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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.

Purpose: Medulloblastoma in children can be categorized into at least four molecular subgroups, offering the potential for targeted therapeutic approaches to reduce treatment-related morbidities. Little is known about the role of tumor microenvironment in medulloblastoma or its contribution to these molecular subgroups. Tumor microenvironment has been shown to be an important source for therapeutic targets in both adult and pediatric neoplasms. In this study, we investigated the hypothesis that expression of genes related to tumor-associated macrophages (TAM) correlates with the medulloblastoma molecular subgroups and contributes to a diagnostic signature.Methods: Gene-expression profiling using human exon array (n = 168) was analyzed to identify medulloblastoma molecular subgroups and expression of inflammation-related genes. Expression of 45 tumor-related and inflammation-related genes was analyzed in 83 medulloblastoma samples to build a gene signature predictive of molecular subgroups. TAMs in medulloblastomas (n = 54) comprising the four molecular subgroups were assessed by immunohistochemistry (IHC).Results: A 31-gene medulloblastoma subgroup classification score inclusive of TAM-related genes (CD163 and CSF1R) was developed with a misclassification rate of 2%. Tumors in the Sonic Hedgehog (SHH) subgroup had increased expression of inflammation-related genes and significantly higher infiltration of TAMs than tumors in the Group 3 or Group 4 subgroups (P < 0.0001 and P < 0.0001, respectively). IHC data revealed a strong association between location of TAMs and proliferating tumor cells.Conclusions: These data show that SHH tumors have a unique tumor microenvironment among medulloblastoma subgroups. The interactions of TAMs and SHH medulloblastoma cells may contribute to tumor growth revealing TAMs as a potential therapeutic target. Clin Cancer Res; 21(6); 1457–65. ©2014 AACR.

PDF file 92K, Supplemental Fig 5: Expression of PID1 protein in human brain tumors and cell lines

PDF file - 190K, Supplemental Fig 2: Proneural and Neural GBMs have higher PID1 mRNA; higher PID1 mRNA correlates with higher OS in patients with gliomas.

Supplemental Methods and Supplemental Figures 1-9 Supplemental Figure 1. Overview of samples studies and 31-gene signature development Supplemental Figure. 2. Non-negative matrix factorization (NMF) analysis of the combined cohort of medulloblastoma samples (n=168) (A) Cophenetic correlation coefficient based on 2000 clustering runs for 2-10 clusters utilizing genes obtained at varying coefficients of variation; the data demonstrate that the highest cophenetic correlation coefficient was obtained at 4 clusters with 369 genes. (B) NMF consensus heatmap of 4 subgroups using the dataset containing 369 genes with high coefficient of variation. (C) Silhouette plot for identification of outliers. Outliers (n=31) were defined as samples with a silhouette width

PDF file - 159K, Supplemental Fig 1: PID1 mRNA is lower in less favorable medulloblastomas in two additional independent datasets; PID1 mRNA correlates with rf-PFS when analyzing CHLA medulloblastomas by tertiles.

PDF file - 17K, Supplemental Fig 4: PID1 increases the proportion of cells in Sub G0/G1 in CHLA-259 medulloblastoma cells.

Purpose: We present here the first report of PID1 (Phosphotyrosine Interaction Domain containing 1; NYGGF4) in cancer. PID1 was identified in 2006 as a gene that modulates insulin signaling and mitochondrial function in adipocytes and muscle cells.Experimental Design and Results: Using four independent medulloblastoma datasets, we show that mean PID1 mRNA levels were lower in unfavorable medulloblastomas (groups 3 and 4, and anaplastic histology) compared with favorable medulloblastomas (SHH and WNT groups, and desmoplastic/nodular histology) and with fetal cerebellum. In two large independent glioma datasets, PID1 mRNA was lower in glioblastomas (GBM), the most malignant gliomas, compared with other astrocytomas, oligodendrogliomas and nontumor brains. Neural and proneural GBM subtypes had higher PID1 mRNA compared with classical and mesenchymal GBM. Importantly, overall survival and radiation-free progression-free survival were longer in medulloblastoma patients whose tumors had higher PID1 mRNA (univariate and multivariate analyses). Higher PID1 mRNA also correlated with longer overall survival in patients with glioma and GBM. In cell culture, overexpression of PID1 inhibited colony formation in medulloblastoma, atypical teratoid rhabdoid tumor (ATRT), and GBM cell lines. Increasing PID1 also increased cell death and apoptosis, inhibited proliferation, induced mitochondrial depolaization, and decreased serum-mediated phosphorylation of AKT and ERK in medulloblastoma, ATRT, and/or GBM cell lines, whereas siRNA to PID1 diminished mitochondrial depolarization.Conclusions: These data are the first to link PID1 to cancer and suggest that PID1 may have a tumor inhibitory function in these pediatric and adult brain tumors. Clin Cancer Res; 20(4); 827–36. ©2013 AACR.

PDF file - 440K, Supplemental Table 1: Characteristics of the CHLA Medulloblastoma Patients. Supplemental Table 2: Neural and Proneural GBM subgroups have higher PID1 mRNA compared to Classical and Mesenchymal.

Supplemental Tables 1-6 Supplemental Table 1. Patient and tumor characteristics Supplemental Table 2. TLDA design (45 genes) Supplemental Table 3. Information for samples evaluated using HuE Supplemental Table 4. TLDA 31-gene molecular classification of core samples Supplemental Table 5. TLDA 31-gene classification for samples without HuEx data Supplemental Table 6. TLDA 31-gene Confusion Matrix

PDF file - 42K, Supplemental Fig 3: PID1 also inhibits colony formation using two other plasmid expression vectors.

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.

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.

BACKGROUND: Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology. OBJECTIVE: To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD. METHODS: The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD. Comparisons were made between subjects who received PFD alone and those with PFD + OCF or PFD + OCF/VD. RESULTS: All 637 patients underwent PFD, 505 (79.2%) with and 132 (20.8%) without duraplasty. A total of 12 subjects went on to have OCF at some point in their management (PFD + OCF), whereas 4 had OCF and VD (PFD + OCF/VD). Of those with complete data, a history of platybasia (3/10, P = .011), Klippel-Feil (2/10, P = .015), and basilar invagination (3/12, P

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.

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.

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.

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.

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).

Isocitrate dehydrogenase (IDH) mutations are rare in pediatric and adolescent gliomas. We recently identified three adolescent/young adult (AYA) patients with IDH‐mutant low grade gliomas of the brainstem with several key clinicopathologic and molecular features in common. We discuss these three cases and review the current literature.

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.

Adamantinomatous craniopharyngioma (ACP) is a biologically benign but clinically aggressive lesion that has a significant impact on quality of life. The incidence of the disease has a bimodal distribution, with peaks occurring in children and older adults. Our group previously published the results of a transcriptome analysis of pediatric ACPs that identified several genes that were consistently overexpressed relative to other pediatric brain tumors and normal tissue. We now present the results of a transcriptome analysis comparing pediatric to adult ACP to identify biological differences between these groups that may provide novel therapeutic insights or support the assertion that potential therapies identified through the study of pediatric ACP may also have a role in adult ACP. Using our compiled transcriptome dataset of 27 pediatric and 9 adult ACPs, obtained through the Advancing Treatment for Pediatric Craniopharyngioma Consortium, we interrogated potential age-related transcriptional differences using several rigorous mathematical analyses. These included: canonical differential expression analysis; divisive, agglomerative, and probabilistic based hierarchical clustering; information theory based characterizations; and the deep learning approach, HD Spot. Our work indicates that there is no therapeutically relevant difference in ACP gene expression based on age. As such, potential therapeutic targets identified in pediatric ACP are also likely to have relvance for adult patients.

BACKGROUND/AIMS: Children with congenital cochleovestibular abnormalities associated with profound hearing loss have few treatment options if cochlear implantation does not yield benefit. An alternative is the auditory brainstem implant (ABI). Regulatory authority device approvals currently include a structured benefit-risk assessment. Such an assessment, for regulatory purposes or to guide clinical decision-making, has not been published, to our knowledge, for the ABI and may lead to the design of a research program which incorporates regulatory authority, family, and professional input. METHODS: Much structured benefit-risk research has been conducted in the context of drug trials, here we apply this approach to device studies. A qualitative framework organized benefit (speech recognition, parent self-report measures) and risk (surgery- and device-related) information to guide the selection of candidates thought to have potential benefit from ABI. RESULTS: Children with cochleovestibular anatomical abnormalities are challenging for appropriate assessment of candidacy for a cochlear implant or an ABI. While the research is still preliminary, children with an ABI appear to slowly obtain benefit over time. A team of professionals, including audiological, occupational, and educational therapy, affords maximum opportunity for benefit. CONCLUSIONS: Pediatric patients who have abnormal anatomy and are candidates for an implantable auditory prosthetic require an individualized, multi-systems review. The qualitative benefit-risk assessment used here to characterize the condition, the medical need, potential benefits, risks, and risk management strategies has revealed the complex factors involved. After implantation, continued team support for the family during extensive post-implant therapy is needed to develop maximum auditory skill benefit.

METHODS Through the Advancing Treatment for Pediatric Craniopharyngioma (ATPC) consortium we accumulated preoperative MRIs and tumor RNA for 50 unique ACP patients. MRIs were assessed quantitatively for 28 different features and analyzed using Multiple Factor Analysis (MFA) and optimal clustering was determined via maximization of Bayesian Information Criterion (BIC). Following bulk RNAseq, differential expression and pathway enrichment were performed using standard methodologies (i.e., DESeq2 and GSEA). RESULTS MRI features were well represented in the first 3 dimensions of MFA (variance explained=67.32%); specifically tumor/cyst size, ventricular size, and cyst fluid diffusivity. Using this three-way axis, we identified 3 patient subgroups. Transcriptional differences between these subgroups indicated one group was enriched for DNA damage response and MYC related pathways, one group enriched for SHH, and one group enriched for WNT/β-catenin and EMT-related pathways. CONCLUSION This preliminary work suggests that there may be unique gene expression variants within ACP, which may be identified preoperatively using easily quantifiable MRI parameters. These radiogenomic signatures could provide prognostic information and/or guidance in the selection of antitumor therapies for children with ACP.

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.

Posterior hemivertebrae are wedge shaped deformities that can result in progressive kyphosis. Surgical intervention at an early age may be required, however choice of surgical technique is controversial. The aim of this report was to describe a case of progressive congenital lumbar kyphosis and bilateral posterior hemivertebra with retropulsion of tissue into the spinal canal treated successfully by posterior spinal fusion and instrumentation without anterior hemivertebra resection or decompression. We report on a patient with bilateral lumbar posterior hemivertebra at L1-L2 treated with posterior spinal fusion and instrumentation at less than 1 year of age. At 10 mo of age, the patient underwent posterior spinal fusion and instrumentation with resection of L1 and L2 posterior elements. No resection of the anterior aspect of the bilateral hemivertebrae was performed. Correction of the kyphotic deformity was maintained at last radiographic follow-up at five years post-operatively and there is no evidence of spinal stenosis. Early intervention with resection of posterior elements and fusion with instrumentation for bilateral congenital lumbar hemivertebrae provided adequate deformity correction and maintenance of the spinal canal width without anterior resection. Despite his young age, instrumentation was both feasible and beneficial in maintaining alignment.

Background Some patients with isolated sagittal craniosynostosis have demonstrated mild neurodevelopmental delays. This study examined potential preoperative risk factors for developmental delay. Methods Patients completed preoperative Bayley Scales of Infant and Toddler Development, Third Edition, and medical records were reviewed. Multivariate analyses of covariance and correlations were calculated. Results Participants (n = 77) were predominantly male (77.9 percent) and were aged 2 to 12 months (mean, 5.1 ± 2.3 months). Patients were classified with no delays [n = 63 (82 percent)] or delays [n = 14 (18 percent)] in one or more developmental area(s). There were no group sociodemographic differences. Prenatally, patients with delays versus no delays had lower mean gestational age in weeks (36.9 ± 2.8 weeks versus 39.1 ± 1.7 weeks; p = 0.001) with higher rates of gestational diabetes (36 percent versus 5 percent; p = 0.006) and premature rupture of membranes (14 percent versus 2 percent; p = 0.026). At birth, patients with delays had lower mean birth weight (2982 ± 714 g versus 3374 ± 544 g; p = 0.053), higher rates of respiratory distress (29 percent versus 5 percent; p = 0.005), additional medical diagnoses (57 percent versus 13 percent; p = 0.001), and longer mean neonatal intensive care unit stays (1.4 ± 1.8 weeks versus 0.2 ± 0.9 week; p = 0.002). Variables differing by group had moderate correlations. Conclusions Patients with nonsyndromic sagittal craniosynostosis that had delays in development had lower gestational age and birth weight, with more prenatal and birth complications. These factors can help identify patients who might be at risk for delay and need close monitoring. Clinical question/level of evidence Risk, III.

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.

BACKGROUND: This study aims to identify the prevalence of and risk factors for seizure development after supratentorial brain tumor resection in pediatric patients. This could be used to guide the postoperative management and usage of anti-epileptic drugs (AEDs). METHODS: Retrospective study was conducted for patients between 0 and 21 years with supratentorial tumor resection between 2005 and 2015 at a single institution. RESULTS: Two hundred patients (114 males/86 females) were identified. Median age at resection (±SD) was 9.025 ± 5.720 years and mean follow-up was 4 ± 2 years. Resection was gross total in 82 patients (41%) and partial in 118 patients (59%); 66 patients (33%) experienced preoperative seizures, and 67 patients (34%) experienced postoperative seizures; 18 patients (27%) had early seizures, and 49 patients (73%) had late seizures. Univariate analysis identified risk factors for postoperative seizures as: preoperative seizures (P < 0.001), age less than 2 years (P = 0.003), temporal location (P < 0.001), thalamic location (P = 0.017), preoperative hyponatremia (P = 0.017), World Health Organization grade (P = 0.008), and pathology (P = 0.005). Multivariate regression identified 5 robust risk factors: temporal location (odds ratio [OR] 4.7, 95% CI: 1.7–13.3, P = 0.003), age 0.05). CONCLUSIONS: This study reveals 5 risk factors for postoperative seizures after resection of supratentorial tumors. These factors should be considered in postoperative management of these patients.

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.

INTRODUCTION: Diffuse intrinsic pontine glioma (DIPG) remains a devastating and incurable disease. The DIPG-BATs clinical trial incorporates diagnostic biopsy with molecularly determined treatment stratification. Here we present the initial genomic analysis of the DIPG-BATs cohort. METHODS: Children enrolled on the DIPG-BATs clinical trial underwent upfront diagnostic biopsies prior to commencement of therapy. RNA and DNA were extracted from single core biopsies and subjected to whole-genome sequencing (WGS) and RNA-sequencing. Tumor samples were also collected at autopsy if there was parental consent. RESULTS: Fifty-three patients were enrolled on study, of whom 50 underwent biopsy. There were no biopsy-related deaths. A mean of 5ug of RNA and 10ug of DNA were extracted from single frozen cores. WGS on 41 DIPG samples (including eight autopsy samples) revealed a mean mutation rate of 0.753 mutations per Mb. We confirmed TP53, PIK3CA, H3F3A, ACVR1, PPM1D, and HIST1H3B to be recurrently mutated in DIPG. Additional mutations were found in epigenetic modifiers including ASXL1. Copy-number analysis revealed PDGFRA to meet statistical significance as a recurrent amplification peak in DIPG (q