While these findings did not achieve statistical significance, surgical approach other than total removal at initial CSF shunt infection was consistently associated with a higher hazard of reinfection in this study and suggests the feasibility of controlling and standardizing the surgical approach (shunt removal with EVD placement). Considerably more variation and equipoise exists in the duration and selection of intravenous antibiotic treatment. Further consideration should be given to the use of rifampin in the treatment of CSF shunt infection. High-quality studies of the optimal duration of antibiotic treatment are critical to the creation of evidence-based guidelines for CSF shunt infection treatment.

OBJECTIVEPrevious Hydrocephalus Clinical Research Network (HCRN) retrospective studies have shown a 15% difference in rates of conversion to permanent shunts with the use of ventriculosubgaleal shunts (VSGSs) versus ventricular reservoirs (VRs) as temporization procedures in the treatment of hydrocephalus due to high-grade intraventricular hemorrhage (IVH) of prematurity. Further research in the same study line revealed a strong influence of center-specific decision-making on shunt outcomes. The primary goal of this prospective study was to standardize decision-making across centers to determine true procedural superiority, if any, of VSGS versus VR as a temporization procedure in high-grade IVH of prematurity.METHODSThe HCRN conducted a prospective cohort study across 6 centers with an approximate 1.5- to 3-year accrual period (depending on center) followed by 6 months of follow-up. Infants with premature birth, who weighed less than 1500 g, had Grade 3 or 4 IVH of prematurity, and had more than 72 hours of life expectancy were included in the study. Based on a priori consensus, decisions were standardized regarding the timing of initial surgical treatment, upfront shunt versus temporization procedure (VR or VSGS), and when to convert a VR or VSGS to a permanent shunt. Physical examination assessment and surgical technique were also standardized. The primary outcome was the proportion of infants who underwent conversion to a permanent shunt. The major secondary outcomes of interest included infection and other complication rates.RESULTSOne hundred forty-five premature infants were enrolled and met criteria for analysis. Using the standardized decision rubrics, 28 infants never reached the threshold for treatment, 11 initially received permanent shunts, 4 were initially treated with endoscopic third ventriculostomy (ETV), and 102 underwent a temporization procedure (36 with VSGSs and 66 with VRs). The 2 temporization cohorts were similar in terms of sex, race, IVH grade, head (orbitofrontal) circumference, and ventricular size at temporization. There were statistically significant differences noted between groups in gestational age, birth weight, and bilaterality of clot burden that were controlled for in post hoc analysis. By Kaplan-Meier analysis, the 180-day rates of conversion to permanent shunts were 63.5% for VSGS and 74.0% for VR (p = 0.36, log-rank test). The infection rate for VSGS was 14% (5/36) and for VR was 17% (11/66; p = 0.71). The overall compliance rate with the standardized decision rubrics was noted to be 90% for all surgeons.CONCLUSIONSA standardized protocol was instituted across all centers of the HCRN. Compliance was high. Choice of temporization techniques in premature infants with IVH does not appear to influence rates of conversion to permanent ventricular CSF diversion. Once management decisions and surgical techniques are standardized across HCRN sites, thus minimizing center effect, the observed difference in conversion rates between VSGSs and VRs is mitigated.

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 This study aimed to examine factors associated with adherence to recommended treatment among pediatric patients with positional skull deformity by reviewing a single-institution experience (2007–2014) with the treatment of positional plagiocephaly. METHODS A retrospective chart review was conducted. Risk factors, treatment for positional head shape deformity, and parent-reported adherence were recorded. Univariate and multivariate analyses were used to assess the impact of patient clinical and demographic characteristics on adherence. RESULTS A total of 991 patients under age 12 months were evaluated for positional skull deformity at the Texas Children's Hospital Cranial Deformity Clinic between 2007 and 2014. According to an age- and risk factor–based treatment algorithm, patients were recommended for repositioning, physical therapy, or cranial orthosis therapy or crossover from repositioning/physical therapy into cranial orthosis therapy. The patients' average chronological age at presentation was 6.2 months; 69.3% were male. The majority were white (40.7%) or Hispanic (32.6%); 38.7% had commercial insurance and 37.9% had Medicaid. The most common initial recommended treatment was repositioning or physical therapy; 85.7% of patients were adherent to the initial recommended treatment. Univariate analysis showed differences in adherence rates among subgroups. Children's families with Medicaid were less likely to be adherent to treatment recommendations (adherence rate, 80.2%). Families with commercial insurance were more likely to be adherent to the recommended treatment (89.6%). Multivariate logistic regression confirmed that factors associated with parent-reported adherence to recommended treatment included primary insurance payer, diagnosis (plagiocephaly vs brachycephaly), and the nature of the recommended treatment. Families were less likely to be adherent if they had Medicaid, a child with a diagnosis of brachycephaly, or were initially recommended for cranial orthosis therapy than families with commercial insurance, a child with a diagnosis of plagiocephaly, or an initial recommendation for repositioning or physical therapy. Factors associated with treatment completion included corrected age, insurance, diagnosis, recommended treatment, and distance to provider from patient's residence. Patients with commercial insurance (OR 1.49, 95% CI 1.10–2.02, p = 0.009), those diagnosed with both brachycephaly and plagiocephaly (OR 2.26, 95% CI 1.31–3.90, p = 0.003), those recommended for treatment with cranial orthosis (OR 4.55, 95% CI = 3.24–6.38, p < 0.001), and those living in proximity to the provider (OR 1.40, 95% CI 1.00–1.96, p = 0.047) were more likely to complete treatment. CONCLUSIONS Insurance type, degree of head shape deformity, and types of recommended treatment appear to affect rates of adherence to recommended treatments for positional skull deformation.

OBJECTIVE Accurate placement of ventricular catheters may result in prolonged shunt survival, but the best target for the hole-bearing segment of the catheter has not been rigorously defined. The goal of the study was to define a target within the ventricle with the lowest risk of shunt failure. METHODS Five catheter placement variables (ventricular catheter tip location, ventricular catheter tip environment, relationship to choroid plexus, catheter tip holes within ventricle, and crosses midline) were defined, assessed for interobserver agreement, and evaluated for their effect on shunt survival in univariate and multivariate analyses. De-identified subjects from the Shunt Design Trial, the Endoscopic Shunt Insertion Trial, and a Hydrocephalus Clinical Research Network study on ultrasound-guided catheter placement were combined (n = 858 subjects, all first-time shunt insertions, all patients < 18 years old). The first postoperative brain imaging study was used to determine ventricular catheter placement for each of the catheter placement variables. RESULTS Ventricular catheter tip location, environment, catheter tip holes within the ventricle, and crosses midline all achieved sufficient interobserver agreement (κ > 0.60). In the univariate survival analysis, however, only ventricular catheter tip location was useful in distinguishing a target within the ventricle with a survival advantage (frontal horn; log-rank, p = 0.0015). None of the other catheter placement variables yielded a significant survival advantage unless they were compared with catheter tips completely not in the ventricle. Cox regression analysis was performed, examining ventricular catheter tip location with age, etiology, surgeon, decade of surgery, and catheter entry site (anterior vs posterior). Only age (p < 0.001) and entry site (p = 0.005) were associated with shunt survival; ventricular catheter tip location was not (p = 0.37). Anterior entry site lowered the risk of shunt failure compared with posterior entry site by approximately one-third (HR 0.65, 95% CI 0.51–0.83). CONCLUSIONS This analysis failed to identify an ideal target within the ventricle for the ventricular catheter tip. Unexpectedly, the choice of an anterior versus posterior catheter entry site was more important in determining shunt survival than the location of the ventricular catheter tip within the ventricle. Entry site may represent a modifiable risk factor for shunt failure, but, due to inherent limitations in study design and previous clinical research on entry site, a randomized controlled trial is necessary before treatment recommendations can be made.

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.

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.

To examine the impact of demographic factors, including insurance type, family income, and race/ethnicity, on patient age at the time of surgical intervention for craniosynostosis surgery in the US.The Kids' Inpatient Database was queried for admissions of children younger than 3 years of age undergoing craniosynostosis surgery in 2009. Descriptive data regarding age at surgery for various substrata are reported. Multivariate regression was used to evaluate the effect of patient and hospital characteristics on the age at surgery.Children with private insurance were, on average, 6.8 months of age (95% CI 6.2-7.5) at the time of surgery; children with Medicaid were 9.1 months old (95% CI 8.4-9.8). White children received surgery at mean age of 7.2 months (95% CI 6.5-8.0) and black and Hispanic children at a mean age of 9.1 months (95% CI 8.2-10.1). Multivariate regression analysis found Medicaid insurance (beta coefficient [B]=1.93, P.001), black or Hispanic race/ethnicity (B=1.34, P=.022), and having 2 or more chronic conditions (B=2.86, P.001) to be significant independent predictors of older age at surgery.Public insurance and nonwhite race/Hispanic ethnicity were statistically significant predictors for older age at surgery, adjusted for sex, zip code median family income, year, and hospital factors such as size, type, region, and teaching status. Further research into these disparities is warranted.

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 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.

Unplanned intraoperative extubations (UIEs), rare but high-risk events. Unintentional extubations are used as quality improvement metrics in neonatal and pediatric intensive care units, but intraoperative events have received scant attention in the literature. Complexity of patient positioning and proximity of the operative field to anesthesia make neurosurgical procedures unique. UIEs prolong operative time, increase risk of adverse outcomes, including cardiopulmonary collapse, and potentially require additional procedures. Investigating each event is critical to prevention. We aimed to analyze occurrences of UIEs in the pediatric population.We retrospectively reviewed UIE cases (12/2014-4/2017) in pediatric neurosurgical patients at a metropolitan pediatric Level I trauma center. Data were collected on patient demographics, procedure, operating room events before the event, and patient outcomes.Over 27 months, 5 UIEs in pediatric neurosurgical cases occurred, with an event rate of0.3%. Two occurred in patients1 year old. Two UIEs occurred in patients undergoing surgery for epilepsy. Root cause analysis identified varied etiologies of UIE: 2 were attributed to endotracheal tube securement, 2 were attributed to lighter anesthesia planes in epilepsy cases with limb movement, and 1 occurred while supinating a prone patient. Postoperative outcomes for these patients were no different from routine cases.Findings suggest an inverse correlation between patient age and UIE, with patient manipulation and anesthesia depth as risk factors. Meticulous attention to securing the endotracheal tube and streamlined communication between the surgical and anesthesia teams are critical for the goal of zero UIE occurrences.

OBJECTIVE Following institution of the Back to Sleep Campaign, the incidence of sudden infant death syndrome decreased while the prevalence of positional skull deformation increased dramatically. The management of positional deformity is controversial, and treatment recommendations and outcomes reporting are variable. The authors reviewed their institutional experience (2008–2014) with the treatment of positional plagiocephaly to explore factors associated with measured improvement. METHODS A retrospective chart review was conducted with risk factors and treatment for positional head shape deformity recorded. Univariate and multivariate analyses were used to assess the impact of these variables on the change in measured oblique diagonal difference (ODD) on head shape surface scanning pre- and posttreatment. RESULTS A total of 991 infants aged less than 1 year were evaluated for cranial positional deformity in a dedicated clinical program. The most common deformity was occipital plagiocephaly (69.5%), followed by occipital brachycephaly (18.4%) or a combination of both deformities (12.1%). Recommended treatment included repositioning (RP), physical therapy (PT) if indicated, or orthotic treatment with a customized cranial orthosis (CO) according to an age- and risk factor–dependent algorithm that the authors developed for this clinic. Of the 991 eligible patients, 884 returned for at least 1 follow-up appointment. A total of 552 patients were followed to completion of their treatment and had a full set of records for analysis: these patients had pre- and posttreatment 2D surface scanner evaluations. The average presenting age was 6.2 months (corrected for prematurity for treatment considerations). Of the 991 patients, 543 (54.8%) had RP or PT as first recommended treatment. Of these 543 patients, 137 (25.2%) transitioned to helmet therapy after the condition did not improve over 4–8 weeks. In the remaining cases, RP/PT had already failed before the patients were seen in this program, and the starting treatment recommendation was CO. At the end of treatment, the measured improvements in ODD were 36.7%, 33.5%, and 15.1% for patients receiving CO, RP/PT/CO, and RP/PT, respectively. Univariate analysis showed that sex, race, insurance, diagnosis, sleep position preference, torticollis history, and multiple gestation were not significantly associated with magnitude of ODD change during treatment. On multivariate analysis, corrected age at presentation and type of treatment received were significantly associated with magnitude of ODD change. Orthotic treatment corresponded with the largest ODD change, while the RP/PT group had the least change in ODD. Earlier age at presentation corresponded with larger ODD change. CONCLUSIONS Earlier age at presentation and type of treatment impact the degree of measured deformational head shape correction in positional plagiocephaly. This retrospective study suggests that treatment with a custom CO can result in more improvement in objective measurements of head shape.

Objective The aim of the study was to evaluate the utility of the emergency department observation unit (EDOU) for neurologically intact children with closed head injuries (CHIs) and computed tomography (CT) abnormalities. Methods A retrospective cohort study of children aged 0 to 18 years with acute CHI, abnormal head CT, and a Glasgow Coma Scales score of 14 or higher admitted to the EDOU of a tertiary care children's hospital from 2007 to 2010. Children with multisystem trauma, nonaccidental trauma, and previous neurosurgical or coagulopathic conditions were excluded. Medical records were abstracted for demographic, clinical, and radiographic findings. Poor outcome was defined as death, intensive care unit admission, or medically/surgically treated increased intracranial pressure. Results Two hundred two children were included. Median (range) age was 14 (4 days-16 years) months; 51% were male. The most common CT findings were nondisplaced (136, 67%) or displaced (46, 23%) as well as skull fractures and subdural hematomas (38, 19%); 54 (27%) had less than 1 CT finding. The most common interventions included repeat CT (42, 21%), antiemetics (26, 13%), and pain medication (29, 14%). Eighty-nine percent were discharged in less than 24 hours. Inpatient admission from the EDOU occurred in 6 (3%); all were discharged in less than 3 days. One patient required additional intervention (corticosteroid therapy). She had a subdural hematoma, persistent vomiting, intractable headache, and a nonevolving CT. Conclusions Neurologically intact patients on initial ED evaluation had a very low likelihood of requiring further interventions, irrespective of CT findings. Although prospective evidence is necessary, this supports reliance on clinical findings when evaluating a well-appearing child with an acute CHI.

Object Cerebrospinal fluid shunt ventricular catheters inserted into the frontal horn or trigone are associated with prolonged shunt survival. Developing surgical techniques for accurate catheter insertion could, therefore, be beneficial to patients. This study was conducted to determine if the rate of accurate catheter location with intraoperative ultrasound guidance could exceed 80%. Methods The authors conducted a prospective, multicenter study of children (< 18 years) requiring first-time treatment for hydrocephalus with a ventriculoperitoneal shunt. Using intraoperative ultrasound, surgeons were required to target the frontal horn or trigone for catheter tip placement. An intraoperative ultrasound image was obtained at the time of catheter insertion. Ventricular catheter location, the primary outcome measure, was determined from the first postoperative image. A control group of patients treated by nonultrasound surgeons (conventional surgeons) were enrolled using the same study criteria. Conventional shunt surgeons also agreed to target the frontal horn or trigone for all catheter insertions. Patients were triaged to participating surgeons based on call schedules at each center. A pediatric neuroradiologist blinded to method of insertion, center, and surgeon determined ventricular catheter tip location. Results Eleven surgeons enrolled as ultrasound surgeons and 6 as conventional surgeons. Between February 2009 and February 2010, 121 patients were enrolled at 4 Hydrocephalus Clinical Research Network centers. Experienced ultrasound surgeons (> 15 cases prior to study) operated on 67 patients; conventional surgeons operated on 52 patients. Experienced ultrasound surgeons achieved accurate catheter location in 39 (59%) of 66 patients, 95% CI (46%–71%). Intraoperative ultrasound images were compared with postoperative scans. In 32.7% of cases, the catheter tip moved from an accurate location on the intraoperative ultrasound image to an inaccurate location on the postoperative study. This was the most significant factor affecting accuracy. In comparison, conventional surgeons achieved accurate location in 24 (49.0%) of 49 cases (95% CI [34%–64%]). The shunt survival rate at 1 year was 70.8% in the experienced ultrasound group and 66.9% in the conventional group (p = 0.66). Ultrasound surgeons had more catheters surrounded by CSF (30.8% vs 6.1%, p = 0.0012) and away from the choroid plexus (72.3% vs 58.3%, p = 0.12), and fewer catheters in the brain (3% vs 22.4%, p = 0.0011) and crossing the midline (4.5% vs 34.7%, p < 0.001), but they had a higher proportion of postoperative pseudomeningocele (10.1% vs 3.8%, p = 0.30), wound dehiscence (5.8% vs 0%, p = 0.13), CSF leak (10.1% vs 1.9%, p = 0.14), and shunt infection (11.6% vs 5.8%, p = 0.35). Conclusions Ultrasound-guided shunt insertion as performed in this study was unable to consistently place catheters into the frontal horn or trigone. The technique is safe and achieves outcomes similar to other conventional shunt insertion techniques. Further efforts to improve accurate catheter location should focus on prevention of catheter migration that occurs between intraoperative placement and postoperative imaging. Clinical trial registration no.: NCT01007786 (ClinicalTrials.gov).

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.

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.

OBJECT This study explored antibiotic prophylaxis (AP) in pediatric patients undergoing intrathecal baclofen pump (ITBP) surgery and factors associated with perioperative AP compliance with clinical guidelines. METHODS Data were obtained from the Pediatric Health Information System. The study cohort comprised patients who underwent ITBP surgery within 3 days of admission, between July 1, 2004, and March 31, 2014, with a minimum prior screening period and follow-up of 180 days. Exclusion criteria were prior infection, antibiotic use within 30 days of admission, and/or missing financial data. Chi-square tests and multivariate logistic regressions were used to determine factors associated with compliance with AP guidelines in ITBP surgeries. RESULTS A total of 1,534 patients met the inclusion criteria; 91.5% received AP and 37.6% received dual coverage or more. Overall bundled compliance comprised 2 components: 1) perioperative antibiotic administration and 2) < 24-hour postoperative antibiotic course. The most frequently used antibiotics in surgery were cefazolin (n = 873, 62.2%) and vancomycin (n = 351, 25%). Documented bundled AP compliance rates were 70.2%, 62.0%, 66.0%, and 55.2% in West, South, Midwest, and Northeast regions of the US, respectively. Compared with surgeries in the Northeast, procedures carried out in the West (OR 2.0, 95% C11.4-2.9, p < 0.001), Midwest (OR 1.6, 95% C11.1-2.3, p = 0.007), and South (OR 1.5, 95% C11.1-2.0, p = 0.021) were more likely to have documented AP compliance. Black (OR 0.74, 95% CI 0.55-1.00, p = 0.05) and Hispanic (OR 0.63, 95% CI 0.47-0.86, p = 0.004) patients were less likely to have documented AP compliance in ITBP surgeries than white patients. There were no significant differences in compliance rate by age, sex, type of insurance, and diagnosis. AP process measures were associated with shorter length of stay, lower hospitalization costs, and lower 6-month rates of surgical infection/complication. One of the 2 noncompliance subgroups, missed preoperative antibiotic administration, was correlated with a significantly higher 6-month surgical complication/infection rate (27.03%) compared with bundled compliance (20.00%, p = 0.021). For the other subgroup, prolonged antibiotic use > 24 hours postoperatively, the rate was insignificantly higher (22.00%, p = 0.368). Thus, of direct relevance to practicing clinicians, missed preoperative antibiotics was associated with 48% higher risk of adverse complication/infection outcome in a 6-month time frame. Adjusted hospitalization costs associated with baclofen pump surgery differed significantly (p < 0.001) with respect to perioperative antibiotic practices: 22.83, 29.10, 37.66 (× 1000 USD) for bundled compliance, missed preoperative antibiotics, and prolonged antibiotic administration, respectively. CONCLUSIONS Significant variation in ITBP antibiotic prophylaxis was found. Documented AP compliance was associated with higher value of care, showing favorable clinical and financial outcomes. Of most impact to clinical outcome, missed preoperative antibiotics was significantly associated with higher risk of 6-month surgical complication/infection. Prolonged antibiotic use was associated with significantly higher hospital costs compared with those with overall bundled antibiotic compliance. Future research is warranted to examine factors associated with practice variation and how AP compliance is associated with outcomes and quality, aiming for improving delivery of care to pediatric patients undergoing ITBP procedures.

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.

Object The aim of this study was to determine whether the presence of intracranial pathophysiology on computed tomography (CT) scans obtained within 24 hours of mild traumatic brain injury (MTBI) in children adversely affects neuropsychological outcome during the 1st year postinjury. Methods A prospective longitudinal design was used to examine the neuropsychological outcomes in children (ages 5–15 years) who had been treated for MTBI, which was defined as a loss of consciousness for up to 30 minutes and a lowest Glasgow Coma Scale (GCS) score of 13–15. Exclusion criteria included any preinjury neurological disorder. Outcome assessments were performed within 2 weeks and at 3, 6, and 12 months postinjury. Outcomes were compared between patients with MTBI whose postinjury CT scans revealed complications of brain pathophysiology (32 patients, CMTBI group) and those with MTBI but without complications (48 patients, MTBI group). Results Significant interactions confirmed that the pattern of recovery over 12 months after injury differed depending on the intracranial pathology, presence and severity of injuries to body regions other than the head, preinjury attention-deficit hyperactivity disorder (ADHD), and socioeconomic status. Children in the CMTBI group had significantly poorer episodic memory, slower cognitive processing, diminished recovery in managing cognitive interference, and poorer performance in calculating and reading than patients in the MTBI group. Among the patients with mild or no extracranial injury, visuomotor speed was slower in those in the CMTBI group; and among patients without preinjury ADHD, working memory was worse in those in the CMTBI group. Conclusions Neuropsychological recovery during the 1st year following MTBI is related to the presence of radiographically detectable intracranial pathology. Children with intracranial pathology on acute CT performed more poorly in several cognitive domains when compared with patients whose CT findings were normal or limited to a linear skull fracture. Depending on the presence of preinjury ADHD and concomitant extracranial injury, working memory and visuomotor speed were also diminished in patients whose CT findings revealed complications following MTBI. Computed tomography within 24 hours postinjury appears to be useful for identifying children with an elevated risk for residual neuropsychological changes.

OBJECT Several studies have indicated that the 30-day morbidity and mortality risks are higher among pediatric and adult patients who are admitted on the weekends. This “weekend effect” has been observed among patients admitted with and fora variety of diagnoses and procedures, including myocardial infarction, pulmonary embolism, ruptured abdominal aortic aneurysm, stroke, peptic ulcer disease, and pediatric surgery. In this study, morbidity and mortality outcomes for emergency pediatric neurosurgical procedures carried out on the weekend or after hours are compared with emergency surgical procedures performed during regular weekday business hours. METHODS A retrospective analysis of operative data was conducted. Between December 1, 2011, and August 20, 2014, a total of 710 urgent or emergency neurosurgical procedures were performed at Texas Children’s Hospital in children younger than than 18 years of age. These procedures were then stratified into 3 groups: weekday regular hours, weekday after hours, and weekend hours. By cross-referencing these events with a prospectively collected morbidity and mortality database, the impact of the day and time on complication incidence was examined. Outcome metrics were compared using logistic regression models. RESULTS The weekday regular hours and after-hours (weekday after hours and weekends) surgery groups consisted of 341 and 239 patients and 434 and 276 procedures, respectively. There were no significant differences in the types of cases performed (p = 0.629) or baseline preoperative health status as determined by American Society of Anesthesiologists classifications (p = 0.220) between the 2 cohorts. After multivariate adjustment and regression, children undergoing emergency neurosurgical procedures during weekday after hours or weekends were more likely to experience complications (p = 0.0227). CONCLUSIONS Weekday after-hours and weekend emergency pediatric neurosurgical procedures are associated with significantly increased 30-day morbidity and mortality risk compared with procedures performed during weekday regular hours.

BACKGROUND Pediatric hydrocephalus represents a high health care burden in the United States (US). Surgery is the mainstay of treatment. OBJECTIVE To perform a comparative effectiveness analysis for endoscopic third ventriculostomy (ETV) and cerebrospinal fluid shunt placement in pediatric hydrocephalus patients in the US using a large administrative claims database through the application of propensity scores matching. METHODS The MarketScan® database (Truven Health Analytics, Atlanta, Georgia) 2003 to 2011 was used. Patients 19 yr or younger at first occurrence of ETV or shunt during the study period were included. The study outcome, surgery failure, was defined as further surgical treatment for hydrocephalus subsequent to initial ETV or shunt procedure. Age, etiology of hydrocephalus, and history of shunt were used to create matched samples for the ETV and shunt cohorts. Kaplan-Meier survival curves, stratified log-rank test, and Cox proportional-hazard models were used to analyze samples. RESULTS There were 3231 eligible cases (478 ETV; 2753 shunt). Propensity scores matching produced 455 balanced pairs. For matched samples, 326 of 455 (72%) pairs were concordant, while 129 pairs were discordant in surgery outcomes within 3 mo. Among discordant pairs, ETV patients were more likely to experience surgery failure compared to patients receiving shunt (relative risk = 1.4, P value = .011). Furthermore, patients' age < 1 yr had lower ETV success rates than those with shunt (P value = .009). No similar pattern was found in patients' age ≥ 1 yr. CONCLUSION There was no significant effect on time to failure between patients undergoing ETV and shunt, except in infants' age

OBJECT Low-molecular-weight heparins (LMWHs), mainly enoxaparin, offer several advantages over standard anticoagulation therapies such as unfractionated heparin and warfarin, including predictable pharmacokinetics, minimal monitoring, and subcutaneous administration. The purpose of this study was to determine the safety and efficacy of LMWHs in pediatric neurosurgical patients. METHODS A retrospective study was performed with patients 18 years old or younger who were admitted to the Pediatric Neurosurgery Service at Texas Children's Hospital and treated with LMWH for either therapeutic or prophylactic purposes between March 1, 2011, and December 30, 2013. Demographic and clinical features and outcomes were recorded. RESULTS LMWH was administered for treatment of venous thromboembolic events (VTEs) in 17 children and for prophylaxis in 24 children. Clinical resolution of VTEs occurred in 100% (17 of 17) of patients receiving therapeutic doses of LMWH. No patient receiving prophylactic doses of LMWH developed a new VTE. Major or minor bleeding complications occurred in 18% (3 of 17 children) and 4% (1 of 24 children) of those receiving therapeutic and prophylactic doses, respectively. All 4 patients who experienced hemorrhagic complications had other bleeding risk factors—i.e., coagulopathies and antiplatelet medications. CONCLUSIONS LMWH seems to be safe and efficacious for both management and prophylaxis of VTEs in pediatric neurosurgery. However, pediatric practitioners should be aware of higher risk for bleeding complications with increasing doses of LMWH, especially in patients with preexisting bleeding disorders or concurrent use of antiplatelet agents.

OBJECT The aim of this study was to determine the safety of recombinant human bone morphogenetic protein–2 (rhBMP-2) use in posterior instrumented fusions in the pediatric population, focusing on cancer risk. In a previous study, the authors reported the short-term (mean follow-up of 11 months) safety and efficacy of rhBMP-2 in the pediatric age group. The present study reports their results with a minimum of 24 months' follow-up. METHODS The authors retrospectively reviewed 57 consecutive cases involving pediatric patients who underwent posterior occiptocervical, cervical, thoracic, lumbar, or lumbosacral spine fusion from October 1, 2007, to June 30, 2011, at Texas Children's Hospital. Seven cases were excluded from further analysis because of loss to follow-up. Three patients died during the follow-up period and were placed in a separate cohort. RESULTS The patients' average age at the time of surgery was 11 years, 4 months (range 9 months to 20 years). The mean duration of follow-up was 48.4 months (range 24–70 months). Cancer status was determined at the most recent encounter with the patient and/or caretaker(s) in person, or in telephone follow-up. Twenty-four or more months after administration of rhBMP-2, there were no cases of new malignancy, degeneration, or metastasis of existing tumors. The cause of death of the patients who died during the study period was not related to BMP or to the development, degeneration, or metastasis of cancer. CONCLUSIONS Despite the large number of adult studies reporting increased cancer risk associated with BMP use, the authors' outcomes with rhBMP-2 in the pediatric population suggest that it is a safe adjunct to posterior spine fusions of the occipitocervical, cervical, thoracic, lumbar, and lumbosacral spine. There were no new cases of cancer, or degeneration or metastasis of existing malignancies in this series.

Upper airway obstruction resulting from overflexion of the craniocervical junction after occipitocervical fusion is a rare but potentially life-threatening complication and is associated with morbidity. The authors retrospectively reviewed the medical records and diagnostic images of 2 pediatrie patients who underwent occipitocervical fusion by the Neuro-Spine Program at Texas Children’s Hospital and experienced dyspnea and/or dysphagia from new upper airway obstruction in the postoperative period. Patient demographics, operative data, and preoperative and postoperative occiput-C2 angles were recorded. A review of the literature for similar complications after occipitocervical fusion was performed. A total of 13 cases of prolonged upper airway obstruction after occipitocervical fusion were analyzed. Most of these cases involved adults with rheumatoid arthritis. To the best of the authors’ knowledge, there have been no previous reports of prolonged upper airway obstruction in children after an occipitocervical fusion. Fixation of the neck in increased flexion (−18° to −5°) was a common finding among these adult and pediatrie cases. The authors’ cases involved children with micrognathia and comparatively large tongues, which may predispose the oropharynx to obstruction with even the slightest amount of increased flexion. Close attention to a satisfactory fixation angle (occiput-C2 angle) is necessary to avoid airway obstruction after an occipitocervical fusion. Children with micrognathia are particularly sensitive to changes in flexion at the craniocervical junction after occipitocervical fixation.

BACKGROUND High-grade astrocytomas (HGA) carry a dismal prognosis and compose nearly 20% of all childhood brain tumors. The role of high-dose chemotherapy (HDCT) in the treatment of HGA remains unclear. METHODS In a nationwide study, The Children's Cancer Group (CCG) prospectively evaluated 102 children with HGA and postoperative residual disease for efficacy and toxicity of four courses of HDCT before radiotherapy (RT). Patients were randomly assigned to one of three couplets of drugs: carboplatin/etoposide (Regimen A), ifosfamide/etoposide (Regimen B), or cyclophosphamide/etoposide (Regimen C). After HDCT, all patients were to receive local RT followed by lomustine and vincristine. Twenty-six patients were excluded after central neuroradiographic review (n = 8) or pathology review (n = 18). RESULTS Of 76 evaluable patients (median age, 11.95 yrs; range, 3–20 yrs), 30 patients relapsed during HDCT, and 11 others did not complete HDCT because of toxicity. Nonhematologic serious toxicities were common (29%), and 21% of patients did not receive RT. Objective response rates were not associated with amount of residual disease and did not statistically differ between regimens: 27% (Regimen A), 8% (Regimen B), and 29% (Regimen C). Overall survival (OS) was 24% ± 5% at 5 years and did not differ between groups. Median time to an event was longest for Regimen A (283 days compared with 83 and 91 days for Regimens B and C, respectively). The five-year, event-free survival (EFS) rate for all patients was 8% ± 3% and 14% ± 7% for Regimen A (P = 0.07). CONCLUSIONS OS and EFS were not affected by histologic grade. Patients who responded to HDCT had a nominally higher survival rate (P = 0.03 for trend). The authors conclude that these commonly used HDCT regimens provide no additional clinical benefit to conventional treatment in HGA, regardless of the amount of measurable residual tumor. Cancer 2005. © 2005 American Cancer Society.

Object There have been no large-scale analyses on cost drivers in CSF shunt surgery for the treatment of pediatric hydrocephalus. The objective of this study was to develop a cost model for hospitalization costs in pediatric CSF shunt surgery and to examine risk factors for increased costs. Methods Data were extracted from the Kids' Inpatient Database (KID) of the Healthcare Cost and Utilization Project. Children with initial CSF shunt placement in the 2009 KID were examined. Patient charge was converted to cost using a cost-to-charge ratio. The factors associated with costs of CSF shunt hospitalizations were examined, including patient demographics, hospital characteristics, and clinical data. The natural log transformation of cost per inpatient day (CoPID) was analyzed. Three multivariate linear regression models were used to characterize the cost. Variance inflation factor was used to identify multicollinearity for each model. Results A total of 2519 patients met the inclusion criteria and were included in study. Average cost and length of stay (LOS) for initial shunt placement were $49,317 ± $74,483 (US) and 18.2 ± 28.5 days, respectively. Cost per inpatient day was $4249 ± $2837 (median $3397, range $80–$22,263). The average number of registered nurse (RN) full-time equivalents (FTEs) per 1000 adjusted inpatient days was 5.8 (range 1.6–10.8). The final model had the highest adjusted coefficient of determination (R2 = 0.32) and was determined to be the best among 3 models. The final model showed that child age, hydrocephalus etiology, weekend admission, number of chronic diseases, hospital type, number of RN FTEs per 1000 adjusted inpatient days, number of procedures, race, insurance type, income level, and hospital regions were associated with CoPID. Conclusions A patient's socioeconomic status, such as race, income level, and insurance, in addition to hospitalrelated factors such as number of hospital RN FTEs, hospital type, and US region, could affect the costs of initial CSF shunt placement, in addition to clinical factors such as hydrocephalus origin and LOS. To create a cost model of initial CSF shunt placement in the pediatric population, consideration of such nonclinical factors may be warranted.

Wound-related complications following complex posterior spine procedures in children may result in the need for serial debridements and may place spinal instrumentation at risk. In this study, the authors review their experience with the management of soft-tissue defects from spinal instrumentation in 5 high-risk pediatric patients. The use of various rotational and transpositional flaps in the management of these complicated cases is discussed, as well as their outcomes.The authors retrospectively reviewed the medical records of 5 patients who returned to the Neuro-Spine service at Texas Children's Hospital for erosion of spinal instrumentation through the skin between September 1, 2007, and October 31, 2012. Patient demographics and clinical and operative data were recorded.Risk factors such as young age (1 case), poor nutritional status (1 case), multiple previous surgeries (3 cases), severe neurological deficits (2 cases), and history of radiation therapy for malignancy (2 cases) were noted in the 5 patients. The paraspinous flap (4 cases) was the mainstay of the treatment. Follow-up ranged from 7.5 to 17.5 months (mean 11 ± 4.2 months). One of the patients required more than 1 procedure for revision of the wound. Cultures were positive in 2 of the 5 cases. Spinal instrumentation was removed in 3 of the 5 cases; however, in all 3 of the cases there was evidence of delayed instability that developed after the removal of spinal instrumentation.The use of local tissue flaps is safe and efficacious for treatment of posterior wound complications due to spinal instrumentation in children. Removal of spinal instrumentation should be avoided due to the development of delayed instability. Highly vascularized tissue is used to speed healing, clear bacteria, and eliminate dead space, obviating the need to remove contaminated spinal instrumentation.

Object Shunt survival may improve when ventricular catheters are placed into the frontal horn or trigone of the lateral ventricle. However, techniques for accurate catheter placement have not been developed. The authors recently reported a prospective study designed to test the accuracy of catheter placement with the assistance of intraoperative ultrasound, but the results were poor (accurate placement in 59%). A major reason for the poor accurate placement rate was catheter movement that occurred between the time of the intraoperative ultrasound image and the first postoperative scan (33% of cases). The control group of non–ultrasound using surgeons also had a low rate of accurate placement (accurate placement in 49%). The authors conducted an exploratory post hoc analysis of patients in their ultrasound study to identify factors associated with either catheter movement or poor catheter placement so that improved surgical techniques for catheter insertion could be developed. Methods The authors investigated the following risk factors for catheter movement and poor catheter placement: age, ventricular size, cortical mantle thickness, surgeon experience, surgeon experience with ultrasound prior to trial, shunt entry site, shunt hardware at entry site, ventricular catheter length, and use of an ultrasound probe guide for catheter insertion. Univariate analysis followed by multivariate logistic regression models were used to determine which factors were independent risk factors for either catheter movement or inaccurate catheter location. Results In the univariate analyses, only age < 6 months was associated with catheter movement (p = 0.021); cortical mantle thickness < 1 cm was near-significant (p = 0.066). In a multivariate model, age remained significant after adjusting for cortical mantle thickness (OR 8.35, exact 95% CI 1.20–infinity). Univariate analyses of factors associated with inaccurate catheter placement showed that age < 6 months (p = 0.001) and a posterior shunt entry site (p = 0.021) were both associated with poor catheter placement. In a multivariate model, both age < 6 months and a posterior shunt entry site were independent risk factors for poor catheter placement (OR 4.54, 95% CI 1.80–11.42, and OR 2.59, 95% CI 1.14–5.89, respectively). Conclusions Catheter movement and inaccurate catheter placement are both more likely to occur in young patients (< 6 months). Inaccurate catheter placement is also more likely to occur in cases involving a posterior shunt entry site than those involving an anterior shunt entry site. Future clinical studies aimed at improving shunt placement techniques must consider the effects of young age and choice of entry site on catheter location.

It is common to evaluate children with suspected CSF shunt malfunctions using CT of the head or, more recently, "quick brain" MRI. However, the reliability of using ventricular behavior, as assessed on cranial imaging during previous presentations with shunt obstructions, is not well defined. The authors conducted a study to determine if CT or MRI of ventricular morphology added useful clinical information in the evaluation of shunt malfunctions.A retrospective chart review of children operated on at Texas Children's Hospital from February 20, 2011, to June 18, 2013, for shunt obstruction was conducted. Inclusion criteria involved age 3 years or older in patients who had undergone two or more shunt revisions for intraoperatively confirmed obstructions. Patients with shunt infection but without shunt obstruction and patients with fourth ventricular shunt failure were excluded from the study. Preoperative CT or MRI results were dichotomized into two distinct categories, as determined by a radiologist's report: either dilation of the ventricular system in comparison with prior scans at points the shunt was deemed functional, or no dilation of the ventricular system in comparison such scans. Determination of the presence of shunt obstruction was assessed by findings documented by the surgeon in the operative report. Each case was then analyzed to see if the patient has a reliable pattern of ventricular dilation, or no dilation, at times of shunt obstruction.Forty-two patients (25 males and 17 females) were included in the study. There were a total of 117 patient encounters analyzed and an average of 2.79 encounters per patient. The mean age at shunt failure presentation was 10.8 years (range 3-23 years). In 4 encounters, patients presented with a CSF leak or pseudomeningocele. Twenty-seven patients (64%) consistently demonstrated dilation of the ventricular system during episodes of shunt obstruction. Four patients (10%) consistently demonstrated no dilation during episodes of shunt obstruction. Eleven patients (26%) demonstrated inconsistent changes in ventricular size at times of shunt obstruction. In those first patient encounters with shunt obstruction presenting with ventricular dilation, 92% (49 of 53) of subsequent encounters demonstrated ventricular dilation with shunt obstruction presentations.Historical CT or MRI data regarding ventricular morphology patterns seen during prior examinations of shunt obstructions may inform a clinician's judgment of shunt obstruction on subsequent presentations, but they are not conclusive. In the present series, the authors found that changes in the morphology of a given patient's ventricular system when shunt obstruction occurs were often consistent and predictable, but not always. It remains imperative, however, that cranial images obtained to rule out shunt malfunction be compared with prior studies.

Recent attempts to control health care costs focus on reducing or eliminating payments for complications, hospital-acquired conditions, and provider preventable conditions, with payment restrictions applied uniformly. A patient's preexisting comorbidities likely influence the perioperative complication incidence. This relationship has not previously been examined in pediatric neurosurgery.The authors conducted a retrospective assessment of prospectively collected relevant patient comorbidities and morbidity and mortality events at a large pediatric neurosurgical unit over a 5-year period. The authors examined the impact of specific comorbidities and the cumulative effect of multiple comorbidities on complication incidence.A total of 1990 patients underwent 3195 procedures at the authors' institution during the 5-year study period. Overall, 396 complications were analyzed; 298 patients (15.0%) experienced at least one complication. One or more comorbidities were present in 45.9% of patients. Renal comorbidities were clearly associated with the increased incidence of complications (p = 0.02), and they were specifically associated with infection (p = 0.006). Neurological comorbidities had a borderline association with complications (p = 0.05), and they were specifically associated with death (p = 0.037). A patient's having more comorbidities did not correlate with an increased risk of a perioperative complication (p = 0.8275).The complication incidence in pediatric neurosurgery is variable and may be influenced by the type of neurosurgical procedure and patient-related factors. While patient-related factors beyond the control of the provider can significantly impact complications and hospital-acquired conditions in pediatric neurosurgery, an increasing number of comorbidities do not correlate with an increased risk of complications per patient.

Objectives To develop a cost model for hospitalization costs of surgery among children with Chiari malformation type 1 (CM-1) and to examine risk factors for increased costs. Study design Data were extracted from the US National Healthcare Cost and Utilization Project 2009 Kids' Inpatient Database. The study cohort was comprised of patients aged 0-20 years who underwent CM-1 surgery. Patient charges were converted to costs by cost-to-charge ratios. Simple and multivariable generalized linear models were used to construct cost models and to determine factors associated with increased hospital costs of CM-1 surgery. Results A total of 1075 patients were included. Median age was 11 years (IQR 5-16 years). Payers included public (32.9%) and private (61.5%) insurers. Median wage-adjusted cost and length-of-stay for CM-1 surgery were US $13 598 (IQR $10 475-$18 266) and 3 days (IQR 3-4 days). Higher costs were found at freestanding children's hospitals: average incremental-increased cost (AIIC) was US $5155 (95% CI $2067-$8749). Factors most associated with increased hospitalization costs were patients with device-dependent complex chronic conditions (AIIC $20 617, 95% CI $13 721-$29 026) and medical complications (AIIC $13 632, 95% CI $7163-$21 845). Neurologic and neuromuscular, metabolic, gastrointestinal, and other congenital genetic defect complex chronic conditions were also associated with higher hospital costs. Conclusions This study examined cost drivers for surgery for CM-1; the results may serve as a starting point in informing the development of financial risk models, such as bundled payments or prospective payment systems for these procedures. Beyond financial implications, the study identified specific risk factors associated with increased costs.

Objectives To describe the variation in approaches to surgical and antibiotic treatment for first cerebrospinal fluid (CSF) shunt infection and adherence to Infectious Diseases Society of America (IDSA) guidelines. Study design We conducted a prospective cohort study of children undergoing treatment for first CSF infection at 7 Hydrocephalus Clinical Research Network hospitals from April 2008 through December 2012. Univariate analyses were performed to describe the study population. Results A total of 151 children underwent treatment for first CSF shunt-related infection. Most children had undergone initial CSF shunt placement before the age of 6 months (n = 98, 65%). Median time to infection after shunt surgery was 28 days (IQR 15-52 days). Surgical management was most often shunt removal with interim external ventricular drain placement, followed by new shunt insertion (n = 122, 81%). Median time from first negative CSF culture to final surgical procedure was 14 days (IQR 10-21 days). Median duration of intravenous (IV) antibiotic use duration was 19 days (IQR 12-28 days). For 84 infections addressed by IDSA guidelines, 7 (8%) met guidelines and 61 (73%) had longer duration of IV antibiotic use than recommended. Conclusions Surgical treatment for infection frequently adheres to IDSA guidelines of shunt removal with external ventricular drain placement followed by new shunt insertion. However, duration of IV antibiotic use in CSF shunt infection treatment was consistently longer than recommended by the 2004 IDSA guidelines.

Nonterminal myelocystocele is a rare type of spinal dysraphism characterized by a closed defect with an underlying CSF-filled cyst, either contiguous with the central spinal canal or attached to the spinal cord by a fibrovascular stalk. The authors report the unusual case of a neonate with a prenatal diagnosis of cervicothoracic nonterminal myelocystocele who underwent postnatal surgical untethering of the lesion. Pathological analysis of the excised lesion revealed neuroglial tissue with an ependymal lining associated with a mature teratoma. Three months after surgery, the patient has normal lower-extremity sensorimotor function and no evidence of bowel or bladder dysfunction. To the best of the authors' knowledge, this is the first report of a patient with a nonterminal myelocystocele found to have an associated mature teratoma.

Object Cervical spondylolysis is a rare condition that results from a pars interarticularis defect. The C-6 level is the most frequently involved site in the cervical spine. Its clinical presentations range from incidental radiographic findings to neck pain and, rarely, neurological deficits. Although 150 patients with subaxial cervical spondylolysis have been reported, a mere 24 adult and pediatric patients with C-2 spondylolysis have been described. The long-term outcomes of very young children with bilateral C-2 spondylolysis are of great interest, yet only a few longitudinal studies exist. Methods The authors retrospectively reviewed 5 cases of bilateral C-2 spondylolysis at Texas Children's Hospital and Riley Children's Hospital; these were combined with 5 other cases in the literature, yielding a total of 10 patients. Data regarding the patients' age, sex, C2–3 angulation and displacement, associated spine anomalies, neurological deficits, treatment, and most recent follow-up were recorded. Results The patients' ages ranged from 3 to 36 months (mean 12.9 months). There were 6 boys and 4 girls. The C2–3 angulation, displacement, and width of pars defect were measured when available. The mean C2–3 angulation was 9.5° (range 1–34°), the mean C2–3 displacement was 4.78 mm (range 1.1–10.8 mm), and the mean width of the pars defect was 4.16 mm (range 0.9–7 mm). One patient developed myelopathy and spinal cord injury. All 10 of the patients were treated initially with conservative therapy: 3 with close observation alone, 1 with a rigid cervical collar, 4 with a Minerva jacket, 1 with a sternal-occipital-mandibular immobilizer, and 1 with a halo vest. Three patients ultimately underwent surgery for internal fixation due to progressive instability or development of neurological symptoms. All patients were neurologically intact at the last follow-up (mean 44.3 months, range 14–120 months). Conclusions Based on the literature and the authors' own experience, they conclude that most very young children with C-2 spondylolysis remain neurologically intact and maintain stability in long-term follow-up despite the bony defect. This defect is often an asymptomatic incidental finding and may be managed conservatively. More aggressive therapy including surgery is indicated for those patients with a neurological deficit from spinal cord compromise secondary to stenosis and local C-2 kyphosis, progressive deformity, or worsening C2–3 instability.

Object. Craniopharyngiomas originate from the same cells as squamous cell skin carcinoma, which can be treated successfully with interferon-α (IFNα)-2a. The authors evaluated the activity and toxicity of systemic IFN in young patients with craniopharyngiomas.Methods. Fifteen patients between the ages of 4.2 and 19.8 years who had progressive or recurrent craniopharyngiomas were enrolled in this study. Nine of these patients had never received external-beam radiation therapy. Therapy consisted of 8,000,000 U/m2 IFNα-2a administered daily for 16 weeks (induction phase) followed by the same dose three times per week for an additional 32 weeks (maintenance phase). Of the 12 patients who could be evaluated, radiological studies demonstrated a response to treatment in three with predominantly cystic tumors (one minor response, one partial response, and one complete response); one of these patients also showed improvement in visual fields. The size of the cystic component of the tumors often increased temporarily during the first several months of therapy. Three patients met the criteria for progressive disease during therapy. The median time to progression was 25 months. The need for radiation therapy in patients treated with IFN was delayed for 18 to 35 months (median 25 months) in six patients. All patients developed transient flulike symptoms shortly after receiving the first dose of IFN. Other toxicities (predominantly hepatic, neurological, and cutaneous) were seen in nine (60%) of the 15 patients during the first 8 weeks of treatment but resolved after temporary discontinuation and/or dose reduction.Conclusions. Interferon-α-2a is active against some childhood craniopharyngiomas; its toxicity precludes administration of high daily doses, and the optimum dose level and schedule remain to be defined.

Chiari III malformations are extremely rare hindbrain malformations that are associated with a high early mortality rate, or severe neurologic deficits in the survivors. The preferred treatment is early operative closure and CSF shunting. We report a case of a newborn infant with a Chiari III malformation with displacement of the brainstem and cerebellum into the cervical encephalocele which precluded immediate operative closure of the defect. Instead, a ventriculoperitoneal shunt was placed and the patient was followed with serial imaging studies. The child survived. The shunt allowed the brainstem and cerebellum to regress into the cervical spinal canal as the dilated cerebral aqueduct and fourth ventricle decompressed. A delayed closure of the cervical encephalocele was performed at 30 months of age. Cerebrospinal fluid diversion with delayed closure may be an option for large lesions.

Over 85% of patients with myelomeningoceles require placement of a ventriculoperitoneal shunt for hydrocephalus, and between 25% and 85% of these patients develop scoliosis. Although most patients undergo repeated shunt series radiography to evaluate for device malfunction, scoliosis radiographs are less consistently obtained. The authors sought to determine if a correlation exists between these 2 radiographic techniques for a given patient, as shunt series are obtained with the patient supine, whereas scoliosis radiographs are acquired with the patient standing upright. The authors also endeavored to study if shunt series radiographs can reliably detect significant scoliosis.The authors retrospectively reviewed a single institution's series of 593 patients with myelomeningoceles and identified all patients in whom a shunt series and scoliosis radiographs were obtained within a 6-month period. They reviewed the medical records and radiographs of these patients for demographic and radiographic parameters. They then applied a linear regression model and determined shunt series curve cutoffs to detect scoliotic curves greater than 20° and 50°.Of the 593 patients identified, 116 did not have radiographs available for interpretation. Of the remaining 477 patients, 201 had radiographic evidence of scoliosis (42%), and 66 had both a shunt series and a scoliosis radiographs acquired within a 6-month interval. In 4 patients, both end vertebrae of the scoliotic curve could not be visualized on a single radiograph. The mean age of the remaining cohort was 10.6 ± 5.2 years and the mean curve magnitude was 58° ± 37°. Using identical end vertebrae, the mean shunt series curve magnitude was 49° ± 35°. The mean interval between both radiographs was 2.3 ± 3.3 months. The regression model showed a strong linear association between shunt series and scoliosis series curves. A curve greater than 19° on shunt series radiographs would detect significant curves of greater than 20° on scoliosis series with 91% sensitivity and 78% specificity. A shunt series curve greater than 37° had 100% sensitivity and 93% specificity in identifying significant scoliotic curves greater than 50°.Although shunt series radiographs may not precisely depict scoliotic curve magnitude because the impact of gravity is negated, they may be useful in helping to confirm clinical suspicion of scoliosis. The authors' results suggest a strong correlation between both types of radiographs.

Left ventricular assist devices (LVADs) are continuous or pulsatile flow devices that could potentially be life-saving measures for patients with end-stage heart failure. These devices have clear advantages over extracorporeal membrane oxygenation (ECMO) and are often used in adults. They are only recently being commonly used in the pediatric age group. As the use of LVADs becomes more mainstream in children, it is important to determine the complication profile associated with these devices. Furthermore, with the increasing application of LVADs in children, pediatric neurosurgeons are seeing a correlative increase in associated neurological complications. In this study, the authors reviewed the incidence of neurological complications due to LVAD use in the pediatric age group and the role of neurosurgery in treatment.The authors examined data regarding patients with LVADs from the Texas Children's Hospital Heart Center database (July 01, 2007, to June 30, 2011) and recorded neurological complications requiring neurosurgical consultation. They identified 2 children who underwent craniotomies during LVAD treatment.Intracranial hemorrhage occurred in 3 (6.5%) of the 46 patients treated with an LVAD at the authors' institution. Of these patients, 2 were treated with craniotomies for life-threatening intracranial hemorrhages. The 3 patients in the neurosurgical cohort presented with cerebral infarction, decreased level of consciousness, and/or seizure. At the last follow-up (286, 503, and 550 days), 1 patient (case 2) had no decline in neurological status, underwent a successful heart transplant, and was discharged home; 1 patient (case 1) died of refractory cardiac failure; and 1 patient (case 3) was on an LVAD for destination therapy (that is, the LVAD is not a bridge to transplantation but rather the final treatment). This patient was discharged from the hospital, but he died of overwhelming fungemia at 286 days while on VAD support.Intracranial hemorrhage is a serious and feared complication of LVAD treatment. While the surgical risk is substantial due to systemic anticoagulation and significant medical comorbidities, neurosurgical evacuation of hemorrhage plays an important life-saving role that can yield successful and acceptable outcomes.

Tuberculosis (TB) is a common disease worldwide that is caused by Mycobacterium tuberculosis. Tuberculosis of the spine, also called Pott disease, is the most common site of bony dissemination. Although children are disproportionately affected, spinal TB is nonetheless rare in very young children. Cases involving infants requiring surgical intervention have been previously reported, and they are often associated with greater management challenges given the technical difficulty with instrumentation in very young children. This case involved a 3-year-old girl with TB centered at T-6, who presented with myelopathy from spinal cord compression and a severe kyphotic deformity (> 60°). She underwent a single-stage costotransversectomy for vertebral column resection, followed by reconstruction with an anterior expandable titanium cage and posterior pedicle screw instrumentation. At last follow-up, the patient was clinically and radiographically stable. The authors report on the youngest patient with spinal TB treated surgically with this strategy and review the literature regarding prior cases involving young children. Although limited by the paucity of cases in the literature, surgical debridement and spinal fusion appear to provide a safe alternative to prolonged bed rest or casting and may offer additional benefits of a faster recovery and ambulation.

Multiple-suture synostosis is typically associated with syndromic craniosynostosis but has been occasionally reported in large series of nonsyndromic children. The diagnosis of multiple fused sutures usually occurs at the same time, but rarely has the chronological development of a secondary suture synostosis been noted. The development of secondary bicoronal suture synostosis requiring surgical intervention has only been reported, to date, after surgical intervention and is hypothesized to arise from a disruption of inhibitory factors from the dura. The disinhibition of these factors permits the sutures to then fuse at an early stage. The authors report on a patient who developed secondary unilateral coronal synostosis after the diagnosis of an isolated sagittal synostosis. The secondary synostosis was identified at the time of the initial surgical intervention and ultimately required a second procedure of a frontoorbital advancement. The clinical appearance of this phenomenon may be subtle, and surgeons should monitor for the presence of secondary synostosis during surgery as it may require intervention. Failure to identify the secondary synostosis may necessitate another surgery or result in a poor cosmetic outcome. The authors recommend close clinical follow-up for the short term in patients with isolated sagittal synostosis.