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OBJECTIVE Interhospital transfer (IHT) to obtain a higher level of care for pediatric patients requiring neurosurgical interventions is common. Pediatric patients with malignant brain tumors often require subspecialty care commonly provided at specialized centers. The authors aimed to assess the impact of IHT in pediatric neurosurgical patients with malignant brain tumors to identify areas of improvement in treatment of this patient population. METHODS Pediatric patients (age < 19 years) with malignant primary brain tumors undergoing craniotomy for resection between 2010 and 2018 were retrospectively identified in the Nationwide Readmissions Database. Patient and hospital data for each index admission provided by the Nationwide Readmissions Database was analyzed by univariate and multivariate analyses. Further analysis evaluated association of IHT on specific patient- or hospital-related characteristics. RESULTS In a total of 2279 nonelective admissions for malignant brain tumors in pediatric patients, the authors found only 132 patients (5.8%) who underwent IHT for a higher level of care. There is an increased likelihood of transfer when a patient is younger (< 7 years old, p = 0.006) or the disease process is more severe, as characterized by higher pediatric complex chronic conditions (p = 0.0004) and increased all patient refined diagnosis-related group mortality index (p = 0.02). Patients who are transferred (OR 1.87, 95% CI 1.04–3.35; p = 0.04) and patients who are treated at pediatric centers (OR 6.89, 95% CI 4.23–11.22; p < 0.0001) are more likely to have a routine discharge home. On multivariate analysis, transfer status was not associated with a longer length of stay (incident rate ratio 1.04, 95% CI 0.94–1.16; p = 0.5) or greater overall costs per patient ($20,947.58, 95% CI −$35,078.80 to $76,974.00; p = 0.50). Additionally, IHT is not associated with increased likelihood of death or major complication. CONCLUSIONS IHT has a significant role in the outcome of pediatric patients with malignant brain tumors. Transfer of this patient population to hospitals providing subspecialized care results in a higher level of care without a significant burden on overall costs, risks, or mortality.

OBJECTIVE Endoscopic third ventriculostomy (ETV) is an option for treatment of hydrocephalus, including for patients who have a history of previous treatment with CSF shunt insertion. The purpose of this study was to report the success of postshunt ETV by using data from a multicenter prospective registry. METHODS Prospectively collected data in the Hydrocephalus Clinical Research Network (HCRN) Core Data Project (i.e., HCRN Registry) were reviewed. Children who underwent ETV between 2008 and 2019 and had a history of previous treatment with a CSF shunt were included. A Kaplan-Meier survival curve was created for the primary outcome: time from postshunt ETV to subsequent CSF shunt placement or revision. Univariable Cox proportional hazards models were created to evaluate for an association between clinical and demographic variables and subsequent shunt surgery. Postshunt ETV complications were also identified and categorized. RESULTS A total of 203 children were included: 57% male and 43% female; 74% White, 23% Black, and 4% other race. The most common hydrocephalus etiologies were postintraventricular hemorrhage secondary to prematurity (56, 28%) and aqueductal stenosis (42, 21%). The ETV Success Score ranged from 10 to 80. The median patient age was 4.1 years. The overall success of postshunt ETV at 6 months was 41%. Only the surgeon’s report of a clear view of the basilar artery was associated with a lower likelihood of postshunt ETV failure (HR 0.43, 95% CI 0.23–0.82, p = 0.009). None of the following variables were associated with postshunt ETV success: age at the time of postshunt ETV, etiology of hydrocephalus, sex, race, ventricle size, number of previous shunt operations, ETV performed at time of shunt infection, and use of external ventricular drainage. Overall, complications were reported in 22% of patients, with CSF leak (8.6%) being the most common complication. CONCLUSIONS Postshunt ETV was successful in treating hydrocephalus, without subsequent need for a CSF shunt, in 41% of patients, with a clear view of the basilar artery being the only variable significantly associated with success. Complications occurred in 22% of patients. ETV is an option for treatment of hydrocephalus in children who have previously undergone shunt placement, but with a lower than expected likelihood of success.

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

OBJECTIVE The primary objective of this trial was to determine if shunt entry site affects the risk of shunt failure. METHODS The authors performed a parallel-design randomized controlled trial with an equal allocation of patients who received shunt placement via the anterior entry site and patients who received shunt placement via the posterior entry site. All patients were children with symptoms or signs of hydrocephalus and ventriculomegaly. Patients were ineligible if they had a prior history of shunt insertion. Patients received a ventriculoperitoneal shunt after randomization; randomization was stratified by surgeon. The primary outcome was shunt failure. The planned minimum follow-up was 18 months. The trial was designed to achieve high power to detect a 10% or greater absolute difference in the shunt failure rate at 1 year. An independent, blinded adjudication committee determined eligibility and the primary outcome. The study was conducted by the Hydrocephalus Clinical Research Network. RESULTS The study randomized 467 pediatric patients at 14 tertiary care pediatric hospitals in North America from April 2015 to January 2019. The adjudication committee, blinded to intervention, excluded 7 patients in each group for not meeting the study inclusion criteria. For the primary analysis, there were 229 patients in the posterior group and 224 patients in the anterior group. The median patient age was 1.3 months, and the most common etiologies of hydrocephalus were postintraventricular hemorrhage secondary to prematurity (32.7%), myelomeningocele (16.8%), and aqueductal stenosis (10.8%). There was no significant difference in the time to shunt failure between the entry sites (log-rank test, stratified by age < 6 months and ≥ 6 months; p = 0.061). The hazard ratio (HR) of a posterior shunt relative to an anterior shunt was calculated using a univariable Cox regression model and was nonsignificant (HR 1.35, 95% CI, 0.98–1.85; p = 0.062). No significant difference was found between entry sites for the surgery duration, number of ventricular catheter passes, ventricular catheter location, and hospital length of stay. There were no significant differences between entry sites for intraoperative complications, postoperative CSF leaks, pseudomeningoceles, shunt infections, skull fractures, postoperative seizures, new-onset epilepsy, or intracranial hemorrhages. CONCLUSIONS This randomized controlled trial comparing the anterior and posterior shunt entry sites has demonstrated no significant difference in the time to shunt failure. Anterior and posterior entry site surgeries were found to have similar outcomes and similar complication rates.

OBJECTIVE Facial palsy can be caused by masses within the posterior fossa and is a known risk of surgery for tumor resection. Although well documented in the adult literature, postoperative facial weakness after posterior fossa tumor resection in pediatric patients has not been well studied. The objective of this work was to determine the incidence of postoperative facial palsy after tumor surgery, and to investigate clinical and radiographic risk factors. METHODS A retrospective analysis was conducted at a single large pediatric hospital. Clinical, radiographic, and histological data were examined in children who were surgically treated for posterior fossa tumors between May 1, 1994, and June 1, 2011. The incidence of postoperative facial weakness was documented. A multivariate logistic regression model was used to analyze the predictive ability of clinicoradiological variables for facial weakness. RESULTS A total of 163 patients were included in this study. The average age at surgery was 7.4 ± 4.7 years, and tumor pathologies included astrocytoma (44%), medulloblastoma (36%), and ependymoma (20%). The lesions of 27 patients (17%) were considered high grade in nature. Thirteen patients (8%) exhibited preoperative symptoms of facial palsy. The overall incidence of postoperative facial palsy was 26% (43 patients), and the incidence of new postoperative facial palsy in patients without preoperative facial weakness was 20% (30 patients). The presence of a preoperative facial palsy had a large and significant effect in univariate analysis (OR 11.82, 95% CI 3.07–45.44, p < 0.01). Multivariate logistic regression identified recurrent operation (OR 4.45, 95% CI 1.49–13.30, p = 0.01) and other preoperative cranial nerve palsy (CNP; OR 3.01, 95% CI 1.24–7.29, p = 0.02) as significant risk factors for postoperative facial weakness. CONCLUSIONS Facial palsy is a risk during surgical resection of posterior fossa brain tumors in the pediatric population. The study results suggest that the incidence of new postoperative facial palsy can be as high as 20%. The presence of preoperative facial palsy, an operation for recurrent tumor, and the presence of other preoperative CNPs were found to be significant risk factors for postoperative facial weakness.

OBJECTIVE Posterior fossa decompression with duraplasty (PFDD) is commonly performed for Chiari I malformation (CM-I) with syringomyelia (SM). However, complication rates associated with various dural graft types are not well established. The objective of this study was to elucidate complication rates within 6 months of surgery among autograft and commonly used nonautologous grafts for pediatric patients who underwent PFDD for CM-I/SM. METHODS The Park-Reeves Syringomyelia Research Consortium database was queried for pediatric patients who had undergone PFDD for CM-I with SM. All patients had tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and ≥ 6 months of postoperative follow-up after PFDD. Complications (e.g., pseudomeningocele, CSF leak, meningitis, and hydrocephalus) and postoperative changes in syrinx size, headaches, and neck pain were compared for autograft versus nonautologous graft. RESULTS A total of 781 PFDD cases were analyzed (359 autograft, 422 nonautologous graft). Nonautologous grafts included bovine pericardium (n = 63), bovine collagen (n = 225), synthetic (n = 99), and human cadaveric allograft (n = 35). Autograft (103/359, 28.7%) had a similar overall complication rate compared to nonautologous graft (143/422, 33.9%) (p = 0.12). However, nonautologous graft was associated with significantly higher rates of pseudomeningocele (p = 0.04) and meningitis (p < 0.001). The higher rate of meningitis was influenced particularly by the higher rate of chemical meningitis (p = 0.002) versus infectious meningitis (p = 0.132). Among 4 types of nonautologous grafts, there were differences in complication rates (p = 0.02), including chemical meningitis (p = 0.01) and postoperative nausea/vomiting (p = 0.03). Allograft demonstrated the lowest complication rates overall (14.3%) and yielded significantly fewer complications compared to bovine collagen (p = 0.02) and synthetic (p = 0.003) grafts. Synthetic graft yielded higher complication rates than autograft (p = 0.01). Autograft and nonautologous graft resulted in equal improvements in syrinx size (p < 0.0001). No differences were found for postoperative changes in headaches or neck pain. CONCLUSIONS In the largest multicenter cohort to date, complication rates for dural autograft and nonautologous graft are similar after PFDD for CM-I/SM, although nonautologous graft results in higher rates of pseudomeningocele and meningitis. Rates of meningitis differ among nonautologous graft types. Autograft and nonautologous graft are equivalent for reducing syrinx size, headaches, and neck pain.

OBJECTIVE Long-term follow-up is often recommended for patients with hydrocephalus, but the frequency of clinical follow-up, timing and modality of imaging, and duration of surveillance have not been clearly defined. Here, the authors used the modified Delphi method to identify areas of consensus regarding the modality, frequency, and duration of hydrocephalus surveillance following surgical treatment. METHODS Pediatric neurosurgeons serving as institutional liaisons to the Hydrocephalus Clinical Research Network (HCRN), or its implementation/quality improvement arm (HCRNq), were invited to participate in this modified Delphi study. Thirty-seven consensus statements were generated and distributed via an anonymous electronic survey, with responses structured as a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree). A subsequent, virtual meeting offered the opportunity for open discussion and modification of the statements in an effort to reach consensus (defined as ≥ 80% agreement or disagreement). RESULTS Nineteen pediatric neurosurgeons participated in the first round, after which 15 statements reached consensus. During the second round, 14 participants met virtually for review and discussion. Some statements were modified and 2 statements were combined, resulting in a total of 36 statements. At the conclusion of the session, consensus was achieved for 17 statements regarding the following: 1) the role of standardization; 2) preferred imaging modalities; 3) postoperative follow-up after shunt surgery (subdivided into immediate postoperative imaging, delayed postoperative imaging, routine clinical surveillance, and routine radiological surveillance); and 4) postoperative follow-up after an endoscopic third ventriculostomy. Consensus could not be achieved for 19 statements. CONCLUSIONS Using the modified Delphi method, 17 consensus statements were developed with respect to both clinical and radiological follow-up after a shunt or endoscopic third ventriculostomy. The frequency, modality, and duration of surveillance were addressed, highlighting areas in which no clear data exist to guide clinical practice. Although further studies are needed to evaluate the clinical utility and cost-effectiveness of hydrocephalus surveillance, the current study provides a framework to guide future efforts to develop standardized clinical protocols for the postoperative surveillance of patients with hydrocephalus. Ultimately, the standardization of hydrocephalus surveillance has the potential to improve patient care as well as optimize the use of healthcare resources.

OBJECTIVEThe primary objective of this study was to use the prospective Hydrocephalus Clinical Research Network (HCRN) registry to determine clinical predictors of fast time to shunt failure (≤ 30 days from last revision) and ultrafast time to failure (≤ 7 days from last revision).METHODSRevisions (including those due to infection) to permanent shunt placements that occurred between April 2008 and November 2017 for patients whose entire shunt experience was recorded in the registry were analyzed. All registry data provided at the time of initial shunt placement and subsequent revision were reviewed. Key variables analyzed included etiology of hydrocephalus, age at time of initial shunt placement, presence of slit ventricles on imaging at revision, whether the ventricles were enlarged at the time of revision, and presence of prior fast failure events. Univariable and multivariable analyses were performed to find key predictors of fast and ultrafast failure events.RESULTSA cohort of 1030 patients with initial shunt insertions experienced a total of 1995 revisions. Of the 1978 revision events with complete records, 1216 (61.5%) shunts remained functional for more than 1 year, and 762 (38.5%) failed within 1 year of the procedure date. Of those that failed within 1 year, 423 (55.5%) failed slowly (31–365 days) and 339 (44.5%) failed fast (≤ 30 days). Of the fast failures, 131 (38.6%) were ultrafast (≤ 7 days). In the multivariable analysis specified a priori, etiology of hydrocephalus (p = 0.005) and previous failure history (p = 0.011) were independently associated with fast failure. Age at time of procedure (p = 0.042) and etiology of hydrocephalus (p = 0.004) were independently associated with ultrafast failure. These relationships in both a priori models were supported by the data-driven multivariable models as well.CONCLUSIONSNeither the presence of slit ventricle syndrome nor ventricular enlargement at the time of shunt failure appears to be a significant predictor of repeated, rapid shunt revisions. Age at the time of procedure, etiology of hydrocephalus, and the history of previous failure events seem to be important predictors of fast and ultrafast shunt failure. Further work is required to understand the mechanisms of these risk factors as well as mitigation strategies.

OBJECTIVEFew studies have addressed surgical resource utilization—surgical revisions and associated hospital admission days—following shunt insertion or endoscopic third ventriculostomy (ETV) with or without choroid plexus cauterization (CPC) for CSF diversion in hydrocephalus. Study members of the Hydrocephalus Clinical Research Network (HCRN) investigated differences in surgical resource utilization between CSF diversion strategies in hydrocephalus in infants.METHODSPatients up to corrected age 24 months undergoing initial definitive treatment of hydrocephalus were reviewed from the prospectively maintained HCRN Core Data Project (Hydrocephalus Registry). Postoperative courses (at 1, 3, and 5 years) were studied for hydrocephalus-related surgeries (primary outcome) and hospital admission days related to surgical revision (secondary outcome). Data were summarized using descriptive statistics and compared using negative binomial regression, controlling for age, hydrocephalus etiology, and HCRN center. The study population was organized into 3 groups (ETV alone, ETV with CPC, and CSF shunt insertion) during the 1st postoperative year and 2 groups (ETV alone and CSF shunt insertion) during subsequent years due to limited long-term follow-up data.RESULTSAmong 1090 patients, the majority underwent CSF shunt insertion (CSF shunt, 83.5%; ETV with CPC, 10.0%; and ETV alone, 6.5%). Patients undergoing ETV with CPC had a higher mean number of revision surgeries (1.2 ± 1.6) than those undergoing ETV alone (0.6 ± 0.8) or CSF shunt insertion (0.7 ± 1.3) over the 1st year after surgery (p = 0.005). At long-term follow-up, patients undergoing ETV alone experienced a nonsignificant lower mean number of revision surgeries (0.7 ± 0.9 at 3 years and 0.8 ± 1.3 at 5 years) than those undergoing CSF shunt insertion (1.1 ± 1.9 at 3 years and 1.4 ± 2.6 at 5 years) and exhibited a lower mean number of hospital admission days related to revision surgery (3.8 ± 10.3 vs 9.9 ± 27.0, p = 0.042).CONCLUSIONSAmong initial treatment strategies for hydrocephalus, ETV with CPC yielded a higher surgical revision rate within 1 year after surgery. Patients undergoing ETV alone exhibited a nonsignificant lower mean number of surgical revisions than CSF shunt insertion at 3 and 5 years postoperatively. Additionally, the ETV-alone cohort demonstrated significantly fewer hospital admission days related to surgical management of hydrocephalus within 3 years after surgery. These findings suggest a time-dependent benefit of ETV over CSF shunt insertion regarding surgical resource utilization.

OBJECTIVE Two previous Hydrocephalus Clinical Research Network (HCRN) studies have demonstrated that compliance with a standardized CSF shunt infection protocol reduces shunt infections. In this third iteration, a simplified protocol consisting of 5 steps was implemented. This analysis provides an updated evaluation of protocol compliance and evaluates modifiable shunt infection risk factors. METHODS The new simplified protocol was implemented at HCRN centers on November 1, 2016, for all shunt procedures, excluding external ventricular drains, ventricular reservoirs, and subgaleal shunts. Procedures performed through December 31, 2019, were included (38 months). Compliance with the protocol, use of antibiotic-impregnated catheters (AICs), and other variables of interest were collected at the index operation. Outcome events for a minimum of 6 months postoperatively were recorded. The definition of infection was unchanged from the authors’ previous report. RESULTS A total of 4913 procedures were performed at 13 HCRN centers. The overall infection rate was 5.1%. Surgeons were compliant with all 5 steps of the protocol in 79.4% of procedures. The infection rate for the protocol alone was 8.1% and dropped to 4.9% when AICs were added. Multivariate analysis identified having ≥ 2 complex chronic conditions (odds ratio [OR] 1.76, 95% confidence interval [CI] 1.26–2.44, p = 0.01) and a history of prior shunt surgery within 12 weeks (OR 1.84, 95% CI 1.37–2.47, p < 0.01) as independent risk factors for shunt infection. The use of AICs (OR 0.70, 95% CI 0.50–0.97, p = 0.05) and vancomycin irrigation (OR 0.36, 95% CI 0.21–0.62, p < 0.01) were identified as independent factors protective against shunt infection. CONCLUSIONS The authors report the third iteration of their quality improvement protocol to reduce the risk of shunt infection. Compliance with the protocol was high. These updated data suggest that the incorporation of AICs is an important, modifiable infection prevention measure. Vancomycin irrigation was also identified as a protective factor but requires further study to better understand its role in preventing shunt infection.

OBJECTIVE Primary treatment of hydrocephalus with endoscopic third ventriculostomy (ETV) and choroid plexus cauterization (CPC) is well described in the neurosurgical literature, with wide reported ranges of success and complication rates. The purpose of this study was to describe the safety and efficacy of ETV revision after initial ETV+CPC failure. METHODS Prospectively collected data in the Hydrocephalus Clinical Research Network Core Data Project registry were reviewed. Children who underwent ETV+CPC as the initial treatment for hydrocephalus between 2013 and 2019 and in whom the initial ETV+CPC was completed (i.e., not abandoned) were included. Log-rank survival analysis (the primary analysis) was used to compare time to failure (defined as any other surgical treatment for hydrocephalus or death related to hydrocephalus) of initial ETV+CPC versus that of ETV revision by using random-effects modeling to account for the inclusion of patients in both the initial and revision groups. Secondary analysis compared ETV revision to shunt placement after failure of initial ETV+CPC by using the log-rank test, as well as shunt failure after ETV+CPC to that after ETV revision. Cox regression analysis was used to identify predictors of failure among children treated with ETV revision. RESULTS The authors identified 521 ETV+CPC procedures that met their inclusion criteria. Ninety-one children underwent ETV revision after ETV+CPC failure. ETV revision had a lower 1-year success rate than initial ETV+CPC (29.5% vs 45%, p < 0.001). ETV revision after initial ETV+CPC failure had a lower success rate than shunting (29.5% vs 77.8%, p < 0.001). Shunt survival after initial ETV+CPC failure was not significantly different from shunt survival after ETV revision failure (p = 0.963). Complication rates were similar for all examined surgical procedures (initial ETV+CPC, ETV revision, ventriculoperitoneal shunt [VPS] placement after ETV+CPC, and VPS placement after ETV revision). Only young age was predictive of ETV revision failure (p = 0.02). CONCLUSIONS ETV revision had a significantly lower 1-year success rate than initial ETV+CPC and VPS placement after ETV+CPC. Complication rates were similar for all studied procedures. Younger age, but not time since initial ETV+CPC, was a risk factor for ETV revision failure.

Preterm infants commonly present with a hemodynamically significant patent ductus arteriosus (hsPDA). The authors describe the case of a preterm infant with posthemorrhagic ventricular dilation, which resolved in a temporally coincident fashion to repair of hsPDA. The presence of a PDA with left-to-right shunting was confirmed at birth on echocardiogram and was unresponsive to repeated medical intervention. Initial cranial ultrasound revealed periventricular-intraventricular hemorrhage. Follow-up serial ultrasound showed resolving intraventricular hemorrhage and progressive bilateral hydrocephalus. At 5 weeks, the ductus was ligated with the goal of improving hemodynamic stability prior to CSF diversion. However, neurosurgical intervention was not required due to improvement of ventriculomegaly occurring immediately after PDA ligation. No further ventricular dilation was observed at the 6-month follow-up.Systemic venous flow disruption and abnormal patterns of cerebral blood circulation have been previously associated with hsPDA. Systemic hemodynamic change has been reported to follow hsPDA ligation, although association with ventricular normalization has not. This case suggests that the unstable hemodynamic environment due to left-to-right shunting may also impede CSF outflow and contribute to ventriculomegaly. The authors review the literature surrounding pressure transmission between a PDA and the cerebral vessels and present a mechanism by which PDA may contribute to posthemorrhagic ventricular dilation.

OBJECTIVEPediatric neurosurgery is a core component of neurosurgical residency training. Pediatric case minimums are established by the Neurosurgery Residency Review Committee of the Accreditation Council for Graduate Medical Education (ACGME). Case minimums, by themselves, allow for great variability in training between programs. There are no prior data on how the residency programs meet these requirements. The authors’ objective was to gather information on pediatric neurosurgical education among the ACGME-accredited neurosurgery training programs in order to shape further pediatric neurosurgical educational efforts.METHODSA 25-question survey about pediatric neurosurgical education was created by the Education Committee of the Section on Pediatric Neurological Surgery of the American Association of Neurological Surgeons/Congress of Neurological Surgeons and distributed to program directors of all 111 ACGME-accredited neurosurgery training programs.RESULTSThe response rate was 77% (86/111). In 55% of programs the residents are rotated to a responder-designated “freestanding” children’s hospital, and 39% of programs rotate residents to a children’s hospital within a larger adult hospital or a general hospital. There are 4 or fewer pediatric neurosurgical faculty in 91% of programs. In 12% of programs less than 100 cases are performed per year, and in 45% more than 500 are performed. In 31% of responding neurosurgery residency programs there is also a pediatric neurosurgery fellowship program supported by the same sponsoring institution. Seventy-seven percent of programs have at least one specific pediatric neurosurgery rotation, with 71% of those rotations occurring during postgraduate year 3 and 50% occurring during postgraduate year 4. The duration of pediatric rotation varies from no specific rotation to more than 1 year, with 48% of residents spending 4–6 months on a pediatric rotation and 12% spending 7–11 months. Last, 17% of programs send their residents to external sites sponsoring other residency programs for their pediatric rotation.CONCLUSIONSThere is great variety between neurosurgery training programs with regard to resident education in pediatric neurosurgery. This study’s data will serve as a baseline for future studies, and the authors hope the findings will guide further efforts in pediatric neurosurgical education in residency training programs.

Diffuse leptomeningeal glioneuronal tumors (DLGNTs) are newly recognized as an entity in the 2016 revision of the WHO Classification of tumors of the central nervous system. They typically present as diffuse leptomeningeal infiltrates along the neuraxis with focal and superficial involvement of the parenchyma. Here, we report a DLGNT with unusual radiological and histological features. A 13-year-old girl presented with scoliosis and back pain. Magnetic resonance imaging demonstrated a syrinx from C2 to T11 and an intramedullary mass from T6 to T9-10. No leptomeningeal involvement was recognized. Histological examination of the gross total resection specimen revealed a low-grade neuroepithelial neoplasm predominantly infiltrating the spinal cord and only focally involving the leptomeninges. Chromosome microarray identified co-deletion of the short arm of chromosome 1 and the long arm of chromosome 19 as well as fusion of the KIAA1549 and BRAF genes. Next-generation sequencing demonstrated wild-type alleles at the mutational hotspots of IDH1 (R132) and IDH2 (R140 and R172). In contrast to most reported DLGNTs, the tumor described in this manuscript was characterized by a predominant parenchymal component and only minor leptomeningeal involvement both radiographically and histologically. Our case, therefore, expands the spectrum of radiological and histopathological features of this new entity. It also highlights the critical role of molecular genetic testing in establishing the diagnosis of DLGNT in unusual cases.

Background/Aims: Down syndrome is the most common inherited disorder. Some patients develop atlantoaxial instability. Existing screening guidelines were developed prior to availability of MRI. We present predictors for deficit using dynamic MRI of the craniocervical junction. Methods: A retrospective review of Down syndrome patients from 2001 to 2015 was carried out. Patients were considered symptomatic if they had clinical deficits or signal change on MRI. Measurements were taken at the atlantoaxial junction and structural abnormalities noted. Analysis was performed with SPSS. Results: A total of 36 patients were included. Patients averaged 93 months of age with a follow-up of 57 months. No asymptomatic patients developed myelopathy during follow-up. During dynamic imaging, symptomatic patients had greater changes in space available for the cord (SAC) (5.2 vs. 2.7 mm; p < 0.001) and atlantodental interval (ADI) (2.8 vs. 1.3 mm; p = 0.04). These patients were also more likely to have a bony anomaly (50 vs. 13%; p = 0.03). Conclusion: This study characterizes the range of motion seen on dynamic MRI and provides parameters that can be used to distinguish patients at risk for neurologic injury. Changes greater than 3 mm in ADI or 5 mm in SAC during dynamic MRI or any bony abnormality warrants further investigation. Patients without these features may be able to avoid an unnecessary intervention.

Introduction: Pilocytic astrocytomas (PA) are a common, benign childhood tumor known for their slow growth rates and excellent prognosis. The aim of our study was to characterize patient, tumor, and imaging-related risk factors for recurrence and progression of disease. Methods: We identified 116 patients with PA who underwent surgery at our institution between 2000 and 2015. Data were collected retrospectively from the clinical charts. Results: The mean age at resection was 7 ± 5 years (range 0.5–31) and mean follow-up was 6 ± 3 years. Initial resection was complete in 33 patients (29%), subtotal in 78 patients (67%), and biopsy in 5 patients (4%). A total of 45/116 (40%) patients experienced either recurrence or progression after initial resection with a mean time to recurrence or progression of 2.2 years. Bivariate analysis identified subtotal resection, tumor location, age at diagnosis, and imaging features (i.e., T2 invasion, exophytic component, hemorrhage, and solid tumors) as factors significantly associated with recurrence or progression (p < 0.05). Conversely, PAs that were completely resected, predominately cystic, and located in the cerebellum were significantly associated with no recurrence or progression (p < 0.05). Multivariate regression analysis narrowed down 4 robust risk factors: extent of resection, T2 invasion, predominantly solid lesions, and presence of an exophytic component (p < 0.05). Conclusion: Total surgical removal of PA has been the most important prognostic factor for the clinical course of PA. Our study reveals additional risk factors for the recurrence or progression of disease: tumor invasion, solid composition, and tumors with an exophytic component.

Background/Aims: Intracranial germ-cell tumors (GCTs) are a heterogeneous group of tumors that vary in their response to treatment. Standard treatment consists of chemotherapy and radiation therapy, with the consideration of second-look surgery in resistant disease. The present study aims to inform therapy by characterizing features on pretreatment imaging associated with recurrence. Methods: Children with intracranial GCTs treated at a single institution between January 2000 and October 2016 were retrospectively reviewed under an Institutional Review Board-approved protocol. Imaging variables identified on pretreatment imaging were calcifications, cysts, heterogeneity of enhancement, blood products, hydrocephalus, gradient echo susceptibility, restricted diffusion, invasiveness, and extent of edema. Tumor recurrence was used as the primary outcome variable. Results and Conclusion: Fifty-two patients (39 males, mean age at diagnosis: 13 ± 5 years, 34 germinoma, 18 nongerminomatous GCT [NGGCT]) were reviewed. Thirty-three percent of the patients reviewed had recurrence (7 germinoma, 11 NGGCT). Recurrence was associated with invasiveness as seen on preoperative imaging (p = 0.0385) and cystic tumor (p = 0.048).

OBJECTIVE Treating Dandy-Walker syndrome–related hydrocephalus (DWSH) involves either a CSF shunt-based or endoscopic third ventriculostomy (ETV)–based procedure. However, comparative investigations are lacking. This study aimed to compare shunt-based and ETV-based treatment strategies utilizing archival data from the Hydrocephalus Clinical Research Network (HCRN) registry. METHODS A retrospective review of prospectively collected and maintained data on children with DWSH, available from the HCRN registry (14 sites, 2008–2018), was performed. The primary outcome was revision-free survival of the initial surgical intervention. The primary exposure was either shunt-based (i.e., cystoperitoneal shunt [CPS], ventriculoperitoneal shunt [VPS], and/or dual-compartment) or ETV-based (i.e., ETV alone or with choroid plexus cauterization [CPC]) initial surgical treatment. Primary analysis included multivariable Cox proportional hazards models. RESULTS Of 8400 HCRN patients, 151 (1.8%) had DWSH. Among these, the 102 patients who underwent shunt placement (79 VPSs, 16 CPSs, 3 other, and 4 multiple proximal catheter) were younger (6.6 vs 18.8 months, p < 0.001) and more frequently had 1 or more comorbidities (37.3% vs 14.3%, p = 0.005) than the 49 ETV-treated children (28 ETV-CPC). Fifty percent of the shunt-based and 51% of the ETV-based treatments failed. Notably, 100% (4/4) of the dual-compartment shunts failed. Adjusting for age, baseline ventricular size, and comorbidities, ETV-based treatment was not significantly associated with earlier failure compared with shunt-based treatment (HR for failure 1.32, 95% CI 0.77–2.26; p = 0.321). Complication rates were low: 4.9% and 6.1% (p = 0.715) for shunt- and ETV-based procedures, respectively. There was no difference in survival between ETV-CPC– and ETV-based treatment when adjusting for age (HR for failure 0.86, 95% CI 0.29–2.55, p = 0.783). CONCLUSIONS In this North American, multicenter, prospective database review, shunt-based and ETV-based primary treatment strategies of DWSH appear similarly durable. Pediatric neurosurgeons can reasonably consider ETV-based initial treatment given the similar durability and the low complication rate. However, given the observational nature of this study, the treating surgeon might need to consider subgroups that were too small for a separate analysis. Very young children with comorbidities were more commonly treated with shunts, and older children with fewer comorbidities were offered ETV-based treatment. Future studies may determine preoperative characteristics associated with ETV treatment success in this population.

OBJECTIVE Cerebrospinal fluid diversion via ventricular shunting is a common surgical treatment for hydrocephalus in the pediatric population. No longitudinal follow-up data for a multistate population-based cohort of pediatric patients undergoing ventricular shunting in the United States have been published. In the current review of a nationwide population-based data set, the authors aimed to assess rates of shunt failure and hospital readmission in pediatric patients undergoing new ventricular shunt placement. They also review patient- and hospital-level factors associated with shunt failure and readmission. METHODS Included in this study was a population-based sample of pediatric patients with hydrocephalus who, in 2010–2014, had undergone new ventricular shunt placement and had sufficient follow-up, as recorded in the Nationwide Readmissions Database. The authors analyzed the rate of revision within 6 months, readmission rates at 30 and 90 days, and potential factors associated with shunt failure including patient- and hospital-level variables and type of hydrocephalus. RESULTS A total of 3520 pediatric patients had undergone initial ventriculoperitoneal shunt placement for hydrocephalus at an index admission. Twenty percent of these patients underwent shunt revision within 6 months. The median time to revision was 44.5 days. Eighteen percent of the patients were readmitted within 30 days and 31% were readmitted within 90 days. Different-hospital readmissions were rare, occurring in ≤ 6% of readmissions. Increased hospital volume was not protective against readmission or shunt revision. Patients with grade 3 or 4 intraventricular hemorrhage were more likely to have shunt malfunctions. Patients who had private insurance and who were treated at a large hospital were less likely to be readmitted. CONCLUSIONS In a nationwide, population-based database with longitudinal follow-up, shunt failure and readmission were common. Although patient and hospital factors were associated with readmission and shunt failure, system-wide phenomena such as insufficient centralization of care and fragmentation of care were not observed. Efforts to reduce readmissions in pediatric patients undergoing ventricular shunt procedures should focus on coordinating care in patients with complex neurological diseases and on reducing healthcare disparities associated with readmission.

OBJECTIVE Scoliosis is common in patients with Chiari malformation type I (CM-I)–associated syringomyelia. While it is known that treatment with posterior fossa decompression (PFD) may reduce the progression of scoliosis, it is unknown if decompression with duraplasty is superior to extradural decompression. METHODS A large multicenter retrospective and prospective registry of 1257 pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for patients with scoliosis who underwent PFD with or without duraplasty. RESULTS In total, 422 patients who underwent PFD had a clinical diagnosis of scoliosis. Of these patients, 346 underwent duraplasty, 51 received extradural decompression alone, and 25 were excluded because no data were available on the type of PFD. The mean clinical follow-up was 2.6 years. Overall, there was no difference in subsequent occurrence of fusion or proportion of patients with curve progression between those with and those without a duraplasty. However, after controlling for age, sex, preoperative curve magnitude, syrinx length, syrinx width, and holocord syrinx, extradural decompression was associated with curve progression > 10°, but not increased occurrence of fusion. Older age at PFD and larger preoperative curve magnitude were independently associated with subsequent occurrence of fusion. Greater syrinx reduction after PFD of either type was associated with decreased occurrence of fusion. CONCLUSIONS In patients with CM-I, syrinx, and scoliosis undergoing PFD, there was no difference in subsequent occurrence of surgical correction of scoliosis between those receiving a duraplasty and those with an extradural decompression. However, after controlling for preoperative factors including age, syrinx characteristics, and curve magnitude, patients treated with duraplasty were less likely to have curve progression than patients treated with extradural decompression. Further study is needed to evaluate the role of duraplasty in curve stabilization after PFD.

OBJECTIVEAlthough current pediatric neurosurgery guidelines encourage the treatment of pediatric malignant brain tumors at specialized centers such as pediatric hospitals, there are limited data in support of this recommendation. Previous studies suggest that children treated by higher-volume surgeons and higher-volume hospitals may have better outcomes, but the effect of treatment at dedicated children’s hospitals has not been investigated.METHODSThe authors analyzed the Healthcare Cost and Utilization Project Kids’ Inpatient Database (KID) from 2000–2009 and included all patients undergoing a craniotomy for malignant pediatric brain tumors based on ICD-9-CM codes. They investigated the effects of patient demographics, tumor location, admission type, and hospital factors on rates of routine discharge and mortality.RESULTSFrom 2000 through 2009, 83.6% of patients had routine discharges, and the in-hospital mortality rate was 1.3%. In multivariate analysis, compared with children treated at an institution designated as a pediatric hospital by NACHRI (National Association of Children’s Hospitals and Related Institutions), children receiving treatment at a pediatric unit within an adult hospital (OR 0.5, p < 0.01) or a general hospital without a designated pediatric unit (OR 0.4, p < 0.01) were less likely to have routine discharges. Treatment at a large hospital (> 400 beds; OR 1.8, p = 0.02) and treatment at a teaching hospital (OR 1.7, p = 0.02) were independently associated with greater likelihood of routine discharge. However, patients transferred between facilities had a significantly decreased likelihood of routine discharge (OR 0.5, p < 0.01) and an increased likelihood of mortality (OR 5.0, p < 0.01). Procedural volume was not associated with rate of routine discharge or mortality.CONCLUSIONSThese findings may have implications for planning systems of care for pediatric patients with malignant brain tumors. The authors hope to motivate future research into the specific factors that may lead to improved outcomes at designated pediatric hospitals.

The optimal time to closure of a newborn with an open neural tube defect (NTD–myelomeningocele) has been the subject of a number of investigations. One aspect of timing that has received attention is its relationship to repair site and central nervous system (CNS) infection that can lead to irreversible deficits and prolonged hospital stays. No studies have evaluated infection as a function of surgical timing at a national level. We hypothesized an increase in wound infection in those patients with delays in myelomeningocele repair when evaluated in both a single-center and national database. Treatment outcomes following documented times to transfer and closure were evaluated at Children’s Hospital of Los Angeles (CHLA) for the years 2004 to 2014. Data of newborns with a myelomeningocele with varying time to repair were also obtained from non-overlapping abstracts of the 2000–2010 Kids’ Inpatient Database (KID) and Nationwide Inpatient Sample (NIS). Poisson multivariable regression analyses were used to assess the effect of time to repair on infection and time to discharge. At CHLA, 95 neonates who underwent myelomeningocele repair were identified, with a median time from birth to treatment of 1 day. Six (6 %) patients were noted to have postrepair complications. CHLA data was not sufficiently powered to detect a difference in infection following delay in closure. In the NIS, we identified 3775 neonates with repaired myelomeningocele of whom infection was reported in 681 (18 %) patients. There was no significant difference in rates of infection between same-day and 1-day wait times (p = 0.22). Wait times of two (RR = 1.65 [1.23, 2.22], p

OBJECT Posthemorrhagic hydrocephalus (PHH) in the preterm infant remains a major neurological complication of prematurity. The authors first described insertion of a specially designed low-profile subcutaneous ventricular catheter reservoir for temporary management of hydrocephalus in 1983. This report presents the follow-up experience with the surgical management of PHH in this population and describes outcomes both in infants who were stable for permanent shunt insertion and those initially temporized with a ventricular reservoir (VR) prior to permanent ventriculoperitoneal (VP)/ventriculoatrial (VA) shunt placement. METHODS A retrospective review was undertaken of the medical records of all premature infants surgically treated for posthemorrhagic hydrocephalus (PHH) between 1997 and 2012 at Children’s Hospital Los Angeles. RESULTS Over 14 years, 91 preterm infants with PHH were identified. Fifty neonates received temporizing measures via a VR that was serially tapped for varying time periods. For the remaining 41 premature infants, VP/VA shunt placement was the first procedure. Patients with a temporizing measure as their initial procedure had undergone CSF diversion significantly earlier in life than those who had permanent shunting as the initial procedure (29 vs 56 days after birth, p < 0.01). Of the infants with a VR as their initial procedure, 5/50 (10%) did not undergo subsequent VP/VA shunt placement. The number of shunt revisions and the rates of loculated hydrocephalus and shunt infection did not statistically differ between the 2 groups. CONCLUSIONS Patients with initial VR insertion as a temporizing measure received a CSF diversion procedure significantly earlier than those who received a permanent shunt as their initial procedure. Otherwise, the outcomes with regard to shunt revisions, loculated hydrocephalus, and shunt infection were not different for the 2 groups.

OBJECTIVEComplications after complex tethered spinal cord (cTSC) surgery include infections and cerebrospinal fluid (CSF) leaks. With little empirical evidence to guide management, there is variability in the interventions undertaken to limit complications. Expert-based best practices may improve the care of patients undergoing cTSC surgery. Here, authors conducted a study to identify consensus-driven best practices.METHODSThe Delphi method was employed to identify consensual best practices. A literature review regarding cTSC surgery together with a survey of current practices was distributed to 17 board-certified pediatric neurosurgeons. Thirty statements were then formulated and distributed to the group. Results of the second survey were discussed during an in-person meeting leading to further consensus, which was defined as ≥ 80% agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree).RESULTSSeventeen consensus-driven best practices were identified, with all participants willing to incorporate them into their practice. There were four preoperative interventions: (1, 2) asymptomatic AND symptomatic patients should be referred to urology preoperatively, (3, 4) routine preoperative urine cultures are not necessary for asymptomatic AND symptomatic patients. There were nine intraoperative interventions: (5) patients should receive perioperative cefazolin or an equivalent alternative in the event of allergy, (6) chlorhexidine-based skin preparation is the preferred regimen, (7) saline irrigation should be used intermittently throughout the case, (8) antibiotic-containing irrigation should be used following dural closure, (9) a nonlocking running suture technique should be used for dural closure, (10) dural graft overlay should be used when unable to obtain primary dural closure, (11) an expansile dural graft should be incorporated in cases of lipomyelomeningocele in which primary dural closure does not permit free flow of CSF, (12) paraxial muscles should be closed as a layer separate from the fascia, (13) routine placement of postoperative drains is not necessary. There were three postoperative interventions: (14) postoperative antibiotics are an option and, if given, should be discontinued within 24 hours; (15) patients should remain flat for at least 24 hours postoperatively; (16) routine use of abdominal binders or other compressive devices postoperatively is not necessary. One intervention was prioritized for additional study: (17) further study of additional gram-negative perioperative coverage is needed.CONCLUSIONSA modified Delphi technique was used to develop consensus-driven best practices for decreasing wound complications after cTSC surgery. Further study is required to determine if implementation of these practices will lead to reduced complications. Discussion through the course of this study resulted in the initiation of a multicenter study of gram-negative surgical site infections in cTSC surgery.

OBJECT Racial and socioeconomic disparities within the US health care system are a growing concern. Despite extensive research and efforts to narrow such disparities, minorities and economically disadvantaged patients continue to exhibit inferior health care outcomes. Disparities in the delivery of pediatric neurosurgical care are understudied. Authors of this study examine the impact of race and socioeconomic status on outcomes following pediatric CSF shunting procedures. METHODS Discharge information from the 2000, 2003, 2006, and 2009 Kids' Inpatient Database for individuals (age < 21 years) with a diagnosis of hydrocephalus who had undergone CSF shunting procedures was abstracted for analysis. Multivariate logistic regression analyses, adjusting for patient and hospital factors and annual CSF shunt procedure volume, were performed to evaluate the effects of race and payer status on the likelihood of inpatient mortality and nonroutine hospital discharge (that is, not to home). RESULTS African American patients (p < 0.05) had an increased likelihood of inpatient death and nonroutine discharge compared with white patients. Furthermore, Medicaid patients had a significantly higher likelihood of nonroutine discharge (p < 0.05) as compared with privately insured patients. CONCLUSIONS Findings in this study, which utilized US population-level data, suggest the presence of racial and socioeconomic status outcome disparities following pediatric CSF shunting procedures. Further studies on health disparities in this population are warranted.

Object Cerebrospinal fluid–diverting shunts are often complicated by bacterial infections. Dental procedures are known to cause transient bacteremia that could potentially spread hematogenously to these implanted devices. No literature currently exists to inform practitioners as to the need for prophylactic antibiotics for patients who possess these implants. The authors performed a retrospective study to assess whether dental procedures and poor oral health were associated with a higher likelihood of developing CSF-diverting shunt infections. Methods Neurosurgical and pediatric dental records from January 2007 to December 2012 were reviewed for shunt surgeries and dental encounters. Indications for shunt surgery and infection rates were recorded. Dental records were reviewed for several markers of overall dental health, such as a DMFT (decayed, missing, and filled teeth) score and a gingival health/oral hygiene score. The association between these scores and the incidence of shunt infections were studied. Moreover, the relationship between the incidence of shunt infections and the timing and invasiveness of preceding dental encounters were analyzed. Results A total of 100 pediatric patients were included in our study, for a total of 204 shunt surgeries. Twenty-one shunt infections were noted during the 6-year study period. Five of these shunts infections occurred within 3 months of a dental procedure. The odds ratio (OR) of developing a shunt infection within 3 months of a dental procedure was 0.98 (95% confidence interval [CI] 0.27–3.01), and was not statistically significant. The OR of developing a shunt infection after a high-risk dental procedure compared with a low-risk dental procedure was 1.32 (95% CI 0.02–16.29), and was not statistically significant. There was no significant association between measures of dental health, such as DMFT and gingival health score, and the likelihood of developing a shunt infection. The ORs for these 2 scores were 0.51 (95% CI 0.04–4.96) and 1.58 (95% CI 0.03–20.06), respectively. The study was limited by sample size. Conclusions Dental health status and the number and type of dental procedures performed do not appear to confer a higher risk of developing a CSF-diverting shunt infection in this pediatric population.

Object Most CSF-diverting shunt systems have an access port that can be percutaneously tapped. Tapping the shunt can yield valuable information as to its function and whether an infection is present. The fear of causing a shunt infection by tapping may limit the physician's willingness to do so. The authors of this study investigate the risk of infecting a shunt secondary to percutaneous tapping. Methods Following institutional review board approval, CSF specimens obtained from tapping an indwelling CSF-diverting shunt during the 2011 and 2012 calendar years were identified and matched with clinical information. A culture-positive CSF sample was defined as an infection. If results were equivocal, such as a broth-only–positive culture, a repeat CSF specimen was examined. The CSF was obtained by tapping the shunt access port with a 25-gauge butterfly needle after prepping the unshaven skin with chlorhexidine. Results During the study period, 266 children underwent 542 shunt taps. With 541 taps, no clinical evidence of a subsequent shunt infection was found. One child's CSF went from sterile to infected 11 days later; however, this patient had redness along the shunt tract at the time of the initial sterile tap. Conclusions The risk of infection from tapping a shunt is remote if the procedure is done correctly.

Tumors of the spine in children are rare, and further clinical description is necessary. This study investigated epidemiology, interventions, and outcomes of pediatric patients with spine and spinal cord tumors. The National Inpatient Sample and Kids’ Inpatient Database were used for the study. Outcomes were studied, and bivariate significant trends were analyzed in a multivariate setting. Analysis of 2870 patients between 2000 and 2009 found a median age of diagnosis of 11 years (Tables 1 and 2). Most were white (65.2%) and had private insurance (62.3%), and 46.8% of procedures were emergent operations. Treatment occurred at teaching (93.6%) and non-children’s hospitals (81.1%). Overall mortality rate was 1.7%, non-routine discharges occurred at a rate 19.9%, complications at 21.1%, and average total charges were $66,087. A majority of patients (87.5%) had no intervention, and of those patients receiving treatment, 78.2% underwent surgery and 23.1% had radiotherapy. Treatment with surgery alone increased significantly over time (p

Management of patients with central nervous system tumors relies largely on magnetic resonance imaging scans to document disease progression or recurrence. The finding of new lesions always presents the challenge of differentiating between post-surgical changes, radiation necrosis, gliosis, and tumor, submitting these patients to more aggressive therapy and more toxicity. We reviewed the medical records of three patients with primary central nervous system tumors treated at the Children’s Hospital Los Angeles who had new false-positive magnetic resonance imaging findings suggestive of tumor recurrence. All of them had complete total resection of primary tumor, had received involved-field radiation therapy, had biopsies confirming absence of viable tumor, and all three patients are long-term survivors. These cases exemplify that not everything that enhances on brain or spine magnetic resonance imaging is viable tumor, and a biopsy should always be considered in the decision-making process in evaluation of potentially recurrent central nervous system tumors in pediatric patients. A step-wise approach for such challenging cases is presented in this article.

Object The goal of this study was to review all cases of pediatric spinal arachnoid cysts (SACs) surgically treated at the authors' institution between 1992 and 2008 and to compare these cases to the published literature for the general population. Methods The charts of all pediatric patients with SACs were reviewed for demographics, medical history, presenting symptoms, imaging findings, operative procedure(s), complications, and outcomes. Following a complete literature review, the pediatric data were compared with data from the general population and unique findings associated with pediatric patients were identified. Results Thirty-one pediatric patients (median age 6.9 years) underwent operative intervention for SACs between 1992 and 2008 (median duration of follow-up 4.2 years). There were 17 female patients (55%) and 14 male patients (45%). Twenty-one patients (68%) presented with symptoms of radiculopathy or myelopathy. The most common presenting symptoms were pain (42%), lower-extremity weakness (39%), gait instability (32%), spasticity (19%), sensory loss (10%), and bladder dysfunction (7%). In 3 patients (10%) SACs were incidental findings. Intradural SACs were more common (18 patients, 58%) than extradural SACs (11 patients, 36%). One patient (3%) had extradural and intradural components. One patient (3%) had a purely intramedullary cyst, and 1 patient (3%) had both an intradural and intramedullary component. Of the 18 intradural SACs, 9 (50%) were located ventral to the spinal cord and 9 (50%) were dorsally situated. One dorsal intradural SAC had an intramedullary component. All extradural SACs were located dorsal to the spinal cord. Intradural SACs were primarily concentrated in the cervical and thoracic regions (67%), whereas extradural cysts were more evenly distributed between the thoracic, lumbar, and sacral regions. Of the 18 patients with intradural SACs, 13 (72%) had significant previous CNS abnormalities, compared with 3 (27%) of 11 patients with extradural SACs. There were 2 operative complications. One patient had a CSF leak treated with a lumbar drain, and the second patient had a pseudomeningocele. No patients had neurological deterioration as a result of surgical intervention. Twenty-one patients (68%) had complete remission of symptoms, 6 (19%) had improvement, 3 (10%) were stable, and 1 (3%) has worsening of symptoms with recurrence that ultimately required cystoperitoneal shunting, despite multiple failed attempts at fenestration. Conclusions Spinal arachnoid cysts are rare lesions in the pediatric population. Affected patients present with back pain, weakness, and/or gait instability. In children, SACs predominantly develop in the thoracic region and are more likely to occur intradurally, compared with SACs in the general population. Overall outcomes following surgical fenestration or excision of SACs are excellent, with complete remission or improvement of symptoms achieved in 87% of cases.

The posterior fossa syndrome (PFS) is common after cerebellar tumor resection in pediatric patients. It is characterized by postoperative mutism and ataxia and associated with persistent abnormalities in mood and cognition. Method: A 2-year prospective study of children and adolescents with cerebellar tumors identified by neuroimaging was performed at the Children’s Hospital Los Angeles. Results: There were 8 girls and 14 boys in the study, aged 14 months to 17 years. The tumor sizes ranged from 2 to 6.5 cm in diameter. The patients presented with ataxia, headache, vomiting, depressed or irritable mood and inattention. Symptoms of PFS were present postoperatively in all except for the 2 patients with lateral tumors. The symptoms began before resection, were most prominent immediately after surgery, and improved over time. Neuropsychological assessment of 10 patients documented a persistent cognitive decrement. Conclusion: This small, descriptive study provides information on the natural history of pediatric posterior fossa tumors from before surgery through the postoperative period.

OBJECT Even with improved prenatal and neonatal care, intraventricular hemorrhage (IVH) occurs in approximately 25%–30% of preterm infants, with a subset of these patients developing hydrocephalus. This study was undertaken to describe current trends in hospitalization of preterm infants with posthemorrhagic hydrocephalus (PHH) using the Nationwide Inpatient Sample (NIS) and the Kids’ Inpatient Database (KID). METHODS The KID and NIS were combined to generate data for the years 2000–2010. All neonatal discharges with ICD-9-CM codes for preterm birth with IVH alone or with IVH and hydrocephalus were included. RESULTS There were 147,823 preterm neonates with IVH, and 9% of this group developed hydrocephalus during the same admission. Of patients with Grade 3 and 4 IVH, 25% and 28%, respectively, developed hydrocephalus in comparison with 1% and 4% of patients with Grade 1 and 2 IVH, respectively. Thirty-eight percent of patients with PHH had permanent ventricular shunts inserted. Mortality rates were 4%, 10%, 18%, and 40%, respectively, for Grade 1, 2, 3, and 4 IVH during initial hospitalization. Length of stay has been trending upward for both groups of IVH (49 days in 2000, 56 days in 2010) and PHH (59 days in 2000, 70 days in 2010). The average hospital cost per patient (adjusted for inflation) has also increased, from $201,578 to $353,554 (for IVH) and $260,077 to $495,697 (for PHH) over 11 years. CONCLUSIONS The number of neonates admitted with IVH has increased despite a decrease in the number of preterm births. Rates of hydrocephalus and mortality correlated closely with IVH grade. The incidence of hydrocephalus in preterm infants with IVH remained stable between 8% and 10%. Over an 11-year period, there was a progressive increase in hospital cost and length of stay for preterm neonates with IVH and PHH that may be explained by a concurrent increase in the proportion of patients with congenital cardiac anomalies.

Object The purpose of this study was to evaluate the posterior interhemispheric retrocallosal approach (PIRA) for its safety and efficacy in the resection of pineal region and posterior fossa lesions in children. Methods Twenty-nine PIRAs were performed in 26 children between March 1997 and March 2009, and these cases were retrospectively reviewed. There were 15 girls and 11 boys in the series. The median age at the time of surgery was 7 years (range 7 months–17 years). Twenty-seven cases were treated for tumor, 1 for loculated hydrocephalus, and 1 for an aneurysm. Results Of the 27 cases treated for tumor, there were 20 (74%) gross-total resections, 5 (19%) subtotal resections, and 2 (7%) biopsies. One bridging vein was sacrificed in 6 cases and 2 bridging veins were divided in 1 case, whereas in 3 cases there was sacrifice of a single deep cerebral vein. No patient developed radiographic evidence of venous infarction. Approach-related complications were low, and included 2 cases of transient homonymous hemianopia. There were no surgery-related deaths. Conclusions This approach allows for ample access to pineal region and posterior fossa lesions, with low postoperative morbidity.

Object The interhemispheric transcallosal approach offers an excellent surgical corridor for the treatment of deep-seated midline lesions. The approach typically requires the sacrifice of one or more middle-third superior sagittal sinus (SSS) cortical bridging veins, which introduces the risk of venous infarction and associated neurological injury. The authors studied the incidence of venous infarcts following this operative approach in a pediatric population. Methods The authors performed a retrospective review of surgical cases involving pediatric patients treated at the Children's Hospital Los Angeles between 1990 and 2007, in which an interhemispheric transcallosal operative procedure was performed and one or more middle-third SSS cortical bridging veins were occluded. Postoperative MR imaging studies done 1–3 days following the procedure were analyzed and compared with preoperative studies. Results Sixty-three patients met the inclusion criteria. No patient developed MR imaging evidence of venous infarction. Conclusions The occlusion of one or more middle-third SSS cortical bridging veins related to the interhemispheric transcallosal approach resulted in no incidence of cerebral venous infarction in this pediatric population.

Object The identification of Chiari malformations Type I (CM-Is) has increased in recent years, commonly during MR imaging for evaluation of a possible cause of scoliosis. The treatment of this abnormality remains controversial, and the expected success of treatment is unclear. The goal of the present study is to evaluate the effects of a craniotomy for CM-I decompression on scoliosis in children and adolescents. Methods The authors conducted a 10-year retrospective review of pediatric patients who were found to have a CM-I during evaluation for scoliosis. Seventy-nine patients were identified, ranging in age from 6 months to 18 years (median 12 years). There were 42 girls (54%) and 37 boys (46%). All were noted on MR imaging to have hydrosyringomyelia of the spinal cord. Forty-nine patients had curvatures less than 20° prior to treatment. The other 30 patients had curves ranging from 25° to 80° and underwent orthopedic follow-up and treatment. None of these patients were referred for specific neurological complaints, but 12 (16%) had neurological signs on physical examination. All were treated with a craniocervical decompression in a standard fashion. Follow-up ranged from 6 to 93 months with a median of 35 months. Magnetic resonance images obtained at 6 months postoperatively and serial standing anteroposterior spine radiographs were used to evaluate outcomes. Results On the MR images obtained 6 months postoperatively, 70 patients (89%) had a significant reduction in the syrinx with an associated ascent of the cerebellar tonsils. Persistent large syringes were treated with reoperation in 6 patients, and shunts were inserted for hydrocephalus in 2 patients. None of the 49 patients with curves less than 20° had progression of their curvature postoperatively. Of the 30 patients with curves greater than 25°, 9 had no change in the scoliosis or had a reduction in curve magnitude after Chiari decompression. This group required no further therapy and was effectively treated by Chiari decompression alone. Twenty-one patients required further scoliosis treatment after Chiari decompression; 12 required orthotic treatment, 11 received spinal instrumentation and fusion surgery, and 2 received orthoses followed by fusion and instrumentation. The severity of the curvature beyond 20° did not predict the need for spinal surgery. Conclusions This large series reports on the efficacy of treatment for scoliosis associated with a CM-I and syrinx in children. A CM-I decompression alone was adequate treatment for mild scoliosis of less than 20°. Patients with scoliosis greater than 20° required bracing and/or spinal fusion surgery 70% of the time in addition to the CM-I decompression.

We present a series of children whose head shapes and suture status do not make sense. There were 3 patients with complete absence of sutures and normal head shapes. One patient was evaluated for microcephaly at 6 years of age. In the second, the absence of sutures was discovered on workup for headaches at 8 years of age. The third underwent x-ray imaging for parasagittal bony nodules at 1 month of age. The head circumferences fell at the 4th, 25th, and 50th percentiles, respectively. The 2 older children were in normal classes, and the youngest was meeting milestones appropriately. We encountered 3 patients with fusion of the sagittal suture and normal head shapes. One had a flattened occiput, the second patient was thought to be brachycephalic, and the third was macrocephalic. Head circumferences were at the fourth, 50th, and 75th percentiles. The patient with head circumference at the fourth percentile had fetal alcohol syndrome and speech delay. The other 2 were developing normally with follow-up of 14 months. Finally, 3 patients underwent surgery for characteristic craniosynostotic head shapes and were found to have patent sutures at surgery. One patient had classic unilateral coronal synostosis and a patent suture on the side of the defect. The second patient had Crouzon syndrome with characteristic head shape, but open coronal sutures. The third patient had the appearance of bicoronal synostosis, with a patent suture on one side. They have had good results from their craniofacial reconstructions at 24, 12, and 6 months' follow-up.We have no explanation for these interesting findings.

The successful treatment of children with a primary CNS germ cell tumor can be greatly influenced by the neurosurgeon involved in the diagnostic and therapeutic care of these children. Variability in surgical philosophies no doubt exists due to the relatively infrequent incidence of these tumors, a lack of consensus regarding diagnostic and therapeutic approaches, and the advent of recent surgical innovations. Many of these issues were discussed at the Second International Symposium on Central Nervous System Germ Cell Tumors through presented abstracts and invited presentations. The neurosurgical aspects of these proceedings are summarized here in an effort to present the agreed-upon and debated issues that may confront the pediatric neurosurgeon.

Intracranial ependymomas in children have high rates of recurrence. Salvage therapy typically includes repeat resection, possibly chemotherapy, and re-irradiation. Stereotactic radiosurgery has been used for re-irradiation. It offers the theoretical advantages of delivering a high dose of radiation to a small target, providing a maximum dose to the tumor while avoiding surrounding critical brain structures and previously irradiated tissue.Few reports in the literature describe this technique, with mixed, but not often successful, outcomes.

Object The goal of this study was to review a large series of patients with Langerhans cell histiocytosis (LCH) who had craniospinal lesions to assess the long-term course, outcome, and efficacy of treatment of the disease. Methods Forty-four patients with LCH who presented to a single pediatric neurosurgical department between 1976 and 2006 were retrospectively reviewed. Results This series included 29 boys and 15 girls, ranging in age from 2 months to 13 years, with a mean follow-up duration of 4.5 years. Twenty-seven patients (61%) had unifocal bone lesions, 12 (27%) had multifocal bone disease, 2 (5%) had solitary hypothalamic–pituitary axis lesions, and 3 (7%) had multiple organ involvement at presentation. Five (19%) of the 27 patients with unifocal bone disease and 4 (33%) of the 12 patients with multifocal bone disease had delayed development of new bone lesions during the follow-up period. The time to development of new bone lesions ranged from 1 month to 1 year. Two of the 3 patients with multiple-organ LCH died. Patient age ≤ 2 years at the time of initial presentation was a risk factor for both initial multifocality and eventual dissemination. In all patients with initial multifocal bone involvement or later dissemination of unifocal bone disease, LCH was controlled by chemotherapy, except for 2 who were treated by surgery alone. Three patients had histological evidence of spontaneous resolution of their lesions. Conclusions Patients with unifocal LCH can be effectively treated with surgery alone. Very young patients are more likely to have multifocal disease and disseminations, and will usually require chemotherapy to control their disease. Spontaneously regressing lesions need not be resected; however, a biopsy procedure can be performed for diagnostic purposes.

Background/Purpose: Symptomatic ventriculoperitoneal shunt (VPS) malfunction can occur without progressive ventricular dilatation on imaging studies. A review of VPS revisions performed during an 18-month period was conducted to better characterize this cohort. The incidence of VPS malfunction without ventricular dilatation and the clinical features that may predispose to its occurrence have not been previously described. Methods: A retrospective review was conducted of all patients under the age of 18 who underwent VPS revision due to shunt malfunction from June 2001 to December 2002. The incidence of various factors that might correlate with the development of low compliance ventricles was examined. Results: One hundred and seventy-seven patients underwent a total of 287 VPS revisions during the study period; 16 patients (9%) had signs of raised intracranial pressure and showed no radiographic evidence of progressive ventricular dilatation. History of prior shunt-related ventriculitis positively correlated with the development of low compliance ventricles (Fisher’s exact test, p = 0.043). Conclusion: Nine percent of pediatric patients with symptomatic VPS malfunctions lack radiographic evidence of progressive ventricular dilatation on imaging studies. Patients with prior shunt infections are particularly at risk, necessitating increased vigilance when clinical signs and symptoms are apparent.

OBJECT There is a known association of hydrocephalus with encephaloceles. Risk factors for hydrocephalus and neurological deficit were ascertained in a series of patients born with an encephalocele. METHODS A retrospective analysis was undertaken of patients treated for encephaloceles at Children's Hospital Los Angeles between 1994 and 2012. The following factors were evaluated for their prognostic value: age at presentation, sex, location of encephalocele, size, contents, microcephaly, presence of hydrocephalus, CSF leak, associated cranial anomalies, and neurological outcome. RESULTS Seventy children were identified, including 38 girls and 32 boys. The median age at presentation was 2 months. The mean follow-up duration was 3.7 years. Encephalocele location was classified as anterior (n = 14) or posterior (n = 56) to the coronal suture. The average maximum encephalocele diameter was 4 cm (range 0.5–23 cm). Forty-seven encephaloceles contained neural tissue. Eight infants presented at birth with CSF leaking from the encephalocele, with 1 being infected. Six patients presented with hydrocephalus, while 11 developed progressive hydrocephalus postoperatively. On univariate analysis, the presence of neural tissue, cranial anomalies, encephalocele size of at least 2 cm, seizure disorder, and microcephaly were each positively associated with hydrocephalus. On multivariate logistic regression modeling, the single prognostic factor for hydrocephalus of borderline statistical significance was the presence of neural tissue (odds ratio [OR] = 5.8, 95% confidence interval [CI] = 0.8–74.0). Fourteen patients had severe developmental delay, 28 had mild/moderate delay, and 28 were neurologically normal. On univariate analysis, the presence of cranial anomalies, larger size of encephalocele, hydrocephalus, and microcephaly were positively associated with neurological deficit. In the multivariable model, the only statistically significant prognostic factor for neurological deficit was presence of hydrocephalus (OR 17.2, 95% CI 1.7–infinity). CONCLUSIONS In multivariate models, the presence of neural tissue was borderline significantly associated with hydrocephalus and the presence of hydrocephalus was significantly associated with neurological deficit. The location of the encephalocele did not have a statistically significant association with incidence of hydrocephalus or neurological deficit. In contrast to modestly good/fair neurological outcome in children with an encephalocele without hydrocephalus, the presence of hydrocephalus resulted in a far worse neurological outcome.

The authors analyzed the chemical composition of intracranial arachnoid cyst fluid to gain greater understanding of potential causes of cyst growth.The authors studied 54 pediatric patients who underwent craniotomies for arachnoid cyst fenestration. Cyst fluid was analyzed and results were compared with expected values (EVs) for cerebrospinal fluid (CSF). The arachnoid cyst fluid values were as follows: protein (median 37 mg/dl, mean 178.2 mg/dl, and EV 25.6 mg/dl), osmolality (median 284 mOsm/kg, mean 285.5 mOsm/kg, and EV 287.2 mOsm/kg), potassium (median 2.6 mEq/L, mean 2.68 mEq/L, and EV 2.88 mEq/L), sodium (median 140 mEq/L, mean 142.2 mEq/L, and EV 143.3 mEq/L), and chloride (median 122 mEq/L, mean 120.77 mEq/L, and EV 120.3 mEq/L). Median glucose was 51 mg/dl (range 26-98 mg/dl). The median white blood cell count in cyst fluid was 1/mm3, and the median red blood cell (RBC) count was 1.5/mm3. All Gram stains and cultures were negative. No statistically significant correlations were found between arachnoid cyst fluid protein level and elevated RBC count, patient age, or the subsequent need for a shunt. Elevated RBC count in cyst fluid was also not associated with the subsequent need for a shunt.Fluid from arachnoid cysts has a chemical composition similar to that of CSF, but some of them have significantly elevated protein levels. The authors hypothesize that elevated protein content may contribute to arachnoid cyst expansion in some patients.

Object Atypical teratoid/rhabdoid tumors (ATRTs) represent a relatively newly categorized neoplastic entity. They commonly present in childhood, and have a rapidly progressive clinical course with a survival time of less than 1 year. Treatment regimens have been nonuniform. In this retrospective review of patients with ATRTs who were treated at the authors' institution according to a uniform protocol, the goal was to assess the efficacy of the treatment and its outcome. Methods Over a 7-year period, ATRT was diagnosed in 11 patients (six boys and five girls). The median age of the patients was 61 months, and their ages ranged from 3 months to 17 years. Signs and symptoms began, on average, a little more than 1 month before diagnosis and included the following: headache (36%), nausea and vomiting (46%), lethargy (18%), seizures (27%), cranial nerve findings (46%), ataxia (18%), long tract findings (18%), and hydrocephalus (46%). Tumor location was cortical in four patients, in the pineal region in four, in the posterior fossa in two, and spinal in one. In one patient disseminated disease was revealed on the initial imaging study; seven patients had disseminated tumor subsequently. Treatment consisted of chemotherapy in 11 patients, chemotherapy and local radiation in five, and chemotherapy and craniospinal radiation in three. Six patients are alive, three have died, and two were lost to follow-up review. The mean time to death was 24 months, and ranged from 2 to 67 months. Among the surviving patients the mean duration of follow up is 18.5 months and ranges from 2 to 37 months. The median time to progression was 3.5 months. Conclusions Atypical teratoid/rhabdoid tumors are malignant lesions with rapid progression. Further study is necessary to determine the efficacy of therapy.

Object The management of pediatric intraventricular tumors is highly dependent on identification of the tumor type. Choroid plexus papillomas, a common intraventricular tumor in children, can be difficult to distinguish radiographically from choroid plexus carcinomas and other common pediatric central nervous system (CNS) tumors. In this study to overcome the limitations of current noninvasive imaging modalities, the authors use novel magnetic resonance (MR) spectroscopy techniques in vivo to elucidate the identifying biochemical features of choroid plexus tumors that may facilitate diagnosis and treatment. Methods Based on an Internal Review Board–approved protocol, six children with newly diagnosed, untreated intraventricular brain tumors were identified. On retrospective review, this series included three choroid plexus papillomas and three choroid plexus carcinomas. Single-voxel proton MR spectroscopy with a short echo time was performed, and absolute metabolite concentrations (in mmol/kg) were determined using fully automated quantitation. These results were compared with MR spectroscopy profiles obtained in 54 other untreated CNS neoplasms in children. The myo-inositol (mI) level was significantly higher in choroid plexus papillomas (> 10 mmol/kg), uniquely distinguishing these tumors from choroid plexus carcinomas and all other tumors. Choroid plexus carcinomas, on the other hand, had significantly elevated levels of choline when compared with choroid plexus papillomas. Conclusions In this study the authors find that mI is a biochemical constituent that uniquely identifies choroid plexus papillomas and can be used as a noninvasive means of diagnosis and for follow-up evaluations in patients with this disease.

Background Dystonia is a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both. It can be classified as primary or secondary. There is no cure for dystonia and the goal of treatment is to provide a better quality of life for the patient. Surgical intervention is considered for patients in whom an adequate trial of medical treatment has failed. Deep brain stimulation (DBS), specifically of the globus pallidus interna (GPi), has been shown to be extremely effective in primary generalized dystonia. There is much less evidence for the use of DBS in patients with secondary dystonia. However, given the large number of patients with secondary dystonia, the significant burden on the patients and their families, and the potential for DBS to improve their functional status and comfort level, it is important to continue to investigate the use of DBS in the realm of secondary dystonia. Object The objective of this study is to review a series of cases involving patients with secondary dystonia who have been treated with pallidal DBS. Methods A retrospective review of 9 patients with secondary dystonia who received treatment with DBS between February 2011 and February 2013 was performed. Preoperative and postoperative videos were scored using the Barry-Albright Dystonia Scale (BADS) and Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) by a neurologist specializing in movement disorders. In addition, the patients' families completed a subjective questionnaire to assess the perceived benefit of DBS. Results The average age at DBS unit implantation was 15.1 years (range 6–20 years). The average time to follow-up for the BADS evaluation from battery implantation was 3.8 months (median 3 months). The average time to follow-up for the subjective benefit evaluation was 10.6 months (median 9.5 months). The mean BADS scores improved by 9% from 26.5 to 24 (p = 0.04), and the mean BFMDRS scores improved by 9.3% (p = 0.055). Of note, even in patients with minimal functional improvement, there seemed to be decreased contractures and spasms leading to improved comfort. There were no complications such as infections or hematoma in this case series. In the subjective benefit evaluation, 3 patients' families reported “good” benefit, 4 reported “minimal” benefit, and 1 reported no benefit. Conclusions These early results of GPi stimulation in a series of 9 patients suggest that DBS is useful in the treatment of secondary generalized dystonia in children and young adults. Objective improvements in BADS and BFMDRS scores are demonstrated in some patients with generalized secondary dystonia but not in others. Larger follow-up studies of DBS for secondary dystonia, focusing on patient age, history, etiology, and patterns of dystonia, are needed to learn which patients will respond best to DBS.

Thirty-six cases of pilocytic astrocytomas treated at the Childrens Hospital of Los Angeles from 1984 through 1995 were reviewed. The mean age at initial presentation was 8 years (range 15 months to 14 years). These patients were followed for an average of 5.5 years. No patient was given chemotherapy or radiation therapy after the initial surgery for pilocytic astrocytoma. Twenty-three patients (64%) had a gross total resection with no residual tumor on immediate postoperative imaging studies. Three of these children had tumor recurrences 2-5 years after their initial surgery, requiring re-excision of their tumors. All 3 patients are subsequently tumor-free, with follow-up ranging from 4 to 10 years. In 4 patients with residual tumor involving the brainstem, there has been neither imaging evidence of tumor enlargement nor progression of clinical findings at 2.5, 4, 6 and 6 years, respectively. Nine of the 13 patients with residual tumor underwent re-excision, either for progression of symptoms or documented tumor growth on imaging studies. The second operation was undertaken an average of 1 year (range 1 month to 6 years) after the first. In 4 of these children (11% of our whole series), the recurrent tumor was classified as anaplastic astrocytoma. Three of these 4 children received radiation and/or chemotherapy, with only 1 patient showing disease progression in the follow-up period. Repeat blinded histopathological examination of these tumors confirmed both diagnoses. However, it was noted that 3 of the 4 pilocytic astrocytomas which subsequently showed anaplastic change initially displayed increased perivascular cellularity, while only 2 of the remaining 32 tumors exhibited this feature. These results encourage continued vigilance in the follow-up of pilocytic astrocytomas, and describe a histological feature which might indicate a more aggressive disease course.

The literature reveals several ominous trends in firearm injuries in the United States. The crack epidemic of the mid-1980s, the increasing availability of handguns and more lethal weapons, and, particularly in Southern California, the rise of gang violence have all contributed significantly to this trend. Although the crack epidemic seems to have abated, the weapons it produced and the criminal elements it encouraged are still around. Many public health experts have advocated gun control as a means to stop this violence, and they have several compelling examples to justify their efforts. Gun control, however, is beyond the scope of this article. Neurosurgeons can have some effect at the local level on gang violence, however. The University of Southern California Department of Neurological Surgery has been working with the Think First Organization and Community Youth Gang Service Organization to reach out to children before they become involved with the gangs. These organizations are also working with the justice system to reform young people already involved with gangs. The hope is that these patterns of violence can be arrested on the community level.

Object The objective of this study was to determine the role of intracranial CSF examination in detecting true cases of early tumor dissemination. Cerebrospinal fluid dissemination is an ominous feature of pediatric brain tumors, occurring in as many as 30% of medulloblastomas, 25% of supratentorial primitive neuroectodermal tumors (PNETs), and 5% of ependymomas at diagnosis. Detecting early dissemination is important for determining both treatment and prognosis. Dissemination can be detected by evaluating imaging of the full neuraxis and by examining CSF cytology. Neuraxis MR imaging and lumbar CSF cytology evaluation are widely accepted methods for determining dissemination. However, the value of examining intracranial CSF cytology in detecting early dissemination is uncertain. Methods Under an institutional review board–approved protocol, medical records, pathology reports, and radiology reports for 150 patients who had undergone resection of brain tumors (88 with medulloblastomas, 21 with supratentorial PNETs, and 41 with ependymomas) and who had been evaluated using neuraxis MR imaging studies in the last 15 years were retrospectively reviewed. Radiology results were compared with the CSF cytology results and long-term disease outcomes. Results Between lumbar and intracranial CSF cytology results, 7 of 40 were discordant: in 2 intracranial CSF was negative and lumbar CSF was positive, and in 5 the reverse was true. The discordance percentage was 18%, with a kappa statistic of 0.36. Between MR imaging and lumbar CSF cytology results, 11 of 65 were discordant: in 9 the lumbar CSF was negative and MR imaging was positive, and in 2 the reverse was true. The discordance percentage is 17%, with a kappa statistic of 0.27. Between MR imaging and intracranial CSF cytology results, 8 of 52 were discordant: in 3 intracranial CSF was negative and MR imaging was positive, and in 5 the reverse was true. The discordance rate was 15%, with a kappa statistic of 0.41. Patients with positive and negative results on perioperative neuraxis MR imaging studies had a median survival of 26.8 and 33.1 months, respectively (p = 0.02). Patients with positive and negative results on perioperative lumbar CSF cytology had a median survival of 20.1 and 31.4 months, respectively (p = 0.11). Patients with positive and negative results on intracranial CSF cytology had a median survival of 31 and 31.4 months, respectively (p = 0.84). Conclusions Discordance exists between the results of neuraxis MR imaging and lumbar and intracranial CSF cytology in perioperative detection of tumor dissemination for pediatric medulloblastoma, supratentorial PNETs, and ependymoma. In 1 case in this series, perioperative dissemination was detected by intracranial CSF cytology, but not by lumbar CSF cytology or neuraxis MR imaging. Isolated intracranial CSF cytology positivity may represent an earlier stage of disseminated disease. Complementary use of perioperative neuraxis MR imaging and lumbar and intracranial CSF cytology can reduce the incidence of missed diagnoses of dissemination. Survival analysis revealed that perioperative neuraxis MR imaging findings are correlated with survival, whereas perioperative lumbar and intracranial CSF cytology findings are not.

Giant cell tumors of the bone are rare, locally aggressive lesions that primarily affect the epiphyses of long bones. These tumors can occur in the skull, principally in the sphenoid and temporal bones. Symptoms of these tumors depend on their site of origin but typically include headache, pain, visual field defects, and conductive hearing loss. Histologically, these tumors consist of three cell types: osteoclast-like multinucleated giant cells; round mononuclear cells resembling monocytes; and spindle-shaped, fibroblast-like stromal cells. Radiographically, the tumors appear osteolytic and radiolucent without a sclerotic border. These tumors typically present in the third to fourth decades of life and rarely occur in patients under 20 years of age. The small number of studies of giant cell tumors of the skull has focused on the adolescent and adult populations. The authors report two cases of giant cell tumors of the skull in pediatric patients. In the first case, a 2-year-old girl presented with swelling behind the right ear. In the second case, a 7-week-old girl presented with a mass within the external auditory canal. Both patients underwent metastatic workup and biopsy procedures before resection of the tumor. Both case reports contribute to the literature of giant cell tumors of the skull by describing this condition in pediatric patients. To the authors' knowledge, these cases represent the youngest two patients with giant cell tumors of the skull yet described.