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Background Upper airway obstruction leading to dyspnea and dysphagia after occipitocervical fusion is a rare complication that has significant morbidity. Objective To estimate the frequency of postoperative dyspnea and dysphagia in children after occipitocervical fusion and to identify variables associated with its occurrence. Methods We retrospectively reviewed outcomes from all pediatric occipitocervical fusions at our institution between 2007 and 2014. Pre- and postoperative computed tomography (CT) scans were compared to determine differences in the clivoaxial (OC2) angles. Results Sixty-seven pediatric patients underwent occipitocervical fusions. Median age was 9.6 yr (range 6 mo-18 yr). Fifty-six of 62 patients (90.3%) with at least 1 yr of follow-up had successful fusions. Eleven had pre-existing symptoms or otherwise compromised examination (eg, severe traumatic brain injury). None of 15 patients placed in extension (>2 degrees) relative to preoperative CT in Situ position developed new dyspnea or dysphagia. Nine of forty patients (23%) kept in Situ or flexed position developed new symptoms of dyspnea or dysphagia. Dysphagia in patients fused in the in Situ position was milder and resolved within a few weeks. No patient under age 5 (n = 20) developed symptoms of dyspnea or dysphagia regardless of head position. There were 3 cases of infection, 1 clinically silent vertebral artery injury, and 3 deaths at last follow-up. Conclusion Positioning of the child's head prior to occipitocervical fusion has considerable impact on outcomes, especially in older children. Careful measurements of the OC2 angle during surgery to ensure optimal head positioning in Situ or slightly extended position may prevent postoperative dysphagia or dyspnea.

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 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.

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.

Object 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. Methods 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. Results 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. Conclusions 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 Current national patterns as a function of patient-, hospital-, and procedure-related factors, and complication rates in the use of recombinant human bone morphogenetic protein–2 (rhBMP-2) as an adjunct to the practice of pediatric spine surgery have scarcely been investigated. Methods The authors conducted a cross-sectional study using data from the Healthcare Cost and Utilization Project Kids' Inpatient Database. Univariate and multivariate logistic regression were used to calculate unadjusted and adjusted odds ratios and 95% confidence intervals, and p values < 0.05 were considered to be statistically significant. Results The authors identified 9538 hospitalizations in pediatric patients 20 years old or younger who had undergone spinal fusion in the US in 2009; 1541 of these admissions were associated with rhBMP-2 use. By multivariate logistic regression, the following factors were associated with rhBMP-2 use: patient age 15–20 years; length of hospital stay (adjusted odds ratio [aOR] 1.01, p = 0.017); insurance status (private [aOR 1.49, p < 0.001] compared with Medicaid); hospital type (nonchildren's hospital); region (Midwest [aOR 2.49, p = 0.008] compared with Northeast); spinal refusion (aOR 2.20, p < 0.001); spinal fusion approach/segment (anterior lumbar [aOR 1.73, p < 0.001] and occipitocervical [aOR 1.86, p = 0.013] compared with posterior lumbar); short segment length (aOR 1.42, p = 0.016) and midlength (aOR 1.44, p = 0.005) compared with long; and preoperative diagnosis (Scheuermann kyphosis [aOR 1.56, p < 0.017] and spondylolisthesis [aOR 1.93, p < 0.001]). Conclusions Use of BMP in pediatric spine procedures now comprises more than 10% of pediatric spinal fusion. Patient-related (age, insurance type, diagnosis); hospital-related (children's hospital vs general hospital, region in the US); and procedure-related (redo fusion, anterior vs posterior approach, spinal levels, number of levels fused) factors are associated with the variation in BMP use in the US.

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.

Object Quality improvement methods are being implemented in various areas of medicine. In an effort to reduce the complex (instrumented) spine infection rate in pediatric patients, a standardized protocol was developed and implemented at an institution with a high case volume of instrumented spine fusion procedures in the pediatric age group. Methods Members of the Texas Children's Hospital Spine Study Group developed the protocol incrementally by using the current literature and prior institutional experience until consensus was obtained. The protocol was prospectively applied to all children undergoing complex spine surgery starting August 21, 2012. Acute infections were defined as positive wound cultures within 12 weeks of surgery, defined in alignment with current hospital infection control criteria. Procedures and infections were measured before and after protocol implementation. This protocol received full review and approval of the Baylor College of Medicine institutional review board. Results Nine spine surgeons performed 267 procedures between August 21, 2012, and September 30, 2013. The minimum follow-up was 12 weeks. The annual institutional infection rate prior to the protocol (2007–2011) ranged from 3.4% to 8.9%, with an average of 5.8%. After introducing the protocol, the infection rate decreased to 2.2% (6 infections of 267 cases) (p = 0.0362; absolute risk reduction 3.6%; relative risk 0.41 [95% CI 0.18–0.94]). Overall compliance with data form completion was 63.7%. In 4 of the 6 cases of infection, noncompliance with completion of the data collection form was documented; moreover, 2 of the 4 spine surgeons whose patients experienced infections had the lowest compliance rates in the study group. Conclusions The standardized protocol for complex spine surgery significantly reduced surgical site infection at the authors' institution. The overall compliance with entry into the protocol was good. Identification of factors associated with post–spine surgery wound infection will allow further protocol refinement in the future.

Vertebral hemangiomas are common benign vascular tumors of the spine. It is very rare for these lesions to symptomatically compress neural elements. If spinal cord compression does occur, it usually involves only a single level. Multilevel vertebral hemangiomas causing symptomatic spinal cord compression have never been reported in the pediatric population to the best of our knowledge. We report the case of a 15-year-old boy presenting with progressive paraparesis due to thoracic spinal cord compression from a multilevel thoracic hemangioma (T5–T10) with epidural extension. Because of his progressive neurological deficit, he was initially treated with urgent multilevel decompressive laminectomies from T4 to T11. This was to be followed by radiotherapy for residual tumor, but the patient was unfortunately lost to follow-up. He re-presented 3 years later with recurrent paraparesis and progressive disease. This was treated with urgent radiotherapy with good response. As of 6 months follow-up, he has made an excellent neurological recovery. In this report, we present the first case of a child with multilevel vertebral hemangiomas causing symptomatic spinal cord compression and review the literature to detail the pathophysiology, management, and treatment of other cases of spinal cord compression by vertebral hemangiomas.

Cranial osteosarcoma is very rare in children, rendering the development of optimal treatment algorithms challenging. The authors present 3 cases of pediatric cranial osteosarcoma: a primary calvarial tumor, a cranial metastasis, and a primary osteosarcoma of the cranial base. A review of the literature demonstrates significant variation in the management of cranial osteosarcomas and the outcome for patients with these tumors. This series and literature review is presented to improve the understanding of pediatric cranial osteosarcoma and to reinforce the importance of maximal resection in optimizing outcome.

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.

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.

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).

Object The most common cause of cervical spine arthrodesis in the pediatric population is instability related to congenital or traumatic pathology. Instrumenting the cervical spine can be challenging given smaller anatomical structures, less ossified bone, and future growth potential and development. Studies in adult patients have suggested that using screw constructs results in improved outcomes with lower rates of instrumentation failure. However, the pediatric literature is limited to small retrospective series. Based on a review of the literature and their own patient series, the authors report that instrumenting the pediatric cervical spine with screw constructs may be safer and more effective than using wiring techniques. Methods The authors reviewed the existing pediatric cervical spine arthrodesis literature and contributed 31 of their own cases from September 1, 2007, to January 1, 2011. They reviewed 204 abstracts from January 1, 1966, to December 31, 2010, and 80 manuscripts with 883 total patients were included in the review. They recorded demographic, radiographic, and outcomes data—as well as surgical details—with a focus on fusion rates and complications. Patients were then grouped into categories based upon the procedure performed: 1) patients who underwent fusions bridging the occipitocervical junction and 2) patients who underwent fusion of the cervical spine that did not include the occiput, thus including atlantoaxial and subaxial fusions. Patients were further subdivided according to the type of instrumentation used—some had posterior cervical fusion with wiring (with or without rod implantation); others had posterior cervical fusion with screws. Results The entire series comprised 914 patients with a mean age of 8.30 years. Congenital abnormalities were encountered most often (in 55% of cases), and patients had a mean follow-up of 32.5 months. From the entire cohort, 242 patients (26%) experienced postsurgical complications, and 50 patients (5%) had multiple complications. The overall fusion rate was 94.4%. For occipitocervical fusions (N = 285), both screw and wiring groups had very high fusion rates (99% and 95%, respectively, p = 0.08). However, wiring was associated with a higher complication rate. From a sample of 252 patients, 14% of those treated with screw instrumentation had complications, compared with 50% of patients treated with wiring (p < 0.05). In cervical fusions not involving the occipitocervical junction (N = 181), screw constructs had a 99% fusion rate, whereas wire instrumentation only had an 83% fusion rate (p < 0.05). Similarly, patients who underwent screw fixation had a lower complication profile (15%) when compared with those treated with wiring constructs (54%, p < 0.05). Conclusions The results of this study are limited by variations in construct design, use of orthoses, follow-up duration, and newer adjuvant products promoting fusions. However, a literature review and the authors' own series of pediatric cases suggest that instrumentation of the cervical spine in children may be safer and more efficacious using screw constructs rather than wiring techniques.

Object 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. Methods 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°. Results 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°. Conclusions 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.

Background Graphene has unique electrical, physical, and chemical properties that may have great potential as a bioscaffold for neuronal regeneration after spinal cord injury. These nanoscaffolds have previously been shown to be biocompatible in vitro; in the present study, we wished to evaluate its biocompatibility in an in vivo spinal cord injury model. Methods Graphene nanoscaffolds were prepared by the mild chemical reduction of graphene oxide. Twenty Wistar rats (19 male and 1 female) underwent hemispinal cord transection at approximately the T2 level. To bridge the lesion, graphene nanoscaffolds with a hydrogel were implanted immediately after spinal cord transection. Control animals were treated with hydrogel matrix alone. Histologic evaluation was performed 3 months after the spinal cord transection to assess in vivo biocompatibility of graphene and to measure the ingrowth of tissue elements adjacent to the graphene nanoscaffold. Results The graphene nanoscaffolds adhered well to the spinal cord tissue. There was no area of pseudocyst around the scaffolds suggestive of cytotoxicity. Instead, histological evaluation showed an ingrowth of connective tissue elements, blood vessels, neurofilaments, and Schwann cells around the graphene nanoscaffolds. Conclusions Graphene is a nanomaterial that is biocompatible with neurons and may have significant biomedical application. It may provide a scaffold for the ingrowth of regenerating axons after spinal cord injury.

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 In a previous report by the same research group (Kestle et al., 2011), compliance with an 11-step protocol was shown to reduce CSF shunt infection at Hydrocephalus Clinical Research Network (HCRN) centers (from 8.7% to 5.7%). Antibiotic-impregnated catheters (AICs) were not part of the protocol but were used off protocol by some surgeons. The authors therefore began using a new protocol that included AICs in an effort to reduce the infection rate further. METHODS The new protocol was implemented at HCRN centers on January 1, 2012, for all shunt procedures (excluding external ventricular drains [EVDs], ventricular reservoirs, and subgaleal shunts). Procedures performed up to September 30, 2013, were included (21 months). Compliance with the protocol and outcome events up to March 30, 2014, were recorded. The definition of infection was unchanged from the authors' previous report. RESULTS A total of 1935 procedures were performed on 1670 patients at 8 HCRN centers. The overall infection rate was 6.0% (95% CI 5.1%–7.2%). Procedure-specific infection rates varied (insertion 5.0%, revision 5.4%, insertion after EVD 8.3%, and insertion after treatment of infection 12.6%). Full compliance with the protocol occurred in 77% of procedures. The infection rate was 5.0% after compliant procedures and 8.7% after noncompliant procedures (p = 0.005). The infection rate when using this new protocol (6.0%, 95% CI 5.1%–7.2%) was similar to the infection rate observed using the authors' old protocol (5.7%, 95% CI 4.6%–7.0%). CONCLUSIONS CSF shunt procedures performed in compliance with a new infection prevention protocol at HCRN centers had a lower infection rate than noncompliant procedures. Implementation of the new protocol (including AICs) was associated with a 6.0% infection rate, similar to the infection rate of 5.7% from the authors' previously reported protocol. Based on the current data, the role of AICs compared with other infection prevention measures is unclear.

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.

OBJECT In a previous study, the authors reported on their experience with the use of sublaminar polyester bands as part of segmental spinal constructs. However, the risk of neurological complications with sublaminar passage of instrumentation, such as spinal cord injury, limits the use of this technique. The present study reports the novel use of subtransverse process polyester bands in posterior instrumented spinal fusions of the thoracic and lumbar spines and sacrum or ilium in 4 patients. METHODS The authors retrospectively reviewed the demographic and procedural data of patients who had undergone posterior instrumented fusion using subtransverse process polyester bands. RESULTS Four patients, ranging in age from 11 to 22 years, underwent posterior instrumented fusion for neuromuscular scoliosis (3 patients) and thoracic hyperkyphosis (1 patient). There were 3 instances of transverse process fracture, with application and tensioning of the polyester band in 1 patient. Importantly, there was no instance of spinal cord injury with subtransverse process passage of the polyester band. The lessons learned from this technique are discussed. CONCLUSIONS This study has shown the “Eleghia” technique of passing subtransverse process bands to be a technically straightforward and neurologically safe method of spinal fixation. Pedicle screws, laminar/pedicle/transverse process hooks, and sublaminar metal wires/bands have been incorporated into posterior spinal constructs; they have been widely reported and used in the thoracic and lumbar spines and sacrum or ilium with varying success. This report demonstrates the promising results of hybrid posterior spinal constructs that include the Eleghia technique of passing subtransverse process polyester bands. This technique incorporates technical ease with minimal risk of neurological injury and biomechanical stability.

Positional skull deformities may be present at birth or may develop during the first few months of life. Since the early 1990s, US pediatricians have seen an increase in the number of children with cranial asymmetry, particularly unilateral flattening of the occiput, likely attributable to parents following the American Academy of Pediatrics “Back to Sleep” positioning recommendations aimed at decreasing the risk of sudden infant death syndrome. Positional skull deformities are generally benign, reversible head-shape anomalies that do not require surgical intervention, as opposed to craniosynostosis, which can result in neurologic damage and progressive craniofacial distortion. Although associated with some risk of positional skull deformity, healthy young infants should be placed down for sleep on their backs. The practice of putting infants to sleep on their backs has been associated with a drastic decrease in the incidence of sudden infant death syndrome. Pediatricians need to be able to properly differentiate infants with benign skull deformities from those with craniosynostosis, educate parents on methods of proactively decreasing the likelihood of the development of occipital flattening, initiate appropriate management, and make referrals when necessary. This report provides guidance for the prevention, diagnosis, and management of positional skull deformity in an otherwise normal infant without evidence of associated anomalies, syndromes, or spinal disease.

Congenital long QT syndrome (LQTS) is a rare genetic ion transmembrane disorder that has been associated with multiple various genetic mutations including life-threatening cardiac arrhythmias and sudden death. Left thorascopic sympathectomy is an effective treatment for patients who are refractory to medical therapy or who need frequent epicardial internal cardiodefibrillator intervention. Although there is substantial literature about this therapy in adults, few reports detail the outcomes in children who undergo left thorascopic sympathectomies to treat LQTS. The authors report the successful use of a left thoracic sympathectomy for the treatment of an 11-year-old girl who had persistently symptomatic LQTS, even after implantation of an automatic cardioverter-defibrillator. The patient remained clinically stable without arrhythmias through 6 months' of follow-up. The authors also reviewed the relevant literature and found that it suggests that 77% of patients will have immediate resolution of their symptoms/arrhythmias after the procedure. When the outcome definition was broadened to include patients who had only 1 or 2 cardiac episodes in the follow-up period, 88% of cases were considered successful. The results of this case study and literature review suggest that left thorascopic sympathectomy is a safe and effective approach for treating pediatric patients with LQTS.

Endoscopic third ventriculostomy (ETV) is an accepted option in the treatment of obstructive hydrocephalus in children and is considered by many pediatric neurosurgeons to be the treatment of choice in this population. The procedure involves perforation of the floor of the third ventricle, specifically, the tuber cinereum, which is part of the hypothalamic-pituitary axis of cerebral endocrine regulation. Endocrine dysfunction, such as amenorrhea, weight gain, and precocious puberty, which are recognized only months to years after the procedure, may be underreported because patients and physicians may not relate the endocrine sequelae to the ETV. Few detailed reports of endocrinerelated complications following ETV exist to better understand these issues. In this study, the authors add to the literature with case descriptions of and correlative laboratory findings in 2 adolescent girls who underwent ETV for obstructive hydrocephalus and in whom amenorrhea subsequently developed.

Object Significant lumbar kyphosis is frequently observed in patients with myelomeningocele and has been associated with increasing functional impairment, decreased abdominal volume, respiratory impairment, discomfort, and skin ulcerations overlying the prominent gibbus. Treatment of severe kyphotic deformities can include kyphectomy, with or without ligation of the thecal sac, with posterior spinal fixation. However, most series have reported a high rate of morbidity and complications associated with surgical intervention for correction of kyphosis in patients with myelomeningocele. The authors describe a technique in which pedicle screw (PS)–only constructs are used without transection of the thecal sac to treat severe kyphosis successfully, with minimal morbidity. Methods The authors retrospectively reviewed medical records and radiographic images in 2 patients with myelomeningoceles in whom kyphectomies had been performed at the authors' institution between January 2007 and July 2010. They also reviewed the existing literature for case reports or published series of patients with myelomeningocele treated with kyphectomies, to evaluate the outcomes. Results Both patients were male and had thoracic-level myelomeningoceles that had been repaired at birth, with associated paraplegia. Neither patient had any significant scoliotic deformity associated with the kyphosis, and both had fixation from T-9 to the ilium, which was performed using PS constructs, along with L1–2 kyphectomies. The patient in Case 1 was 20 years old and was treated for progressive kyphosis and an ulcerated nonhealing wound over the gibbus. The patient in Case 2 was 10 years old and was treated for progressive pain and functional impairment. The 2 patients had a mean correction of 63%, with a mean correction of kyphotic deformity from 136° to 51°. Neither patient developed any complication in the short term postoperatively, whereas published series have reported high complication rates, including wound infection, poor wound healing, CSF leakage, pseudarthrosis, and shunt malfunction. Conclusions Severe kyphotic deformities in patients with myelomeningocele can be safely treated using PS-only constructs without ligation of the thecal sac. Further evaluation with a larger sample and longer follow-up are needed to detect any associated complications, such as proximal junctional kyphosis. Further evaluation may also validate whether PS-only constructs permit successful outcomes with a shorter construct and fewer instrumented levels.

Object Lumbar disc herniation is rare in the pediatric age group, but may still cause a significant amount of pain and disability. Whereas minimally invasive surgery (MIS) for lumbar disc herniation is routinely performed in adults, it has not yet been described in the pediatric population. The purpose of this study was to describe the surgical results of pediatric MIS-treated lumbar disc disease. Methods The authors retrospectively reviewed a series of 6 consecutive cases of lumbar microdiscectomy performed using MIS techniques between April 2008 and July 2010. Presenting symptoms, physical examination findings, and preoperative MR imaging results were obtained from medical records. Perioperative results, including blood loss, length of hospital stay, and complications were assessed. Findings at latest follow-up evaluation were also recorded. Results This report represents the first surgical series regarding pediatric lumbar microdiscectomies performed using MIS. The mean patient age was 16 years (range 14–17 years); there were 4 girls and 2 boys. Preoperative signs and symptoms of radiculopathic pain were notable in 100% of patients, and myotomal weakness was noted in 33% of patients. The first line of treatment in all patients was a period of conservative management lasting an average of 11.5 months (range 6–12 months). The mean intraoperative blood loss was 10.8 ml, and the mean postoperative length of stay was 1.3 days. There were no complications in this small series. Conclusions The treatment of pediatric lumbar disc herniation by using MIS techniques can be safe and efficacious. However, further study with a larger number of patients and longer follow-up is needed to compare outcomes between MIS and open microdiscectomies.

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.

Object Translaminar screw (TLS) fixation can be used safely and efficaciously for upper cervical fusion in children. No published studies have evaluated this technique in the thoracic spine of the pediatric population, and thus the authors undertook such an analysis. Methods The upper thoracic spines (T1–4) of 130 patients, consisting of 70 boys and 60 girls, were studied using CT scans. Laminar height and thickness, screw length, and screw angle were measured. Exclusion criteria included the following: patients older than 18 years of age, trauma or congenital abnormalities of the thoracic spine, or absent demographic information or imaging studies through T-4. Statistical analysis was performed using paired or unpaired Student t-tests (p < 0.05) and linear regression analysis. Results The mean laminar heights for T-1, T-2, T-3, and T-4 were as follows: 12.3 ± 3.4, 13.0 ± 3.5, 13.4 ± 3.8, and 14.7 ± 4.1 mm, respectively. The mean laminar widths were 6.5 ± 1.3, 6.6 ± 1.3, 6.6 ± 1.3, and 6.6 ± 1.4 mm, respectively. The mean screw lengths were 29.9 ± 4.1, 25.2 ± 3.5, 22.7 ± 3.2, and 21.6 ± 3.1 mm, respectively. The mean screw angles were 47° ± 4°, 48° ± 4°, 51° ± 4°, and 53° ± 5°, respectively. There were no significant differences between the right and left sides. However, significant differences were found when comparing patients younger than 8 years with those who were 8 years or older, and when comparing boys and girls. Conclusions Careful preoperative thin-cut CT with sagittal reconstruction is mandatory to determine if the placement of TLSs is feasible in the pediatric population. Based on CT analysis, the insertion of TLSs in the pediatric thoracic spine is possible in all patients older than 8 years and in many patients younger than 8 years. Boys could accept longer screws in the upper thoracic spine compared with girls.

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.

Posterior reversible encephalopathy syndrome (PRES) has been described in the setting of malignant hypertension, renal disease, eclampsia, and immunosuppression. In addition, a single case of intraoperative (posterior fossa craniotomy) PRES has been reported; however, this case occurred in an adult. The authors present a clinically and radiographically documented case of intraoperative PRES complicating the resection of a posterior fossa tumor in a 6-year-old child. During tumor resection, untoward force was used to circumferentially dissect the tumor, and excessive manipulation of the brainstem led to severe hypertension for a 10-minute period. An immediate postoperative MR image was obtained to rule out residual tumor, but instead the image showed findings consistent with PRES. Moreover, the patient's postoperative clinical findings were consistent with PRES. Aggressive postoperative management of blood pressure and the institution of anticonvulsant therapy were undertaken. The patient made a good recovery; however, he required a temporary tracheostomy and tube feedings for prolonged lower cranial nerve dysfunction. Posterior reversible encephalopathy syndrome can occur as a result of severe hypertension during surgery, even among young children. With prompt treatment, the patient in the featured case experienced significant clinical and radiographic recovery.

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.

Object The purpose of this study was to define the incidence of permanent shunt placement and infection in patients who have undergone the 2 most commonly performed temporizing procedures for posthemorrhagic hydrocephalus (PHH) of prematurity: ventriculosubgaleal (VSG) shunt placement and ventricular reservoir placement for intermittent tapping. Methods The 4 centers of the Hydrocephalus Clinical Research Network participated in a retrospective chart review of infants with PHH who underwent treatment at each institution between 2001 and 2006. Patients were included if they had received a diagnosis of Grade 3 or 4 intraventricular hemorrhage, weighed < 1500 g at birth, and had received surgical intervention. The authors determined the incidence of conversion from a temporizing device to a permanent shunt, the incidence of CSF infection during temporization, and the 6-month CSF infection rate after permanent shunt placement. Results Thirty-one (86%) of 36 patients who received VSG shunts and 61 (69%) of 88 patients who received ventricular reservoirs received permanent CSF diversion with a shunt (p = 0.05). Five patients (14%) in the VSG shunt group had CSF infections during temporization, compared with 11 patients (13%) in the ventricular reservoir group (p = 0.83). The 6-month incidence of permanent shunt infection in the VSG shunt group was 16% (5 of 31), compared with 12% (7 of 61) in the reservoir placement group (p = 0.65). For the first 6 months after permanent shunt placement, infants with no preceding temporizing procedure had an infection rate of 5% (1 of 20 infants) and those who had undergone a temporizing procedure had an infection rate of 13% (12 of 92; p = 0.45). Conclusions The use of intermittent tapping of ventricular reservoirs in this population appears to lead to a lower incidence of permanent shunt placement than the use of VSG shunts. The incidence of infection during temporization and for the initial 6 months after conversion appears comparable for both groups. The apparent difference identified in this pilot study requires confirmation in a more rigorous study.

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.

The authors describe the novel use of a table-mounted tubular retractor system (MetRx) originally designed for minimally invasive spine surgery, in the resection of an intraventricular arteriovenous malformation (AVM) in a 12-year-old child. The tubular retractor system may have several advantages over traditional Greenberg or Leyla retractors in selected intracranial procedures. In our case, the low-profile 4 × 22–mm tube and fixed table attachment offered excellent exposure of the trigone of the lateral ventricle where the choroidal AVM was located and from which it was completely resected. Immediate postoperative cerebral angiography confirmed that the entire AVM had been resected. The patient suffered no new neurological deficits as a result of the retractor system or the exposure that it afforded. Although the good clinical results of a single case cannot be directly compared with those obtained using other open techniques of intracranial surgery in larger series, microendoscopic surgery of the brain is an alternative to the other techniques and may be recommended as a time-saving, trauma-reducing procedure with the potential to improve postoperative outcomes.

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.

The use of spinal instrumentation to stabilize the occipitocervical junction in pediatric patients has increased and evolved in recent years. Wiring techniques have now given way to screw-rod or screw-plate techniques with or without postoperative external immobilization. Although C-2 translaminar screws have been used in these constructs, subaxial translaminar screws have not, to date, been described in either the pediatric or adult patient populations. The authors describe the feasibility of translaminar screw placement in the C-3 lamina. Rigid fixation with translaminar screws offers an alternative to subaxial fixation with lateral mass screws, allowing for formation of biomechanically sound spinal constructs and minimizing potential neurovascular morbidity. Their use requires careful analysis of preoperative imaging studies, intact posterior elements, and avoidance of violation of the inner laminar wall.

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.

Juvenile xanthogranuloma is a benign, non-Langerhans-cell histiocytic infiltrate that typically presents as a solitary cutaneous lesion in childhood. There are reports of extracutanous involvement, including tumors in the central nervous system. A solitary, intraparenchymal tumor without skin manifestations is a rare event, with only 3 prior cases reported in the literature. Cerebral lesions have been associated with multifocal or systemic forms of the disease, with an occasionally fulminate clinical course. Considering the rarity of this tumor, it is unclear whether patients need adjuvant therapy after excision of a solitary intraparenchymal tumor. Previous reports suggested that complete excision of the lesion was curative; however long-term follow-up was not provided. This report illustrates a case of surgical excision of a solitary juvenile xanthogranuloma in an 8-year-old male with a 3-year follow-up period.

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.