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Objective: Patients with a history of surgery for single-suture craniosynostosis (SSC) as an infant often wish to participate in sports later in childhood. However, there are no established guidelines from neurosurgeons and craniofacial surgeons to guide parents in which sports their child should or should not participate. Therefore, this study aimed to evaluate the attitudes and practice patterns of experienced neurosurgeons and craniofacial surgeons regarding the counseling of caregivers of these patients about sports participation., Methods: A survey was administered to neurosurgeons and craniofacial plastic surgeons of the Synostosis Research Group (SynRG), a group of 9 North American institutions, to identify attitudes toward sports participation in patients with past SSC surgery. Survey responses were collected anonymously in REDCap. Questions regarding specific sports participation recommendations for patients who underwent surgery as an infant for SSC with ideal healing and for those who required a delayed cranioplasty were answered. Questions pertained to patients with nonsyndromic SSC without associated Chiari malformation, syrinx, or other intracranial/intraspinal anomalies., Results: Overall, 20 surgeons were invited to participate in the survey, with 18 (90%) (9 neurosurgeons and 9 craniofacial plastic surgeons) fully completing it. Only 1 (5.6%) surgeon counseled against any sports participation for patients with ideal healing. If cranioplasty was required, 39%-50% of surgeons counseled against some participation (most commonly restricting football/rugby, boxing, ice hockey, lacrosse, and wrestling), depending on the extent of the cranioplasty. Overall, more plastic surgeons (56%-67%) counseled against sports participation (including lower-contact sports such as baseball/softball, basketball, gymnastics, and soccer) than neurosurgeons (22%-33%) in patients who required cranioplasty., Conclusions: SynRG surgeons generally did not counsel against sports participation (including contact sports) for children with a history of SSC surgery as an infant who had ideal healing. In patients requiring cranioplasty, 39%-50% of surgeons recommended against high-contact sports participation.

Purpose: This Hydrocephalus Clinical Research Network (HCRN) study had two aims: (1) to compare the predictive performance of the original ETV Success Score (ETVSS) using logistic regression modeling with other newer machine learning models and (2) to assess whether inclusion of imaging variables improves prediction performance using machine learning models., Methods: We identified children undergoing first-time ETV for hydrocephalus that were enrolled prospectively at HCRN sites between 200 and 2020. The primary outcome was ETV success 6 months after index surgery. The cohort was randomly divided into training (70%) and testing (30%) datasets. The classic ETVSS variables were used for logistic regression and machine learning models. Predictive performance of each model was evaluated on the testing dataset using area under the receiver operating characteristic curve (AUROC)., Results: There were 752 patients that underwent first time ETV, of which 185 patients (24.6%) experienced ETV failure within 6 months. For aim 1, using the classic ETVSS variables, machine learning models did not outperform logistic regression with AUROC 0.60 (95% CI: 0.52-0.69) for Naïve Bayes (highest machine learning model performance) and 0.68 (95% CI: 0.60-0.76) for logistic regression. After inclusion of imaging features (aim 2), machine learning model prediction improved but remained no better than the above logistic regression with the highest AUROC of 0.67 (95% CI: 0.59-0.75) attained using Naïve Bayes architecture compared to 0.68 (95% CI: 0.59-0.76) for logistic regression., Conclusions: This contemporary multicenter observational cohort study demonstrated that machine learning modeling strategies did not improve performance of the ETVSS model over logistic regression., Competing Interests: Declarations. Competing interests: The authors declare no competing interests., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Study Design: Systematic review., Objective: To identify commonly reported indications and outcomes in spinal column shortening (SCS) procedures., Background: SCS is a surgical procedure used in patients with tethered cord syndrome-characterized by abnormal attachment of neural components to surrounding tissues-to shorten the vertebral column, release tension on the spinal cord/neural elements, and alleviate associated symptoms., Patients and Methods: PubMed and EMBASE searches captured SCS literature published between 1950 and 2023. Prospective/retrospective cohort studies and case series were included without age limit or required follow-up period. Review articles without new patient presentations, meta-analyses, systematic reviews, conference abstracts, and letters were excluded. Studies included adult and pediatric patients., Results: The 29 identified studies represented 278 patients (aged 5-76 yr). In 24.1% of studies, patients underwent primary tethered cord syndrome intervention through SCS. In 41.4% of studies, patients underwent SCS after failed previous primary detethering (24.1% of studies were mixed and 10.3% were unspecified). The most commonly reported nongenitourinary/bowel surgical indications were back pain (55.2%), lower-extremity pain (48.3%), lower-extremity weakness (48.3%), lower-extremity numbness (34.5%), and lower-extremity motor dysfunction (34.5%). Genitourinary/bowel symptoms were most often described as nonspecific bladder dysfunction (58.6%), bladder incontinence (34.5%), and bowel dysfunction (31.0%). After SCS, nongenitourinary/bowel outcomes included lower-extremity pain (44.8%), back pain (31.0%), and lower-extremity sensory and motor function (both 31.0%). Bladder dysfunction (79.3%), bowel dysfunction (34.5%), and bladder incontinence (13.8%) were commonly reported genitourinary/bowel outcomes. In total, 40 presenting surgical indication categories and 33 unique outcome measures were reported across studies. Seventeen of the 278 patients (6.1%) experienced a complication., Conclusion: The SCS surgical literature displays variability in operative indications and postoperative outcomes. The lack of common reporting mechanisms impedes higher-level analysis. A standardized outcomes measurement tool, encompassing both patient-reported outcome measures and objective metrics, is necessary., Level of Evidence: Level IV., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: The Subaxial Cervical Spine Injury Classification (SLIC) score has not been previously validated for a pediatric population. The authors compared the SLIC treatment recommendations for pediatric subaxial cervical spine trauma with real-world pediatric spine surgery practice., Methods: A retrospective cohort study at a pediatric level 1 trauma center was conducted in patients < 18 years of age evaluated for trauma from 2012 to 2021. An SLIC score was calculated for each patient, and the subsequent recommendations were compared with actual treatment delivered. Percentage misclassification, sensitivity, specificity, positive (PPV) and negative predictive value (NPV), and area under the receiver operating characteristic (ROC) curve (AUC) were calculated., Results: Two hundred forty-three pediatric patients with trauma were included. Twenty-five patients (10.3%) underwent surgery and 218 were managed conservatively. The median SLIC score was 2 (interquartile range = 2). Sixteen patients (6.6%) had an SLIC score of 4, for which either conservative or surgical treatment is recommended; 27 children had an SLIC score ≥ 5, indicating a recommendation for surgical treatment; and 200 children had an SLIC score ≤ 3, indicating a recommendation for conservative treatment. Of the 243 patients, 227 received treatment consistent with SLIC score recommendations (p < 0.001). SLIC sensitivity in determining surgically treated patients was 79.2% and the specificity for accurately determining who underwent conservative treatment was 96.1%. The PPV was 70.3% and the NPV was 97.5%. There was a 5.7% misclassification rate (n = 13) using SLIC. Among patients for whom surgical treatment would be recommended by the SLIC, 29.6% (n = 8) did not undergo surgery; similarly, 2.5% (n = 5) of patients for whom conservative management would be recommended by the SLIC had surgery. The ROC curve for determining treatment received demonstrated excellent discriminative ability, with an AUC of 0.96 (OR 3.12, p < 0.001). Sensitivity decreased when the cohort was split by age (< 10 and ≥ 10 years old) to 0.5 and 0.82, respectively; specificity remained high at 0.98 and 0.94., Conclusions: The SLIC scoring system recommended similar treatment when compared with the actual treatment delivered for traumatic subaxial cervical spine injuries in children, with a low misclassification rate and a specificity of 96%. These findings demonstrate that the SLIC can be useful in guiding treatment for pediatric patients with subaxial cervical spine injuries. Further investigation into the score in young children (< 10 years) using a multicenter cohort is warranted.

Objective: When the peritoneal cavity cannot serve as the distal shunt terminus, nonperitoneal shunts, typically terminating in the atrium or pleural space, are used. The comparative effectiveness of these two terminus options has not been evaluated. The authors directly compared shunt survival and complication rates for ventriculoatrial (VA) and ventriculopleural (VPl) shunts in a pediatric cohort., Methods: The Hydrocephalus Clinical Research Network Core Data Project was used to identify children ≤ 18 years of age who underwent either VA or VPl shunt insertion. The primary outcome was time to shunt failure. Secondary outcomes included distal site complications and frequency of shunt failure at 6, 12, and 24 months., Results: The search criteria yielded 416 children from 14 centers with either a VA (n = 318) or VPl (n = 98) shunt, including those converted from ventriculoperitoneal shunts. Children with VA shunts had a lower median age at insertion (6.1 years vs 12.4 years, p < 0.001). Among those children with VA shunts, a hydrocephalus etiology of intraventricular hemorrhage (IVH) secondary to prematurity comprised a higher proportion (47.0% vs 31.2%) and myelomeningocele comprised a lower proportion (17.8% vs 27.3%) (p = 0.024) compared with those with VPl shunts. At 24 months, there was a higher cumulative number of revisions for VA shunts (48.6% vs 38.9%, p = 0.038). When stratified by patient age at shunt insertion, VA shunts in children < 6 years had the lowest shunt survival rate (p < 0.001, log-rank test). After controlling for age and etiology, multivariable analysis did not find that shunt type (VA vs VPl) was predictive of time to shunt failure. No differences were found in the cumulative frequency of complications (VA 6.0% vs VPl 9.2%, p = 0.257), but there was a higher rate of pneumothorax in the VPl cohort (3.1% vs 0%, p = 0.013)., Conclusions: Shunt survival was similar between VA and VPl shunts, although VA shunts are used more often, particularly in younger patients. Children < 6 years with VA shunts appeared to have the shortest shunt survival, which may be a result of the VA group having more cases of IVH secondary to prematurity; however, when age and etiology were included in a multivariable model, shunt location (atrium vs pleural space) was not associated with time to failure. The baseline differences between children treated with a VA versus a VPl shunt likely explain current practice patterns.

Objective: As many as 5% of normocephalic children may have a prematurely fused sagittal suture, yet the clinical significance and best course of management of this finding remain unclear. Providers in the Synostosis Research Group were surveyed to create a multicenter consensus on an optimal treatment and monitoring algorithm for this condition., Methods: A four-round modified Delphi method was utilized. The first two rounds consisted of anonymous surveys distributed to 10 neurosurgeons and 9 plastic surgeons with expertise in craniosynostosis across 9 institutions, and presented 3 patients (aged 3 years, 2 years, and 2 months) with incidentally discovered fused sagittal sutures, normal cephalic indices, and no parietal dysmorphology. Surgeons were queried about their preferred term for this entity and how best to manage these patients. Results were synthesized to create a treatment algorithm. The third and fourth feedback rounds consisted of open discussion of the algorithm until no further concerns arose., Results: Most surgeons preferred the term "premature fusion of the sagittal suture" (93%). At the conclusion of the final round, all surgeons agreed to not operate on the 3- and 2-year-old patients unless symptoms of intracranial hypertension or papilledema were present. In contrast, 50% preferred to operate on the 2-month-old. However, all agreed to utilize shared decision-making, taking into account any concerns about future head shape and neurodevelopment. Panelists agreed that patients over 18 months of age without signs or symptoms suggesting elevated intracranial pressure (ICP) should not undergo surgical treatment., Conclusions: Through the Delphi method, a consensus regarding management of premature fusion of the sagittal suture was obtained from a panel of North American craniofacial surgeons. Without signs or symptoms of ICP elevation, surgery is not recommended in patients over 18 months of age. However, for children younger than 18 months, surgery should be discussed with caregivers using a shared decision-making process.

Purpose: Tethered cord syndrome (TCS) is characterized by abnormal attachment of the spinal cord neural elements to surrounding tissues. The most common symptoms include pain, motor or sensory dysfunction, and urologic deficits. Although TCS is common in children, there is a significant heterogeneity in outcomes reporting. We systematically reviewed surgical indications and postoperative outcomes to assess the need for a grading/classification system., Methods: PubMed and EMBASE searches identified pediatric TCS literature published between 1950 and 2023. Studies reporting surgical interventions, ≥ 6-month follow-up, and ≥ 5 patients were included., Results: Fifty-five studies representing 3798 patients were included. The most commonly reported non-urologic symptoms were nonspecific lower-extremity motor disturbances (36.4% of studies), lower-extremity/back pain (32.7%), nonspecific lower-extremity sensory disturbances (29.1%), gait abnormalities (29.1%), and nonspecific bowel dysfunction/fecal incontinence (25.5%). Urologic symptoms were most commonly reported as nonspecific complaints (40.0%). After detethering surgery, retethering was the most widely reported non-urologic outcome (40.0%), followed by other nonspecific findings: motor deficits (32.7%), lower-extremity/back/perianal pain (18.2%), gait/ambulation function (18.2%), sensory deficits (12.7%), and bowel deficits/fecal incontinence (12.7%). Commonly reported urologic outcomes included nonspecific bladder/urinary deficits (27.3%), bladder capacity (20.0%), bladder compliance (18.2%), urinary incontinence/enuresis/neurogenic bladder (18.2%), and nonspecific urodynamics/urodynamics score change (16.4%)., Conclusion: TCS surgical literature is highly variable regarding surgical indications and reporting of postsurgical outcomes. The lack of common data elements and consistent quantitative measures inhibits higher-level analysis. The development and validation of a standardized outcomes measurement tool-ideally encompassing both patient-reported outcome and objective measures-would significantly benefit future TCS research and surgical management., (© 2023. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.)

Objective: The Hydrocephalus Clinical Research Network (HCRN) conducted a prospective study 1) to determine if a new, better-performing version of the Endoscopic Third Ventriculostomy Success Score (ETVSS) could be developed, 2) to explore the performance characteristics of the original ETVSS in a modern endoscopic third ventriculostomy (ETV) cohort, and 3) to determine if the addition of radiological variables to the ETVSS improved its predictive abilities., Methods: From April 2008 to August 2019, children (corrected age ≤ 17.5 years) who underwent a first-time ETV for hydrocephalus were included in a prospective multicenter HCRN study. All children had at least 6 months of clinical follow-up and were followed since the index ETV in the HCRN Core Data Registry. Children who underwent choroid plexus cauterization were excluded. Outcome (ETV success) was defined as the lack of ETV failure within 6 months of the index procedure. Kaplan-Meier curves were constructed to evaluate time-dependent variables. Multivariable binary logistic models were built to evaluate predictors of ETV success. Model performance was evaluated with Hosmer-Lemeshow and Harrell's C statistics., Results: Seven hundred sixty-one children underwent a first-time ETV. The rate of 6-month ETV success was 76%. The Hosmer-Lemeshow and Harrell's C statistics of the logistic model containing more granular age and etiology categorizations did not differ significantly from a model containing the ETVSS categories. In children ≥ 12 months of age with ETVSSs of 50 or 60, the original ETVSS underestimated success, but this analysis was limited by a small sample size. Fronto-occipital horn ratio (p = 0.37), maximum width of the third ventricle (p = 0.39), and downward concavity of the floor of the third ventricle (p = 0.63) did not predict ETV success. A possible association between the degree of prepontine adhesions on preoperative MRI and ETV success was detected, but this did not reach statistical significance., Conclusions: This modern, multicenter study of ETV success shows that the original ETVSS continues to demonstrate good predictive ability, which was not substantially improved with a new success score. There might be an association between preoperative prepontine adhesions and ETV success, and this needs to be evaluated in a future large prospective study.

Introduction: The fibrous posterior atlanto-occipital membrane (PAOM) at the craniocervical junction is typically removed during decompression surgery for Chiari malformation type I (CM-I); however, its importance and ultrastructural architecture have not been investigated in children. We hypothesized that there are structural differences in the PAOM of patients with CM-I and those without., Methods: In this prospective study, blinded pathological analysis was performed on PAOM specimens from children who had surgery for CM-I and children who had surgery for posterior fossa tumors (controls). Clinical and radiographic data were collected. Statistical analysis included comparisons between the CM-I and control cohorts and correlations with imaging measures., Results: A total of 35 children (mean age at surgery 10.7 years; 94.3% white) with viable specimens for evaluation were enrolled: 24 with CM-I and 11 controls. There were no statistical demographic differences between the two cohorts. Four children had a family history of CM-I and five had a syndromic condition. The cohorts had similar measurements of tonsillar descent, syringomyelia, basion to C2, and condylar-to-C2 vertical axis (all p>0.05). The clival-axial angle was lower in patients with CM-I (138.1 vs. 149.3 degrees, p = 0.016). Morphologically, the PAOM demonstrated statistically higher proportions of disorganized architecture in patients with CM-I (75.0% vs. 36.4%, p = 0.012). There were no differences in PAOM fat, elastin, or collagen percentages overall and no differences in imaging or ultrastructural findings between male and female patients. Posterior fossa volume was lower in children with CM-I (163,234 mm3 vs. 218,305 mm3, p<0.001), a difference that persisted after normalizing for patient height (129.9 vs. 160.9, p = 0.028)., Conclusions: In patients with CM-I, the PAOM demonstrates disorganized architecture compared with that of control patients. This likely represents an anatomic adaptation in the presence of CM-I rather than a pathologic contribution., Competing Interests: The authors have declared that no competing interests exist., (Copyright: This is an open access article, free of all copyright, and may be freely reproduced, distributed, transmitted, modified, built upon, or otherwise used by anyone for any lawful purpose. The work is made available under the Creative Commons CC0 public domain dedication.)

Objective: Knowledge-based tools used to standardize perioperative care, such as the shunt infection prevention protocol of the Hydrocephalus Clinical Research Network (HCRN), have demonstrated their ability to reduce surgeon-based and center-based variations in outcomes and improve patient care. The mere presence of high-quality evidence, however, does not necessarily translate into improved patient outcomes owing to the implementation gap. To advance understanding of how knowledge-based tools are being utilized in the routine clinical care of children with hydrocephalus, the HCRN-Quality (HCRNq) network was started in 2019. With a focus on CSF shunt infection, the authors present baseline data regarding CSF shunt infection rates and current shunt infection prevention practices in use at HCRNq sites., Methods: Baseline shunt surgery practices, infection rate, and risk factor data were prospectively collected within HCRNq. No standard infection protocol was recommended, but site use of a protocol was implied if at least 3 of 6 common shunt infection prevention practices were used in > 80% of shunt surgical procedures. Univariable and multivariable analyses of shunt infection risk factors were performed., Results: Thirty sites accrued data on 2437 procedures between November 2019 and June 2021. The unadjusted infection rate across all sites was 3.9% (range 0%-13%) and did not differ among shunt insertion, shunt revision, or shunt insertion after infection. Protocol use was implied for only 15/30 centers and 60% of shunt operations. On univariable analysis, iodine/DuraPrep (OR 0.57, 95% CI 0.37-0.88, p = 0.02) and the use of an antibiotic-impregnated catheter in any segment of the shunt (or both) decreased infection risk (OR 0.53, 95% CI 0.34-0.82, p = 0.01). Iodine-based prep solutions (OR 0.56, 95% 0.36-0.86, p = 0.02) and the use of antibiotic-impregnated catheters (OR 0.52, 95% CI 0.34-0.81, p = 0.01) retained significance in the multivariable model, but no relationship between protocol use and infection risk was demonstrated in this baseline analysis., Conclusions: The authors have demonstrated that children undergoing CSF shunt surgery at HCRNq sites share similar demographic characteristics with other large North American multicenter cohorts, with similar observed baseline infection rates and risk factors. Many centers have implemented standardized shunt infection prevention practices, but considerable practice variation remains. As such, there is an opportunity to decrease shunt infection rates in these centers through continued standardization of care.

Purpose: Pediatric hydrocephalus is a common and challenging condition. To date, the ventriculoperitoneal shunt (VPS) is still the main lifesaving treatment option. Nonetheless, it remains imperfect and is associated with multiple short- and long-term complications. This paper is a reflective review of the current state of the VPS, our knowledge gaps, and the future state of shunts in neurosurgical practice., Methods and Results: The authors' reflections are based on a review of shunts and shunt-related literature., Conclusion: Overall, there is still an urgent need for the neurosurgical community to actively improve current strategies for shunt failures and shunt-related morbidity. The authors emphasize the role of collaborative efforts amongst like-minded clinicians to establish pragmatic approaches to avoid shunt complications., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Abdominal pseudocyst (APC) can cause distal site failure in children with ventriculoperitoneal shunts and is specifically designated as an infection in Hydrocephalus Clinical Research Network (HCRN) protocols. Specific management and outcomes of children with APCs have not been reported in a multicenter study. In this study, the authors investigated the management and outcomes of APC in children with shunted hydrocephalus who were treated at centers in the HCRN., Methods: The HCRN Registry was queried to identify children < 18 years old with shunts who were diagnosed with an APC (i.e., a loculated abdominal fluid collection containing the peritoneal catheter with abdominal distention and/or displacement of peritoneal contents). The primary outcome was shunt failure after APC treatment. The primary variable was reimplantation of the distal catheter after pseudocyst treatment back into the peritoneum versus implantation in a nonperitoneal site. Other risk factors for shunt failure after APC treatment and variability in APC management were investigated., Results: Among 141 children from 14 centers who underwent first-time management of an APC over a 14-year period, the median time from previous shunt surgery to APC diagnosis was 3.8 months. Overall, 17.7% of children had a positive culture: APC cultures were positive in 14.2% and CSF cultures in 15.6%. Six other children underwent shunt revision without removal; all underwent reoperation within 1 month. There was no difference in shunt survival (log-rank test, p = 0.42) or number of subsequent revisions within 6, 12, or 24 months for shunts reimplanted in the abdomen versus those implanted in a nonperitoneal location. Nonperitoneal implantation was associated with more noninfectious revisions (42.3% vs 22.9%, p = 0.019), whereas infection was more common after reimplantation in the abdomen (25.7% vs 7.0%, p = 0.003). Univariable analysis demonstrated that younger age at APC diagnosis (8.3 vs 12.2 years, p = 0.006) and prior shunt procedure within 12 weeks of APC diagnosis (59.5% vs 40.5%, p = 0.012) were associated with shunt failure after APC treatment. Multivariable modeling confirmed that prior shunt surgery within 12 weeks of APC diagnosis was independently associated with failure (HR 1.79 [95% CI 1.04-3.07], p = 0.035)., Conclusions: In the HCRN, APCs in the setting of CSF shunts are usually managed with externalization. Shunt surgery within 12 weeks of APC diagnosis was associated with risk of failure after APC treatment. Although no differences were found in overall shunt failure rate, noninfectious shunt revisions were more common in the nonperitoneal distal catheter sites, and infection was a more common reason for failure after reimplantation of the shunt in the abdomen.

Objective: Persistent hydrocephalus following posterior fossa brain tumor (PFBT) resection is a common cause of morbidity in pediatric brain tumor patients, for which the optimal treatment is debated. The purpose of this study was to compare treatment outcomes between VPS and ETV in patients with persistent hydrocephalus following surgical resection of a PFBT., Methods: A post-hoc analysis was performed of the Hydrocephalus Clinical Research Network (HCRN) prospective observational study evaluating VPS and ETV for pediatric patients. Children who experienced hydrocephalus secondary to PFBT from 2008 to 2021 were included. Primary outcomes were VPS/ETV treatment failure and time-to-failure (TTF)., Results: Among 241 patients, the VPS (183) and ETV (58) groups were similar in age, extent of tumor resection, and preoperative ETV Success Score. There was no difference in overall treatment failure between VPS and ETV (33.9% vs 31.0%, p = 0.751). However, mean TTF was shorter for ETV than VPS (0.45 years vs 1.30 years, p = 0.001). While major complication profiles were similar, compared to VPS, ETV patients had relatively higher incidence of minor CSF leak (10.3% vs. 1.1%, p = 0.003) and pseudomeningocele (12.1% vs 3.3%, p = 0.02). No ETV failures were identified beyond 3 years, while shunt failures occurred beyond 5 years. Shunt infections occurred in 5.5% of the VPS cohort., Conclusions: ETV and VPS offer similar overall success rates for PFBT-related postoperative hydrocephalus. ETV failure occurs earlier, while susceptibility to VPS failure persists beyond 5 years. Tumor histology and grade may be considered when selecting the optimal means of CSF diversion., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Ependymomas account for 10% of all malignant pediatric central nervous system tumors. Standard therapy includes maximal safe surgical resection, followed by focal radiation. Despite the aggressive therapy, progression-free survival is poor. Most ependymoma relapses occur locally at the original tumor site. Extraneural presentations of ependymoma are extremely rare, and no standard of care treatment exists. We present a single-institution case series of 3 patients who experienced extraneural relapses of supratentorial ependymoma and describe their treatment and outcome. These cases of extraneural relapse highlight the possible modes of extraneural spread, including hematogenous, lymphatic, and microscopic seeding through surgical drains and shunts. In addition, they illustrate the increase in histologic grade and mutational burden that may occur at the time of relapse. These cases illustrate the role of aggressive, individualized treatment interventions using a combination of surgery, radiation, and chemotherapy., Competing Interests: The authors declare no conflict of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives Despite their comparative rarity, about 10,000 ischemic strokes occur in children every year, and no standardized method of treatment exists. Protocols have been effective at increasing diagnosis accuracy and treatment efficacy in adults, but little has been done to evaluate such tools in children. A survey was developed to identify the proportion of pediatric hospitals that have stroke protocols and analyze the components used for diagnosis and treatment to identify consensus. Materials and methods Physicians at 50 pediatric hospitals that contributed to the Pediatric Hospital Inpatient Sample in specialties involved in the treatment of stroke (i.e, neurology, neurosurgery, radiology, pediatric intensive care, and emergency medicine) were invited in a purposive and referral manner to complete and 18-question survey. Consensus agreement was predefined as >75%. Results Of 264 surveys distributed, 93 (35%) were returned, accounting for 46 (92%) hospitals. Among the respondents, 76 (82%) reported the presence of a pediatric stroke protocol at their hospital. Consensus agreement was reached in 9 components, including the use of intravenous tissue plasminogen activator (90%) and mechanical thrombectomy (77%) as treatments for acute stroke. Consensus agreement was not reached in 10 components, including the use of prehospital (16%) and emergency department (59%) screening tools and a centralized contact method (57%). Conclusions Pediatric ischemic stroke is a potentially devastating disease that is potentially reversible if treated early. Most pediatric hospitals have developed stroke protocols to aid in diagnosis and treatment, but there is a lack of consensus on what the protocols should contain., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: Surgical treatment of sagittal craniosynostosis is challenging in older patients. This study aimed to assess the effect of increasing age on open surgical technique selection and patient outcomes using the multi-institutional Synostosis Research Group (SynRG) collaboration., Methods: Surgeons in SynRG were surveyed for key influences on their preferred open calvarial vault remodeling techniques at various patient ages: < 6, 6-12, and > 12 months. The SynRG database was then queried for open repairs of nonsyndromic sagittal craniosynostosis performed for patients older than 12 months of age. Perioperative measures, complications, and preoperative and postoperative cephalic indices were reviewed., Results: All surgeons preferred to treat patients at an earlier age, and most (89%) believed that less-optimal outcomes were achieved at ages older than 12 months. The modified pi procedure was the dominant technique in those younger than 12 months, while more involved open surgical techniques were performed for older patients, with a wide variety of open calvarial vault remodeling techniques used. Forty-four patients met inclusion criteria, with a mean (± SD) age at surgery of 29 ± 16 months. Eleven patients underwent parietal reshaping, 10 parietal-occipital switch, 9 clamshell craniotomy, 7 geometric parietal expansion, 6 modified pi procedure, and 1 parietal distraction. There were no readmissions, complications, or mortality within 30 days postoperatively. Patients' cephalic indices improved a mean of 6.4% ± 4.0%, with a mean postoperative cephalic index of 74.2% ± 4.9%. Differences in postoperative cephalic index (p < 0.04) and hospital length of stay (p = 0.01) were significant between technique cohorts. Post hoc Tukey-Kramer analysis identified the parietal reshaping technique as being significantly associated with a reduced hospital length of stay., Conclusions: Patient age is an important driver in technique selection, with surgeons selecting a more involved calvarial vault remodeling technique in older children. A variety of surgical techniques were analyzed, with the parietal reshaping technique being significantly associated with reduced length of stay; however, multiple perioperative factors may be contributory and require further analysis. When performed at high-volume centers by experienced pediatric neurosurgeons and craniofacial surgeons, open calvarial vault techniques can be a safe method for treating sagittal craniosynostosis in older children.

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: 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.

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.

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: 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: In pediatric hydrocephalus, shunts tend to result in smaller postoperative ventricles compared with those following an endoscopic third ventriculostomy (ETV). The impact of the final treated ventricle size on neuropsychological and quality-of-life outcomes is currently undetermined. Therefore, the authors sought to ascertain whether treated ventricle size is associated with neurocognitive and academic outcomes postoperatively., Methods: This prospective cohort study included children aged 5 years and older at the first diagnosis of hydrocephalus at 8 Hydrocephalus Clinical Research Network sites from 2011 to 2015. The treated ventricle size, as measured by the frontal and occipital horn ratio (FOR), was compared with 25 neuropsychological tests 6 months postoperatively after adjusting for age, hydrocephalus etiology, and treatment type (ETV vs shunt). Pre- and posttreatment grade point average (GPA), quality-of-life measures (Hydrocephalus Outcome Questionnaire [HOQ]), and a truncated preoperative neuropsychological battery were also compared with the FOR., Results: Overall, 60 children were included with a mean age of 10.8 years; 17% had ≥ 1 comorbidity. Etiologies for hydrocephalus were midbrain lesions (37%), aqueductal stenosis (22%), posterior fossa tumors (13%), and supratentorial tumors (12%). ETV (78%) was more commonly used than shunting (22%). Of the 25 neuropsychological tests, including full-scale IQ (q = 0.77), 23 tests showed no univariable association with postoperative ventricle size. Verbal learning delayed recall (p = 0.006, q = 0.118) and visual spatial judgment (p = 0.006, q = 0.118) were negatively associated with larger ventricles and remained significant after multivariate adjustment for age, etiology, and procedure type. However, neither delayed verbal learning (p = 0.40) nor visual spatial judgment (p = 0.22) was associated with ventricle size change with surgery. No associations were found between postoperative ventricle size and either GPA or the HOQ., Conclusions: Minimal associations were found between the treated ventricle size and neuropsychological, academic, or quality-of-life outcomes for pediatric patients in this comprehensive, multicenter study that encompassed heterogeneous hydrocephalus etiologies.

Objective: Hydrocephalus can impact all areas of health, including physical, cognitive, and social-emotional functioning. The social-emotional health of children who have had surgery for hydrocephalus is not well characterized. In this study, the authors sought to examine social-emotional functioning using the Behavior Assessment System for Children, Third Edition (BASC-3) and the Hydrocephalus Outcome Questionnaire (HOQ) in 66 children aged 5 to 17 years., Methods: Caregivers of pediatric patients with hydrocephalus completed the BASC-3 and the HOQ. BASC-3 internalizing, externalizing, and executive functioning caregiver-reported scores were compared with the BASC-3 normative sample using one-sample t-tests to evaluate overall social-emotional functioning. BASC-3 scores were correlated with the social-emotional domain of the HOQ using Pearson's r to determine if the HOQ accurately captured the social-emotional functioning of children with hydrocephalus in a neurosurgery setting. BASC-3 and HOQ scores of children with different etiologies of hydrocephalus were compared using the Kruskal-Wallis one-way analysis of variance to determine if differences existed between the following etiologies: intraventricular hemorrhage secondary to prematurity, myelomeningocele, communicating congenital hydrocephalus, aqueductal stenosis, or other., Results: Children with hydrocephalus of all etiologies had more difficulties with social-emotional functioning compared with normative populations. Children with different hydrocephalus etiologies differed in executive functioning and overall HOQ scores but not in internalizing symptoms, externalizing symptoms, or social-emotional HOQ scores. The social-emotional domain of the HOQ correlated more strongly with the BASC-3 than did the physical and cognitive domains., Conclusions: These results have provided evidence that children who have had surgery for hydrocephalus may be at increased risk of social-emotional and behavioral difficulties, but etiology may not be particularly helpful in predicting what kinds or degree of difficulty. The results of this study also support the convergent and divergent validity of the social-emotional domain of the HOQ.

Objective: Iron has been implicated in the pathogenesis of brain injury and hydrocephalus after preterm germinal matrix hemorrhage-intraventricular hemorrhage, however, it is unknown how external or endogenous intraventricular clearance of iron pathway proteins affect the outcome in this group., Methods: This prospective multicenter cohort included patients with posthemorrhagic hydrocephalus (PHH) who underwent (1) temporary and permanent cerebrospinal fluid (CSF) diversion and (2) Bayley Scales of Infant Development-III testing around 2 years of age. CSF proteins in the iron handling pathway were analyzed longitudinally and compared to ventricle size and neurodevelopmental outcomes., Results: Thirty-seven patients met inclusion criteria with a median estimated gestational age at birth of 25 weeks; 65% were boys. Ventricular CSF levels of hemoglobin, iron, total bilirubin, and ferritin decreased between temporary and permanent CSF diversion with no change in CSF levels of ceruloplasmin, transferrin, haptoglobin, and hepcidin. There was an increase in CSF hemopexin during this interval. Larger ventricle size at permanent CSF diversion was associated with elevated CSF ferritin (p = 0.015) and decreased CSF hemopexin (p = 0.007). CSF levels of proteins at temporary CSF diversion were not associated with outcome, however, higher CSF transferrin at permanent CSF diversion was associated with improved cognitive outcome (p = 0.015). Importantly, longitudinal change in CSF iron pathway proteins, ferritin (decrease), and transferrin (increase) were associated with improved cognitive (p = 0.04) and motor (p = 0.03) scores and improved cognitive (p = 0.04), language (p = 0.035), and motor (p = 0.008) scores, respectively., Interpretation: Longitudinal changes in CSF transferrin (increase) and ferritin (decrease) are associated with improved neurodevelopmental outcomes in neonatal PHH, with implications for understanding the pathogenesis of poor outcomes in PHH. ANN NEUROL 2021;90:217-226., (© 2021 American Neurological Association.)

Objective: The diagnosis of single-suture craniosynostosis can be made by physical examination, but the use of confirmatory imaging is common practice. The authors sought to investigate preoperative imaging use and to describe intracranial findings in children with single-suture synostosis from a large, prospective multicenter cohort., Methods: In this study from the Synostosis Research Group, the study population included children with clinically diagnosed single-suture synostosis between March 1, 2017, and October 31, 2020, at 5 institutions. The primary analysis correlated the clinical diagnosis and imaging diagnosis; secondary outcomes included intracranial findings by pathological suture type., Results: A total of 403 children (67% male) were identified with single-suture synostosis. Sagittal (n = 267), metopic (n = 77), coronal (n = 52), and lambdoid (n = 7) synostoses were reported; the most common presentation was abnormal head shape (97%), followed by a palpable or visible ridge (37%). Preoperative cranial imaging was performed in 90% of children; findings on 97% of these imaging studies matched the initial clinical diagnosis. Thirty-one additional fused sutures were identified in 18 children (5%) that differed from the clinical diagnosis. The most commonly used imaging modality by far was CT (n = 360), followed by radiography (n = 9) and MRI (n = 7). Most preoperative imaging was ordered as part of a protocolized pathway (67%); some images were obtained as a result of a nondiagnostic clinical examination (5.2%). Of the 360 patients who had CT imaging, 150 underwent total cranial vault surgery and 210 underwent strip craniectomy. The imaging findings influenced the surgical treatment 0.95% of the time. Among the 24% of children with additional (nonsynostosis) abnormal findings on CT, only 3.5% required further monitoring., Conclusions: The authors found that a clinical diagnosis of single-suture craniosynostosis and the findings on CT were the same with rare exceptions. CT imaging very rarely altered the surgical treatment of children with single-suture synostosis.

Purpose: While conventional statistical approaches have been used to identify risk factors for cerebrospinal fluid (CSF) shunt failure, these methods may not fully capture the complex contribution of clinical, radiologic, surgical, and shunt-specific variables influencing this outcome. Using prospectively collected data from the Hydrocephalus Clinical Research Network (HCRN) patient registry, we applied machine learning (ML) approaches to create a predictive model of CSF shunt failure., Methods: Pediatric patients (age < 19 years) undergoing first-time CSF shunt placement at six HCRN centers were included. CSF shunt failure was defined as a composite outcome including requirement for shunt revision, endoscopic third ventriculostomy, or shunt infection within 5 years of initial surgery. Performance of conventional statistical and 4 ML models were compared., Results: Our cohort consisted of 1036 children undergoing CSF shunt placement, of whom 344 (33.2%) experienced shunt failure. Thirty-eight clinical, radiologic, surgical, and shunt-design variables were included in the ML analyses. Of all ML algorithms tested, the artificial neural network (ANN) had the strongest performance with an area under the receiver operator curve (AUC) of 0.71. The ANN had a specificity of 90% and a sensitivity of 68%, meaning that the ANN can effectively rule-in patients most likely to experience CSF shunt failure (i.e., high specificity) and moderately effective as a tool to rule-out patients at high risk of CSF shunt failure (i.e., moderately sensitive). The ANN was independently validated in 155 patients (prospectively collected, retrospectively analyzed)., Conclusion: These data suggest that the ANN, or future iterations thereof, can provide an evidence-based tool to assist in prognostication and patient-counseling immediately after CSF shunt placement.

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: Sagittal synostosis is the most common form of isolated craniosynostosis. Although some centers have reported extensive experience with this condition, most reports have focused on a single center. In 2017, the Synostosis Research Group (SynRG), a multicenter collaborative network, was formed to study craniosynostosis. Here, the authors report their early experience with treating sagittal synostosis in the network. The goals were to describe practice patterns, identify variations, and generate hypotheses for future research., Methods: All patients with a clinical diagnosis of isolated sagittal synostosis who presented to a SynRG center between March 1, 2017, and October 31, 2019, were included. Follow-up information through October 31, 2020, was included. Data extracted from the prospectively maintained SynRG registry included baseline parameters, surgical adjuncts and techniques, complications prior to discharge, and indications for reoperation. Data analysis was descriptive, using frequencies for categorical variables and means and medians for continuous variables., Results: Two hundred five patients had treatment for sagittal synostosis at 5 different sites. One hundred twenty-six patients were treated with strip craniectomy and 79 patients with total cranial vault remodeling. The most common strip craniectomy was wide craniectomy with parietal wedge osteotomies (44%), and the most common cranial vault remodeling procedure was total vault remodeling without forehead remodeling (63%). Preoperative mean cephalic indices (CIs) were similar between treatment groups: 0.69 for strip craniectomy and 0.68 for cranial vault remodeling. Thirteen percent of patients had other health problems. In the cranial vault cohort, 81% of patients who received tranexamic acid required a transfusion compared with 94% of patients who did not receive tranexamic acid. The rates of complication were low in all treatment groups. Five patients (2%) had an unintended reoperation. The mean change in CI was 0.09 for strip craniectomy and 0.06 for cranial vault remodeling; wide craniectomy resulted in a greater change in CI in the strip craniectomy group., Conclusions: The baseline severity of scaphocephaly was similar across procedures and sites. Treatment methods varied, but cranial vault remodeling and strip craniectomy both resulted in satisfactory postoperative CIs. Use of tranexamic acid may reduce the need for transfusion in cranial vault cases. The wide craniectomy technique for strip craniectomy seemed to be associated with change in CI. Both findings seem amenable to testing in a randomized controlled trial.

Objective: Hydrocephalus may be seen in patients with multisuture craniosynostosis and, less commonly, single-suture craniosynostosis. The optimal treatment for hydrocephalus in this population is unknown. In this study, the authors aimed to evaluate the success rate of ventriculoperitoneal shunt (VPS) treatment and endoscopic third ventriculostomy (ETV) both with and without choroid plexus cauterization (CPC) in patients with craniosynostosis., Methods: Utilizing the Hydrocephalus Clinical Research Network (HCRN) Core Data Project (Registry), the authors identified all patients who underwent treatment for hydrocephalus associated with craniosynostosis. Descriptive statistics, demographics, and surgical outcomes were evaluated., Results: In total, 42 patients underwent treatment for hydrocephalus associated with craniosynostosis. The median gestational age at birth was 39.0 weeks (IQR 38.0, 40.0); 55% were female and 60% were White. The median age at first craniosynostosis surgery was 0.6 years (IQR 0.3, 1.7), and at the first permanent hydrocephalus surgery it was 1.2 years (IQR 0.5, 2.5). Thirty-three patients (79%) had multiple different sutures fused, and 9 had a single suture: 3 unicoronal (7%), 3 sagittal (7%), 2 lambdoidal (5%), and 1 unknown (2%). Syndromes were identified in 38 patients (90%), with Crouzon syndrome being the most common (n = 16, 42%). Ten patients (28%) received permanent hydrocephalus surgery before the first craniosynostosis surgery. Twenty-eight patients (67%) underwent VPS treatment, with the remaining 14 (33%) undergoing ETV with or without CPC (ETV ± CPC). Within 12 months after initial hydrocephalus intervention, 14 patients (34%) required revision (8 VPS and 6 ETV ± CPC). At the most recent follow-up, 21 patients (50%) required a revision. The revision rate decreased as age increased. The overall infection rate was 5% (VPS 7%, 0% ETV ± CPC)., Conclusions: This is the largest prospective study reported on children with craniosynostosis and hydrocephalus. Hydrocephalus in children with craniosynostosis most commonly occurs in syndromic patients and multisuture fusion. It is treated at varying ages; however, most patients undergo surgery for craniosynostosis prior to hydrocephalus treatment. While VPS treatment is performed more frequently, VPS and ETV are both reasonable options, with decreasing revision rates with increasing age, for the treatment of hydrocephalus associated with craniosynostosis.

Background: When resident physicians fail to demonstrate appropriate milestone competencies early in their neurologic surgery residency, a plan of remediation is necessary., Methods: Once any psychologic, physical, or behavioral causes of identified knowledge or psychomotor deficiencies have been identified and addressed, the next step is to develop a plan to close these gaps. Specific areas that are assessed for deficits include medical knowledge, clinical reasoning and judgment, clinical skills, time management and organization, interpersonal skills, communication, and professionalism. Specific learning goals and objectives, as well as teaching and learning methods, pertain to the unique areas of deficit, and all of these must be considered with the goal of developing a resident-specific remediation plan., Results: A plan for assessment of the remediation process is described, including an evaluation of what constitutes individual resident remediation success., Conclusions: Finally, a discussion of the prior resident remediation studies across many disciplines is made., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Abstract: Posterior cranial vault distraction is an important modality in the management of craniosynostosis. This surgical technique increases intracranial volume and improves cranial aesthetics. A single procedure is often inadequate in patients with complex multisuture craniosynostosis, as some will go on to develop intracranial hypertension despite the operation. Considering the negative effects of intracranial hypertension, some patients may warrant 2 planned distractions to prevent this scenario from ever occurring. Three patients with complex multiple-suture synostosis and severe intracranial volume restriction (occipital frontal head circumferences [OFCs] <1st percentile) were treated with 2 planned serial posterior cranial vault distractions at the institution between 2013 and 2018. Demographics, intraoperative data, and postoperative distraction data were collected. The OFC was recorded pre- and postdistraction, at 3- and 6-month follow-up appointments. Patients had a corrected average age of 18 weeks at the time of their initial procedure. There was an average of 38 weeks between the end of consolidation and the time for their 2nd distraction procedure. There was an average age of 79 weeks at the time of the 2nd procedure. All patients had a substantial increase in OFC and improvement of the posterior calvarium shape. The average increase in OFC was 5.2 cm after first distraction and 4.3 cm after 2nd distraction. No postoperative complications were encountered. Planned serial posterior cranial vault distraction is a safe and effective strategy for increasing intracranial volume, improving aesthetic appearance, and preventing the consequences of intracranial hypertension in patients with multisuture craniosynostosis and severe intracranial volume restriction., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by Mutaz B. Habal, MD.)

Objective: Analysis of temporal trends in patient populations and procedure types may provide important information regarding the evolution of hydrocephalus treatment. The purpose of this study was to use the Hydrocephalus Clinical Research Network's Core Data Project to identify meaningful trends in patient characteristics and the surgical management of pediatric hydrocephalus over a 9-year period., Methods: The Core Data Project prospectively collected patient and procedural data on the study cohort from 9 centers between 2008 and 2016. Logistic and Poisson regression were used to test for significant temporal trends in patient characteristics and new and revision hydrocephalus procedures., Results: The authors analyzed 10,149 procedures in 5541 patients. New procedures for hydrocephalus (shunt or endoscopic third ventriculostomy [ETV]) decreased by 1.5%/year (95% CI -3.1%, +0.1%). During the study period, new shunt insertions decreased by 6.5%/year (95% CI -8.3%, -4.6%), whereas new ETV procedures increased by 12.5%/year (95% CI 9.3%, 15.7%). Revision procedures for hydrocephalus (shunt or ETV) decreased by 4.2%/year (95% CI -5.2%, -3.1%), driven largely by a decrease of 5.7%/year in shunt revisions (95% CI -6.8%, -4.6%). Concomitant with the observed increase in new ETV procedures was an increase in ETV revisions (13.4%/year, 95% CI 9.6%, 17.2%). Because revisions decreased at a faster rate than new procedures, the Revision Quotient (ratio of revisions to new procedures) for the Network decreased significantly over the study period (p = 0.0363). No temporal change was observed in the age or etiology characteristics of the cohort, although the proportion of patients with one or more complex chronic conditions significantly increased over time (p = 0.0007)., Conclusions: Over a relatively short period, important changes in hydrocephalus care have been observed. A significant temporal decrease in revision procedures amid the backdrop of a more modest change in new procedures appears to be the most notable finding and may be indicative of an improvement in the quality of surgical care for pediatric hydrocephalus. Further studies will be directed at elucidation of the possible drivers of the observed trends.

Objective: Imaging evaluation of the cerebral ventricles is important for clinical decision-making in pediatric hydrocephalus. Although quantitative measurements of ventricular size, over time, can facilitate objective comparison, automated tools for calculating ventricular volume are not structured for clinical use. The authors aimed to develop a fully automated deep learning (DL) model for pediatric cerebral ventricle segmentation and volume calculation for widespread clinical implementation across multiple hospitals., Methods: The study cohort consisted of 200 children with obstructive hydrocephalus from four pediatric hospitals, along with 199 controls. Manual ventricle segmentation and volume calculation values served as "ground truth" data. An encoder-decoder convolutional neural network architecture, in which T2-weighted MR images were used as input, automatically delineated the ventricles and output volumetric measurements. On a held-out test set, segmentation accuracy was assessed using the Dice similarity coefficient (0 to 1) and volume calculation was assessed using linear regression. Model generalizability was evaluated on an external MRI data set from a fifth hospital. The DL model performance was compared against FreeSurfer research segmentation software., Results: Model segmentation performed with an overall Dice score of 0.901 (0.946 in hydrocephalus, 0.856 in controls). The model generalized to external MR images from a fifth pediatric hospital with a Dice score of 0.926. The model was more accurate than FreeSurfer, with faster operating times (1.48 seconds per scan)., Conclusions: The authors present a DL model for automatic ventricle segmentation and volume calculation that is more accurate and rapid than currently available methods. With near-immediate volumetric output and reliable performance across institutional scanner types, this model can be adapted to the real-time clinical evaluation of hydrocephalus and improve clinician workflow.

Introduction: Endoscope-assisted craniectomy and spring-assisted cranioplasty with post-surgical helmet molding are minimally invasive alternatives to the traditional craniosynostosis treatment of open cranial vault remodeling. Families are often faced with deciding between techniques. This study aimed to understand providers' practice patterns in consulting families about surgical options., Methods: An online survey was developed and distributed to 31 providers. The response rate was 84% (26/31)., Results: Twenty-six (100%) respondents offer a minimally invasive surgical option for sagittal craniosynostosis, 21 (81%) for coronal, 20 (77%) for metopic, 18 (69%) for lambdoid, and 12 (46%) for multi-suture. Social issues considered in determining whether to offer a minimally invasive option include anticipated likelihood of compliance (23 = 88%), distance traveled for care (16 = 62%) and financial considerations (6 = 23%). Common tools to explain options include verbal discussion (25 = 96%), 3D reconstructed CT scans (17 = 65%), handouts (13 = 50%), 3D models (12 = 46%), hand drawings (11 = 42%) and slides (10 = 38%). Some respondents strongly (7 = 27%) or somewhat (3 = 12%) encourage a minimally invasive option over open repair. Others indicate they remain neutral (7 = 27%) or tailor their approach to meet perceived needs (8 = 31%). One (4%) somewhat encourages open repair. Despite this variation, all completely (17 = 65%), strongly (5 = 19%) or somewhat agree (4 = 15%) they use shared decision making in presenting surgical options., Conclusion: This survey highlights the range of practice patterns in presenting surgical options to families and reveals possible discrepancies in the extent providers believe they use shared decision making and the extent it is actually used.

Objective: Few 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., Methods: Patients 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., Results: Among 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)., Conclusions: Among 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: Surgical options for managing hydrocephalus secondary to CNS tumors have traditionally included ventriculoperitoneal shunting (VPS) when tumor resection or medical management alone are ineffective. Endoscopic third ventriculostomy (ETV) has emerged as an attractive treatment strategy for tumor-associated hydrocephalus because it offers a lower risk of infection and hardware-related complications; however, relatively little has been written on the topic of ETV specifically for the treatment of tumor-associated hydrocephalus. Here, the authors reviewed the existing literature on the use of ETV in the treatment of tumor-associated hydrocephalus, focusing on the frequency of ETV use and the failure rates in patients with hydrocephalus secondary to CNS tumor., Methods: The authors queried PubMed for the following terms: "endoscopic third ventriculostomy," "tumor," and "pediatric." Papers with only adult populations, case reports, and papers published before the year 2000 were excluded. The authors analyzed the etiology of hydrocephalus and failure rates after ETV, and they compared failure rates of ETV with those of VPS where reported., Results: Thirty-two studies with data on pediatric patients undergoing ETV for tumor-related hydrocephalus were analyzed. Tumors, particularly in the posterior fossa, were reported as the etiology of hydrocephalus in 38.6% of all ETVs performed (984 of 2547 ETVs, range 29%-55%). The ETV failure rate in tumor-related hydrocephalus ranged from 6% to 38.6%, and in the largest studies analyzed (> 100 patients), the ETV failure rate ranged from 10% to 38.6%. The pooled ETV failure rate was 18.3% (199 failures after 1087 procedures). The mean or median follow-up for ETV failure assessment ranged from 6 months to 8 years in these studies. Only 5 studies directly compared ETV with VPS for tumor-associated hydrocephalus, and they reported mixed results in regard to failure rate and time to failure. Overall failure rates appear similar for ETV and VPS over time, and the risk of infection appears to be lower in those patients undergoing ETV. The literature is mixed regarding the need for routine ETV before resection for posterior fossa tumors with associated hydrocephalus., Conclusions: Treatment of tumor-related hydrocephalus with ETV is common and is warranted in select pediatric patient populations. Failure rates are overall similar to those of VPS for tumor-associated hydrocephalus.

Objective: Despite significant advances in diagnostic and surgical techniques, the surgical management of Chiari malformation type I (CM-I) with associated syringomyelia remains controversial, and the type of surgery performed is surgeon dependent. This study's goal was to determine the feasibility of a prospective, multicenter, cohort study for CM-I/syringomyelia patients and to provide pilot data that compare posterior fossa decompression and duraplasty (PFDD) with and without tonsillar reduction., Methods: Participating centers prospectively enrolled children suffering from both CM-I and syringomyelia who were scheduled to undergo surgical decompression. Clinical data were entered into a database preoperatively and at 1-2 weeks, 3-6 months, and 1 year postoperatively. MR images were evaluated by 3 independent, blinded teams of neurosurgeons and neuroradiologists. The primary endpoint was improvement or resolution of the syrinx., Results: Eight clinical sites were chosen based on the results of a published questionnaire intended to remove geographic and surgeon bias. Data from 68 patients were analyzed after exclusions, and complete clinical and imaging records were obtained for 55 and 58 individuals, respectively. There was strong agreement among the 3 radiology teams, and there was no difference in patient demographics among sites, surgeons, or surgery types. Tonsillar reduction was not associated with > 50% syrinx improvement (RR = 1.22, p = 0.39) or any syrinx improvement (RR = 1.00, p = 0.99). There were no surgical complications., Conclusions: This study demonstrated the feasibility of a prospective, multicenter surgical trial in CM-I/syringomyelia and provides pilot data indicating no discernible difference in 1-year outcomes between PFDD with and without tonsillar reduction, with power calculations for larger future studies. In addition, the study revealed important technical factors to consider when setting up future trials. The long-term sequelae of tonsillar reduction have not been addressed and would be an important consideration in future investigations.

Objective: Large experiences with the treatment of pediatric arteriovenous malformations (AVMs) remain relatively rare, with limited data on presentation, treatment, and long-term functional outcomes. Because of the expected long lifespan of children, caregivers are especially interested in outcome measures that assess quality of life. The authors' intention was to describe the long-term functional outcomes of pediatric patients who undergo AVM surgery and to identify predictors of sustained neurological deficits., Methods: The authors analyzed a 21-year retrospective cohort of pediatric patients with intracranial AVMs treated with microsurgery at two institutions. The primary outcome was a persistent neurological deficit at last follow-up. Secondary outcome measures included modified Rankin Scale (mRS) score and independent living., Results: Overall, 97 patients (mean age 11.1 ± 4.5 years; 56% female) were treated surgically for intracranial AVMs (mean follow-up 77.5 months). Sixty-four patients (66%) presented with hemorrhage, and 45 patients (46%) had neurological deficits at presentation. Radiologically, 39% of lesions were Spetzler-Martin grade II. Thirty-seven patients (38%) with persistent neurological deficits at last follow-up were compared with those without deficits; there were no differences in patient age, presenting Glasgow Coma Scale score, AVM size, surgical blood loss, or duration of follow-up. Multivariate analysis demonstrated that a focal neurological deficit on presentation, AVM size > 3 cm, and lesions in eloquent cortex were independent predictors of persistent neurological deficits at long-term follow-up. Overall, 92% of the children had an mRS score ≤ 2 on long-term follow-up., Conclusions: Pediatric patients with AVMs treated with microsurgical resection have good functional and radiological outcomes. There is a high rate (38%) of persistent neurological deficits, which were independently predicted by preoperative deficits, AVMs > 3 cm, and lesions located in eloquent cortex. This information can be useful in counseling families on the likelihood of long-term neurological deficits after cerebral AVM surgery.

Objective: The analysis of resident research productivity in neurosurgery has gained significant recent interest. Resident scholarly output affects departmental productivity, recruitment of future residents, and likelihood of future research careers. To maintain and improve opportunities for resident research, the authors evaluated factors that affect resident attitudes toward neurosurgical research on a national level., Methods: An online survey was distributed to all US neurosurgical residents. Questions assessed interest in research, perceived departmental support of research, and resident-perceived limitations in pursuing research. Residents were stratified based on number of publications above the median (AM; ≥ 14) or below the median (BM; < 14) for evaluation of factors influencing productivity., Results: A total of 278 resident responses from 82 US residency programs in 30 states were included (a 20% overall response rate). Residents predominantly desired future academic positions (53.2%), followed by private practice with some research (40.3%). Residents reported a mean ± SD of 11 ± 14 publications, which increased with postgraduate year level. The most common type of research involved retrospective cohort studies (24%) followed by laboratory/benchtop (19%) and case reports (18%). Residents as a group spent on average 14.1 ± 18.5 hours (median 7.0 hours) a week on research. Most residents (53.6%) had ≥ 12 months of protected research time. Mentorship (92.4%), research exposure (89.9%), and early interest in science (78.4%) had the greatest impact on interest in research while the most limiting factors were time (91.0%), call scheduling (47.1%), and funding/grants (37.1%). AM residents cited research exposure (p = 0.003), neurosurgery conference exposure (p = 0.02), formal research training prior to residency (p = 0.03), internal funding sources (p = 0.05), and software support (p = 0.02) as most important for their productivity. Moreover, more productive residents applied and received a higher number of < $10,000 and ≥ $10,000 grants (p < 0.05). A majority of residents (82.4%) agreed or strongly agreed with pursuing research throughout their professional careers. Overall, about half of residents (49.6%) were encouraged toward continued neurosurgical research, while the rest were neutral (36.7%) or discouraged (13.7%). Free-text responses helped to identify solutions on a departmental, regional, and national level that could increase interest in neurosurgical research., Conclusions: This survey evaluates various factors affecting resident views toward research, which may also be seen in other specialties. Residents remain enthusiastic about neurosurgical research and offer several solutions to the ever-scarce commodities of time and funding within academic medicine.

Background: Previous studies of cerebrospinal fluid (CSF) shunt infection treatment have been limited in size and unable to compare patient and treatment characteristics by infecting organism. Our objective was to describe variation in patient and treatment characteristics for children with first CSF shunt infection, stratified by infecting organism subgroups outlined in the 2017 Infectious Disease Society of America's (IDSA) guidelines., Methods: We studied a prospective cohort of children <18 years of age undergoing treatment for first CSF shunt infection at one of 7 Hydrocephalus Clinical Research Network hospitals from April 2008 to December 2012. Differences between infecting organism subgroups were described using univariate analyses and Fisher's exact tests., Results: There were 145 children whose infections were diagnosed by CSF culture and addressed by IDSA guidelines, including 47 with Staphylococcus aureus, 52 with coagulase-negative Staphylococcus, 37 with Gram-negative bacilli, and 9 with Propionibacterium acnes. No differences in many patient and treatment characteristics were seen between infecting organism subgroups, including age at initial shunt, gender, race, insurance, indication for shunt, gastrostomy, tracheostomy, ultrasound, and/or endoscope use at all surgeries before infection, or numbers of revisions before infection. A larger proportion of infections were caused by Gram-negative bacilli when antibiotic-impregnated catheters were used at initial shunt placement (12 of 23, 52%) and/or subsequent revisions (11 of 23, 48%) compared with all other infections (9 of 68 [13%] and 13 of 68 [19%], respectively). No differences in reinfection were observed between infecting organism subgroups., Conclusions: The organism profile encountered at infection differs when antibiotic-impregnated catheters are used, with a higher proportion of Gram-negative bacilli. This warrants further investigation given increasing adoption of antibiotic-impregnated catheters., (© The Author(s) 2018. Published by Oxford University Press on behalf of The Journal of the Pediatric Infectious Diseases Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objective: Infection is a common complication of cerebrospinal fluid (CSF) shunts, occurring in 6%-20% of children. Although studies are limited, Staphylococcus aureus is thought to cause more rapid and aggressive infection than coagulase-negative Staphylococcus (CONS) or gram-negative organisms. The authors' objective was to evaluate the relationship between the causative organisms of CSF shunt infection and the timing of infection., Methods: The authors performed a retrospective cohort study of children who underwent CSF shunt placement at a tertiary care children's hospital over a 9-year period and subsequently developed a CSF shunt infection. The primary predictor variable was the causative organism recovered from CSF culture, characterized as S. aureus, CONS, or gram-negative organisms. The primary outcome was time to infection, defined as the number of days from most recent shunt intervention to the diagnosis of the infection. The association between causative organism and time to infection was visualized using Kaplan-Meier curves, and statistical comparisons were made using nonparametric Kruskal-Wallis tests., Results: Among 103 children in whom a CSF shunt infection developed, the causative organism was CONS in 57 (55%), S. aureus in 19 (18%), and gram-negative organisms in 9 (9%). The median time to infection did not differ (p = 0.81) for infections caused by CONS (20 days, IQR 11-40), S. aureus (26 days, IQR 12-95), and gram-negative organisms (23 days, IQR 17-34)., Conclusions: No significant difference in time to infection based on the causative organism was observed among children with a CSF shunt infection.

Objective: Endoscopic third ventriculostomy combined with choroid plexus cauterization (ETV+CPC) has been adopted by many pediatric neurosurgeons as an alternative to placing shunts in infants with hydrocephalus. However, reported success rates have been highly variable, which may be secondary to patient selection, operative technique, and/or surgeon training. The objective of this prospective multicenter cohort study was to identify independent patient selection, operative technique, or surgical training predictors of ETV+CPC success in infants., Methods: This was a prospective cohort study nested within the Hydrocephalus Clinical Research Network's (HCRN) Core Data Project (registry). All infants under the age of 2 years who underwent a first ETV+CPC between June 2006 and March 2015 from 8 HCRN centers were included. Each patient had a minimum of 6 months of follow-up unless censored by an ETV+CPC failure. Patient and operative risk factors of failure were examined, as well as formal ETV+CPC training, which was defined as traveling to and working with the experienced surgeons at CURE Children's Hospital of Uganda. ETV+CPC failure was defined as the need for repeat ETV, shunting, or death., Results: The study contained 191 patients with a primary ETV+CPC conducted by 17 pediatric neurosurgeons within the HCRN. Infants under 6 months corrected age at the time of ETV+CPC represented 79% of the cohort. Myelomeningocele (26%), intraventricular hemorrhage associated with prematurity (24%), and aqueductal stenosis (17%) were the most common etiologies. A total of 115 (60%) of the ETV+CPCs were conducted by surgeons after formal training. Overall, ETV+CPC was successful in 48%, 46%, and 45% of infants at 6 months, 1 year, and 18 months, respectively. Young age (< 1 month) (adjusted hazard ratio [aHR] 1.9, 95% CI 1.0-3.6) and an etiology of post-intraventricular hemorrhage secondary to prematurity (aHR 2.0, 95% CI 1.1-3.6) were the only two independent predictors of ETV+CPC failure. Specific subgroups of ages within etiology categories were identified as having higher ETV+CPC success rates. Although training led to more frequent use of the flexible scope (p < 0.001) and higher rates of complete (> 90%) CPC (p < 0.001), training itself was not independently associated (aHR 1.1, 95% CI 0.7-1.8; p = 0.63) with ETV+CPC success., Conclusions: This is the largest prospective multicenter North American study to date examining ETV+CPC. Formal ETV+CPC training was not found to be associated with improved procedure outcomes. Specific subgroups of ages within specific hydrocephalus etiologies were identified that may preferentially benefit from ETV+CPC.

Objective: CSF shunt infection treatment requires both surgical and antibiotic decisions. Using the Hydrocephalus Clinical Research Network (HCRN) Registry and 2004 Infectious Diseases Society of America (IDSA) guidelines that were not proactively distributed to HCRN providers, the authors previously found high adherence to surgical recommendations but poor adherence to intravenous (IV) antibiotic duration recommendations. In general, IV antibiotic duration was longer than recommended. In March 2017, new IDSA guidelines expanded upon the 2004 guidelines by including recommendations for selection of specific antibiotics. The objective of this study was to describe adherence to both 2004 and 2017 IDSA guideline recommendations for CSF shunt infection treatment, and to report reinfection rates associated with adherence to guideline recommendations., Methods: The authors investigated a prospective cohort of children younger than 18 years of age who underwent treatment for first CSF shunt infection at one of 7 hospitals from April 2008 to December 2012. CSF shunt infection was diagnosed by recovery of bacteria from CSF culture (CSF-positive infection). Adherence to 2004 and 2017 guideline recommendations was determined. Adherence to antibiotics was further classified as longer or shorter duration than guideline recommendations. Reinfection rates with 95% confidence intervals (CIs) were generated., Results: There were 133 children with CSF-positive infections addressed by 2004 IDSA guideline recommendations, with 124 at risk for reinfection. Zero reinfections were observed among those whose treatment was fully adherent (0/14, 0% [95% CI 0%-20%]), and 15 reinfections were observed among those whose infection treatment was nonadherent (15/110, 14% [95% CI 8%-21%]). Among the 110 first infections whose infection treatment was nonadherent, 74 first infections were treated for a longer duration than guidelines recommended and 9 developed reinfection (9/74, 12% [95% CI 6%-22%]). There were 145 children with CSF-positive infections addressed by 2017 IDSA guideline recommendations, with 135 at risk for reinfection. No reinfections were observed among children whose treatment was fully adherent (0/3, 0% [95% CI 0%-64%]), and 18 reinfections were observed among those whose infection treatment was nonadherent (18/132, 14% [95% CI 8%-21%])., Conclusions: There is no clear evidence that either adherence to IDSA guidelines or duration of treatment longer than recommended is associated with reduction in reinfection rates. Because IDSA guidelines recommend shorter IV antibiotic durations than are typically used, improvement efforts to reduce IV antibiotic use in CSF shunt infection treatment can and should utilize IDSA guidelines.

Prospective multicenter clinical research studies in pediatric hydrocephalus are relatively rare. They cover a broad spectrum of hydrocephalus topics, including management of intraventricular hemorrhage in premature infants, shunt techniques and equipment, shunt outcomes, endoscopic treatment of hydrocephalus, and prevention and treatment of infection. The research methodologies include randomized trials, cohort studies, and registry-based studies. This review describes prospective multicenter studies in pediatric hydrocephalus since 1990. Many studies have included all forms of hydrocephalus and used device or procedure failure as the primary outcome. Although such studies have yielded useful findings, they might miss important treatment effects in specific subgroups. As multicenter study networks grow, larger patient numbers will allow studies with more focused entry criteria based on known and evolving prognostic factors. In addition, increased use of patient-centered outcomes such as neurodevelopmental assessment and quality of life should be measured and emphasized in study results. Well-planned multicenter clinical studies can significantly affect the care of children with hydrocephalus and will continue to have an important role in improving care for these children and their families.

The bar for clinical research in surgical subspecialties has been rising over the past 10 yr. It is now essential that neurosurgeons are familiar with the principles of evidence-based medicine to evaluate and conduct sound clinical research. In this review, we highlight the importance of formulating a good research question, which serves as the foundation for meaningful investigation.