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Objective: The pediatric neurosurgical community has increasingly recognized the importance of healthcare transition, the process of moving a patient from a pediatric to an adult model of care. However, surveys of pediatric neurosurgeons have revealed that few institutions have formal transition programs. Here, the authors share their preliminary experience with the development of a formal transition pilot program for patients with spina bifida and/or hydrocephalus., Methods: Patients 18 years of age or older with a diagnosis of spina bifida and/or hydrocephalus who were followed by a pediatric neurosurgeon at Connecticut Children's from January 2017 to December 2023 and were recommended to transition to an adult neurosurgeon were retrospectively reviewed. Patients in the informal transition program (ITP) cohort (i.e., the recommendation to transition was made before the formal transition program [FTP] was developed in early 2020) were compared with those in the FTP cohort., Results: Twenty-two patients met inclusion criteria with 7 (31.8%) in the ITP cohort and 15 (68.2%) in the FTP cohort. The median age at the time of the recommendation to transition was similar in both ITP and FTP cohorts (24 [IQR 20-35] years vs 25 [IQR 24-27] years, respectively). Four (57.1%) patients in the ITP cohort had a confirmed visit with an adult neurosurgeon, compared with 13 (86.7%) patients in the FTP cohort (p = 0.274). One patient in the ITP cohort with a failed transition returned to pediatric neurosurgical care, and 1 patient in the FTP cohort required a shunt revision by an adult neurosurgeon within 1 year of the recommendation to transition., Conclusions: Healthcare transition is recognized as a priority within pediatric neurosurgery, but structured, formal transition programs remain underdeveloped. The authors' preliminary experience with a pilot transition program demonstrated that patients who underwent a formal transition were more likely to successfully establish care with an adult neurosurgeon and trended toward less resource utilization.

Background: Cellular senescence can have positive and negative effects on the body, including aiding in damage repair and facilitating tumor growth. Adamantinomatous craniopharyngioma (ACP), the most common pediatric sellar/suprasellar brain tumor, poses significant treatment challenges. Recent studies suggest that senescent cells in ACP tumors may contribute to tumor growth and invasion by releasing a senesecence-associated secretory phenotype. However, a detailed analysis of these characteristics has yet to be completed., Methods: We analyzed primary tissue samples from ACP patients using single-cell, single-nuclei, and spatial RNA sequencing. We performed various analyses, including gene expression clustering, inferred senescence cells from gene expression, and conducted cytokine signaling inference. We utilized LASSO to select essential gene expression pathways associated with senescence. Finally, we validated our findings through immunostaining., Results: We observed significant diversity in gene expression and tissue structure. Key factors such as NFKB, RELA, and SP1 are essential in regulating gene expression, while senescence markers are present throughout the tissue. SPP1 is the most significant cytokine signaling network among ACP cells, while the Wnt signaling pathway predominantly occurs between epithelial and glial cells. Our research has identified links between senescence-associated features and pathways, such as PI3K/Akt/mTOR, MYC, FZD, and Hedgehog, with increased P53 expression associated with senescence in these cells., Conclusions: A complex interplay between cellular senescence, cytokine signaling, and gene expression pathways underlies ACP development. Further research is crucial to understand how these elements interact to create novel therapeutic approaches for patients with ACP., (© The Author(s) 2024. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Background: Surgical revascularization decreases the long-term risk of stroke in children with moyamoya arteriopathy but can be associated with an increased risk of stroke during the perioperative period. Evidence-based approaches to optimize perioperative management are limited and practice varies widely. Using a modified Delphi process, we sought to establish expert consensus on key components of the perioperative care of children with moyamoya undergoing indirect revascularization surgery and identify areas of equipoise to define future research priorities., Methods: Thirty neurologists, neurosurgeons, and intensivists practicing in North America with expertise in the management of pediatric moyamoya were invited to participate in a three-round, modified Delphi process consisting of a 138-item practice patterns survey, anonymous electronic evaluation of 88 consensus statements on a 5-point Likert scale, and a virtual group meeting during which statements were discussed, revised, and reassessed. Consensus was defined as ≥ 80% agreement or disagreement., Results: Thirty-nine statements regarding perioperative pediatric moyamoya care for indirect revascularization surgery reached consensus. Salient areas of consensus included the following: (1) children at a high risk for stroke and those with sickle cell disease should be preadmitted prior to indirect revascularization; (2) intravenous isotonic fluids should be administered in all patients for at least 4 h before and 24 h after surgery; (3) aspirin should not be discontinued in the immediate preoperative and postoperative periods; (4) arterial lines for blood pressure monitoring should be continued for at least 24 h after surgery and until active interventions to achieve blood pressure goals are not needed; (5) postoperative care should include hourly vital signs for at least 24 h, hourly neurologic assessments for at least 12 h, adequate pain control, maintaining normoxia and normothermia, and avoiding hypotension; and (6) intravenous fluid bolus administration should be considered the first-line intervention for new focal neurologic deficits following indirect revascularization surgery., Conclusions: In the absence of data supporting specific care practices before and after indirect revascularization surgery in children with moyamoya, this Delphi process defined areas of consensus among neurosurgeons, neurologists, and intensivists with moyamoya expertise. Research priorities identified include determining the role of continuous electroencephalography in postoperative moyamoya care, optimal perioperative blood pressure and hemoglobin targets, and the role of supplemental oxygen for treatment of suspected postoperative ischemia., (© 2023. Springer Science+Business Media, LLC, part of Springer Nature and Neurocritical Care Society.)

Pediatric patients suffering traumatic brain injuries may require a decompressive craniectomy to accommodate brain swelling by removing a portion of the skull. Once the brain swelling subsides, the preserved calvarial bone flap is ideally replaced as an autograft during a cranioplasty to restore protection of the brain, as it can reintegrate and grow with the patient during immature skeletal development. However, pediatric patients exhibit a high prevalence of calvarial bone flap resorption post-cranioplasty, causing functional and cosmetic morbidity. This review examines possible solutions for mitigating pediatric calvarial bone flap resorption by delineating methods of stimulating mechanosensitive cell populations with mechanical forces. Mechanotransduction plays a critical role in three main cell types involved with calvarial bone repair, including mesenchymal stem cells, osteoblasts, and dural cells, through mechanisms that could be exploited to promote osteogenesis. In particular, physiologically relevant mechanical forces, including substrate deformation, external forces, and ultrasound, can be used as tools to stimulate bone repair in both in vitro and in vivo systems. Ultimately, combating pediatric calvarial flap resorption may require a combinatorial approach using both cell therapy and bioengineering strategies., (© 2023 Wiley Periodicals LLC.)

Objective: Recruitment of diverse and talented students to the field of neurosurgery is key to its continued growth and scientific advancement. Barriers, including poor perceptions and lack of early exposure, can impact recruitment and have been compounded by the ongoing COVID-19 pandemic. This study examines the impact of an inaugural Neurosurgery Research Consortium meeting on premedical students, assessing whether this exposure generated interest and improved perceptions of a career in neurosurgery., Methods: Premedical students were recruited to virtually attend an inaugural Neurosurgery Research Consortium developed by the affiliated medical school's American Association of Neurological Surgeons (AANS) Student Chapter. Questionnaires were distributed to students before and after the meeting to assess student demographics and perceptions of neurosurgery., Results: A total of 54 students attended the meeting, with general interest in neurosurgery, medicine, and research opportunities being the primary factors for attendance. Following the research meeting, we found that students perceived neurosurgeons to be friendlier and more approachable, with a more positive quality of life (QoL). Overall perceptions of neurosurgery improved after the meeting, but perceptions among racial and ethnic minority students did not significantly change in the areas of diversity, inclusion, and equity., Conclusions: These results suggest recruitment strategies targeting undergraduate students may improve their perception of neurosurgery as a career, and may mitigate some barriers to entry. These strategies are cost effective and easily replicable, making an easily implementable approach to provide direct insight into neurosurgery for future medical students while also promoting academic efforts in the field of neurosurgery., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: Septic cerebral venous sinus thrombosis (CVST) is a recognized complication of pediatric sinogenic and otogenic intracranial infections. The optimal treatment paradigm remains controversial. Proponents of anticoagulation highlight its role in preventing thrombus propagation and promoting recanalization, while others cite the risk of hemorrhagic complications, especially after a neurosurgical procedure for an epidural abscess or subdural empyema. Here, the authors investigated the diagnosis, management, and outcomes of pediatric patients with sinogenic or otogenic intracranial infections and a septic CVST., Methods: All patients 21 years of age or younger, who presented with an intracranial infection in the setting of sinusitis or otitis media and who underwent neurosurgical treatment at Connecticut Children's, Rady Children's Hospital-San Diego, or Ann and Robert H. Lurie Children's Hospital of Chicago from March 2015 to March 2023, were retrospectively reviewed. Demographic, clinical, and radiological data were systematically collated., Results: Ninety-six patients were treated for sinusitis-related and/or otitis media-related intracranial infections during the study period, 15 (15.6%) of whom were diagnosed with a CVST. Of the 60 patients who presented prior to the COVID-19 pandemic, 6 (10.0%) were diagnosed with a septic CVST, whereas of the 36 who presented during the COVID-19 pandemic, 9 (25.0%) had a septic CVST (p = 0.050). The superior sagittal sinus was involved in 12 (80.0%) patients and the transverse and/or sigmoid sinuses in 4 (26.7%). Only 1 (6.7%) patient had a fully occlusive thrombus. Of the 15 patients with a septic CVST, 11 (73.3%) were initiated on anticoagulation at a median interval of 4 (IQR 3-5) days from the most recent neurosurgical procedure. Five (45.5%) patients who underwent anticoagulation demonstrated complete recanalization on follow-up imaging, and 4 (36.4%) had partial recanalization. Three (75.0%) patients who did not undergo anticoagulation demonstrated complete recanalization, and 1 (25.0%) had partial recanalization. None of the patients treated with anticoagulation experienced hemorrhagic complications., Conclusions: Septic CVST is frequently identified among pediatric patients undergoing neurosurgical intervention for sinogenic and/or otogenic intracranial infections and may have become more prevalent during the COVID-19 pandemic. Anticoagulation can be used safely in the acute postoperative period if administered cautiously, in a monitored setting, and with interval cross-sectional imaging. However, some patients exhibit excellent outcomes without anticoagulation, and further studies are needed to identify those who may benefit the most from anticoagulation.

Objective: After becoming widespread during the COVID-19 pandemic, telemedicine continues to play an important role in outpatient neurosurgical care. Nevertheless, the factors that influence individual decisions to choose telemedicine versus in-person appointments remain understudied. Here, we performed a prospective survey of pediatric neurosurgical patients and caregivers presenting for telemedicine or in-person outpatient visits, to identify factors associated with appointment choice., Methods: All patients and caregivers with an outpatient pediatric neurosurgical encounter at Connecticut Children's between January 31st and May 20th, 2022, were invited to participate in this survey. Data related to demographics, socioeconomics, technological access, COVID-19 vaccination status, and appointment preferences were collected., Results: During the study period, there were 858 unique pediatric neurosurgical outpatient encounters (86.1% in-person and 13.9% telemedicine). A total of 212 (24.7%) respondents completed the survey. Those with a telemedicine appointment were more likely to be white (P = 0.005), not Hispanic or Latino (P = 0.020), have private insurance (P = 0.003), be established patients (P < 0.001), have a household income >$80,000 (P = 0.005), and have caregivers who completed a 4-year college degree (P < 0.001). Those who were seen in-person cited the patient's condition, quality of care, and communication as important factors, whereas those who were seen via telemedicine cited time, travel, and convenience., Conclusions: While convenience influences some to choose telemedicine, concerns regarding the quality of care persist among those who prefer in-person encounters. Recognizing these factors will minimize barriers to care, better define the appropriate populations/contexts for each encounter type, and improve the integration of telemedicine within an outpatient neurosurgical setting., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: Ridging along the metopic suture line can be a common cause of concern for parents and has been theorized to represent a mild form of trigonocephaly, a cranial deformity associated with risks of negative cosmetic outcomes, if not surgically corrected. Yet the literature contains sparse reports of long-term cosmetic results or expectations for infants with isolated metopic ridging compared with those with severe trigonocephaly, or even what objective metrics discriminate isolated metopic ridging from severe trigonocephaly. Therefore, the authors' goals for this study were to 1) quantify the degree of frontal deformity among patients with metopic ridge, metopic craniosynostosis, and normocephalic head shapes; and 2) document the natural history of frontal deformities in isolated metopic ridge patients in the 1st year of life., Methods: This was a retrospective cohort study of patients with normocephalic head shapes, metopic ridges, and metopic craniosynostoses who presented at < 1 year of age to the Connecticut Children's neurosurgery clinic from January 2019 to December 2021. Data were collected regarding demographics and photograph-based craniometrics., Results: A total of 212 normocephalic, 34 metopic ridge, and 29 metopic craniosynostosis patients were included. Both the normocephalic and metopic ridge groups had a significantly higher anterior arc angle (AAA) value compared with the metopic craniosynostosis group (p < 0.0001). The AAA did not differ significantly among normocephalic patients and those with ridging. Over the course of 1 year of follow-up, patients with metopic ridging demonstrated a slight decrease in AAA values, but overall remained within the same range as normocephalic patients., Conclusions: Photograph-based craniometrics suggest that metopic ridge patients with frontal bone angulations > 2.2 radians have a mild degree of frontal constriction that does not significantly worsen over the 1st year of life.

Odontoid fractures are common, often presenting in the elderly after a fall and infrequently associated with traumatic spinal cord injury (tSCI). The goal of this study was to analyze predictors of mortality and neurological outcome when odontoid fractures were associated with signal change on magnetic resonance imaging (MRI) at admission. Over an 18-year period (2001-2019), 33 patients with odontoid fractures and documented tSCI on MRI were identified. Mean age was 65.3 years (standard deviation [SD] = 17.2), and 21 patients were male. The mechanism of injury was falls in 25 patients, motor vehicle accidents in 5, and other causes in 3. Mean Injury Severity Score (ISS) was 40.5 (SD = 30.2), Glasgow Coma Scale (GCS) score was 13 (SD = 3.4), and American Spinal Injury Association (ASIA) motor score (AMS) was 51.6 (SD = 42.7). ASIA Impairment Scale (AIS) grade was A, B, C, and D in 9, 2, 3, and 19 patients, respectively. Mean intramedullary lesion length was 32.3 mm (SD = 18.6). The odontoid peg was displaced ventral or dorsal in 15 patients. Twenty patients had surgical intervention: anterior odontoid screw fixation in 7 and posterior spinal fusion in 13. Eleven (33.3%) patients died in this series: withdrawal of medical care in 5; anoxic brain injury in 4; and failure of critical care management in 2. Univariate logistic regression indicated that GCS score ( p  < 0.014), AMS ( p  < 0.002), AIS grade ( p  < 0.002), and ISS ( p  < 0.009) were risk factors for mortality. Multi-variate regression analysis indicated that only AMS ( p  < 0.002) had a significant relationship with mortality when odontoid fracture was associated with tSCI (odds ratio, 0.963; 95% confidence interval, 0.941-0.986)., Competing Interests: No competing financial interests exist., (© Bizhan Aarabi et al., 2023; Published by Mary Ann Liebert, Inc.)

Objective: Sagittal craniosynostosis is the most common form of craniosynostosis and typically results in scaphocephaly, which is characterized by biparietal narrowing, compensatory frontal bossing, and an occipital prominence. The cephalic index (CI) is a simple metric for quantifying the degree of cranial narrowing and is often used to diagnose sagittal craniosynostosis. However, patients with variant forms of sagittal craniosynostosis may present with a "normal" CI, depending on the part of the suture that is closed. As machine learning (ML) algorithms are developed to assist in the diagnosis of cranial deformities, metrics that reflect the other phenotypic features of sagittal craniosynostosis are needed. In this study the authors sought to describe the posterior arc angle (PAA), a measurement of biparietal narrowing that is obtained with 2D photographs, and elucidate the role of PAA as an adjuvant to the CI in characterizing scaphocephaly and the potential relevance of PAA in new ML model development., Methods: The authors retrospectively reviewed 1013 craniofacial patients treated during the period from 2006 to 2021. Orthogonal top-down photographs were used to calculate the CI and PAA. Distribution densities, receiver operating characteristic (ROC) curves, and chi-square analyses were used to describe the relative predictive utility of each method for sagittal craniosynostosis., Results: In total, 1001 patients underwent paired CI and PAA measurements and a clinical head shape diagnosis (sagittal craniosynostosis, n = 122; other cranial deformity, n = 565; normocephalic, n = 314). The area under the ROC curve (AUC) for the CI was 98.5% (95% confidence interval 97.8%-99.2%, p < 0.001), with an optimum specificity of 92.6% and sensitivity of 93.4%. The PAA had an AUC of 97.4% (95% confidence interval 96.0%-98.8%, p < 0.001) with an optimum specificity of 94.9% and sensitivity of 90.2%. In 6 of 122 (4.9%) cases of sagittal craniosynostosis, the PAA was abnormal while the CI was normal. This means that adding a PAA cutoff branch to a partition model increases the detection of sagittal craniosynostosis., Conclusions: Both CI and PAA are excellent discriminators for sagittal craniosynostosis. Using an accuracy-optimized partition model, the addition of the PAA to the CI increased model sensitivity compared to using the CI alone. Using a model that incorporates both CI and PAA could assist in the early identification and treatment of sagittal craniosynostosis via automated and semiautomated algorithms that utilize tree-based ML models.

Objective: Focal intracranial infections (epidural abscesses, subdural empyemas, and intraparenchymal abscesses) are uncommon complications of sinusitis and otitis media but can be associated with significant morbidity. Treatment typically requires neurosurgical and otolaryngological interventions in combination with antibiotic treatment. Historically, children have presented to the authors' pediatric referral center with sinusitis- or otitis media-related intracranial infections in low numbers. However, since the onset of the COVID-19 pandemic, the incidence of intracranial pyogenic complications has increased at this center. The objective of this study was to compare the epidemiology, severity, microbial causes, and management of pediatric sinusitis- and otitis-related intracranial infections in the periods before and during the COVID-19 pandemic., Methods: All patients 21 years of age or younger who presented with an intracranial infection in the setting of sinusitis or otitis media and who underwent neurosurgical treatment at Connecticut Children's from January 2012 to December 2022 were retrospectively reviewed. Demographic, clinical, laboratory, and radiological data were systematically collated, and variables before and during COVID-19 were compared statistically., Results: Overall, 18 patients were treated for sinusitis-related (n = 16) or otitis media-related (n = 2) intracranial infections during the study period. Ten patients (56%) presented from January 2012 to February 2020, none from March 2020 to June 2021, and 8 (44%) from July 2021 to December 2022. There were no significant demographic differences between the pre-COVID-19 and COVID-19 cohorts. The 10 patients in the pre-COVID-19 cohort underwent a total of 15 neurosurgical and 10 otolaryngological procedures, while the 8 patients in the COVID-19 cohort underwent a total of 12 neurosurgical and 10 otolaryngological procedures. Surgically obtained wound cultures yielded a variety of organisms; Streptococcus constellatus/S. anginosus/S. intermedius were more prevalent in the COVID-19 cohort (87.5% vs 0%, p < 0.001) as was Parvimonas micra (62.5% vs 0%, p = 0.007)., Conclusions: At an institutional level, there has been an approximately threefold increase in cases of sinusitis- and otitis media-related intracranial infections during the COVID-19 pandemic. Multicenter studies are needed to confirm this observation and to investigate whether the mechanisms of infection are related directly to SARS-CoV-2, changes in the respiratory flora, or delayed care. The next steps will include expansion of this study to other pediatric centers throughout the United States and Canada.

Objective: Quantitative measurements of trigonocephaly can be used to characterize and track this phenotype, which is associated with metopic craniosynostosis. Traditionally, trigonocephaly metrics were extracted from CT scans; however, this method exposes patients to ionizing radiation. Three-dimensional optical scans are another option but are not routinely available in most outpatient settings. Recently, the authors developed semiautomated artificial intelligence algorithms that extract craniometric data from orthogonal 2D photographs. Although 2D photographs are safe, inexpensive, and straightforward to obtain, the accuracy of photograph-based craniometrics in comparison to CT and 3D optical scan correlates has not been established. In this study the authors compared the classification power of 2D photograph-based metrics of trigonocephaly with four CT-based metrics and one 3D optical scan-based metric in a heterogeneous series of patients who presented to an outpatient craniofacial clinic., Methods: In this study the authors performed retrospective craniometric analyses of patient 2D photographs, 3D optical scans, and CT scans. Imaging-derived craniometrics include the 2D photograph-based anterior arc angle (AAA2D-photo), anterior-posterior ratio (APR2D-photo), and anterior-middle ratio (AMR2D-photo); the CT-based anterior arc angle (AAACT), metopic index (MICT), endocranial-bifrontal angle (eBFACT), and interfrontal angle (IFACT); and the 3D optical scan-based anterior arc angle (AAA3D-optical). Receiver operating characteristics (ROCs) were used to identify craniometrics strongly descriptive of trigonocephaly. Interrater comparisons were made between paired trigonocephaly measurements obtained from photographs and either CT scans or 3D optical scans., Results: There were 13 photograph-based and CT-based pairs and 22 paired measurements from 2D photographs and 3D optical scans. AAA displayed the strongest classification capacity across all three imaging modalities. Significant agreement was observed between AAACT and AAA2D-photo (intraclass correlation coefficient [ICC] = 0.68 [95% CI 0.24-0.89], p = 0.0035), and AAA3D-optical and AAA2D-photo (ICC = 0.70 [95% CI 0.41-0.87], p < 0.0001). There was no significant correlation between APR2D-photo or AMR2D-photo and conventional CT-based metrics describing longitudinal width ratios (MICT)., Conclusions: Photograph-based craniometrics are powerful tools that can be used to quantify the severity of trigonocephaly and exhibit high concordance with standard measurements derived from CT scans and 3D optical scans. The authors developed and freely share a research-use application to calculate trigonocephaly metrics from 2D photographs. Given the availability of digital photography, lack of ionizing radiation, and low cost of photograph-based craniometric derivation, this technique may be useful to supplement routine ambulatory care and objectively track outcomes following treatment.

Objective: Virtual mentorship and research programs are becoming increasingly popular to facilitate education and career development for students and residents. We review virtual research initiatives for early trainees in neurosurgery and describe our effort to expand access to resources and shared objective mentorship (SOM) via the novel Neurosurgery Education and Research Virtual Group (NERVE)., Methods: A systematic review of neurosurgical programming delivered via a virtual platform was conducted using PubMed, Embase, and Scopus databases. Identified articles were screened. Those meeting prespecified inclusion criteria were reviewed in full and examined for relevant data. Data analysis was performed using Microsoft Excel, and means and standard deviations were calculated. Descriptive analysis of NERVE characteristics was also performed., Results: Of the 2438 identified articles, 10 were included. The most common (70%) implementation style was a webinar-based lecture series. The least common (10%) was a longitudinal curricular interest group. Of the total NERVE cohort, 90% were first generation medical students and 82% attended institutions without home programs. Survey results indicated 73.8% had contributed to at least 2 research projects throughout the year., Conclusions: There is a scarcity of virtual neurosurgical resources which facilitate SOM opportunities for trainees. In our systematic review, NERVE is the only multi-institutional virtual initiative aimed at increasing access to neurosurgical education and research opportunities for the purpose of SOM among early trainees from disadvantaged backgrounds. This highlights the group's niche and potential impact on increasing diversity in neurosurgery, improving trainees' career development, and facilitating future resident research productivity., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Revascularization surgery decreases the long-term risk of stroke in children with moyamoya but carries an increased risk of perioperative ischemic events. Evidence-based approaches to safe perioperative management of children with moyamoya are limited. We aimed to understand practice variability in perioperative moyamoya care., Methods: Neurologists, neurosurgeons, and intensivists practicing in North America with expertise in perioperative pediatric moyamoya care participated in a 138-item anonymous survey focused on interdisciplinary perioperative care surrounding indirect revascularization surgery., Results: Many perioperative care practices vary substantially between participants. Timing of resumption of antiplatelet therapy postoperatively, choice of sedative agents and vasopressors, goal blood pressures, rate and duration of intravenous fluid administration, and red blood cell transfusion thresholds are among the most variable practices., Conclusions: This practice variability survey highlights several important knowledge gaps and areas of equipoise that should be targets for future investigation and consensus-building efforts., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Middle meningeal artery (MMA) embolization is becoming increasingly studied as a safe, effective treatment for chronic subdural hematoma (cSDH) in adults. Among pediatric patients, however, MMA embolization for cSDH has been rarely described, and the potential benefit of this approach for pediatric patients remains unknown., Objective: To systematically review the literature and identify cases of pediatric MMA embolization for cSDH. We also report our experience with pediatric MMA embolization., Methods: A systematic review of the literature was performed to identify cases of pediatric MMA embolization for cSDH. Inclusion criteria included English language availability and pediatric age defined as less than 18 years. A pediatric patient treated with MMA embolization was also identified at our institution., Results: Five cases of pediatric MMA embolization for cSDH were identified in the literature. Two were associated with arachnoid cysts, 2 with antiplatelet/anticoagulation therapy, and 1 with abusive head trauma. There were no adverse events, and all patients demonstrated clinical and radiological improvement on follow-up. At our institution, a previously healthy 8-year-old male was found to have a right-sided acute-on-chronic SDH during a headache evaluation. A diagnostic angiogram was performed to rule out a dural arteriovenous fistula, and right-sided MMA embolization was performed concurrently. Rapid clinical and radiological improvement was observed, with complete resolution by 6 months., Conclusion: MMA embolization may represent a treatment option for pediatric patients with cSDH., (Copyright © Congress of Neurological Surgeons 2022. All rights reserved.)

Objective: Pediatric low-grade gliomas (pLGGs) frequently exhibit dysregulation of the mitogen-activated protein kinase (MAPK) pathway. Targeted therapies, including mutant BRAF inhibitors (dabrafenib) and MEK inhibitors (trametinib), have shown promise in patients in whom conventional chemotherapy has failed. However, few studies have investigated the use of targeted therapy as a first-line treatment for pLGG. Here, the authors reviewed their institutional experience with using a personalized medicine approach to patients with newly diagnosed pLGGs., Methods: All pediatric patients at the authors' institution who had been treated with dabrafenib or trametinib for pLGG without first receiving conventional chemotherapy or radiation were retrospectively reviewed. Demographic, clinical, and radiological data were collected., Results: Eight patients underwent targeted therapy as a first-line treatment for pLGG. Five patients had a BRAF alteration (1 with a BRAFV600E mutation, 4 with a KIAA1549:BRAF fusion), and 3 patients had an NF1 mutation. One of the 8 patients was initially treated with dabrafenib, and trametinib was added later. Seven patients were initially treated with trametinib; of these, 2 later transitioned to dual therapy, whereas 5 continued with trametinib monotherapy. Six patients (75%) demonstrated a partial response to therapy during their treatment course, whereas stable disease was identified in the remaining 2 patients (25%). One patient experienced mild disease progression after completing a course of trametinib monotherapy, but ultimately stabilized after a period of close observation. Another patient experienced tumor progression while on dabrafenib, but subsequently responded to dual therapy with dabrafenib and trametinib. The most common adverse reactions to targeted therapy were cutaneous toxicity (100%) and diarrhea (50%)., Conclusions: Targeted therapies have the potential to become a standard treatment option for pLGG due to their favorable toxicity profile and oral route of administration. This case series provides preliminary evidence that targeted therapies can induce an early disease response as a first-line adjuvant treatment; however, large-scale studies are required to assess long-term durability and safety.

Virtual reality (VR) and augmented reality (AR) are rapidly growing technologies. Both have been applied within neurosurgery for presurgical planning and intraoperative navigation, but VR and AR technology is particularly promising for the education of neurosurgical trainees. With the increasing demand for high impact yet efficient educational strategies, VR- and AR-based simulators allow neurosurgical residents to practice technical skills in a low-risk setting. Initial studies have confirmed that such simulators increase trainees' confidence, improve their understanding of operative anatomy, and enhance surgical techniques. Knowledge of the history and conceptual underpinnings of these technologies is useful to understand their current and future applications towards neurosurgical training. The technological precursors for VR and AR were introduced as early as the 1800s, and draw from the fields of entertainment, flight simulation, and education. However, computer software and processing speeds are needed to develop widespread VR- and AR-based surgical simulators, which have only been developed within the last 15 years. During that time, several devices had become rapidly adopted by neurosurgeons, and some programs had begun to incorporate them into the residency curriculum. With ever-improving technology, VR and AR are promising additions to a multi-modal training program, enabling neurosurgical residents to maximize their efforts in preparation for the operating room. In this review, we outline the historical development of the VR and AR systems that are used in neurosurgical training and discuss representative examples of the current technology., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Solid variant aneurysmal bone cysts (SVABCs) are a rare but well-described subtype of ABCs. While classic ABCs are readily identified radiographically, SVABCs lack these characteristic radiographic features and thus have a wide differential diagnosis on presentation (including Ewing sarcoma, Langerhans cell histiocytosis, osteosarcoma, metastasis, and giant cell tumor). Genomic/molecular analyses are often necessary for the diagnosis of SVABCs, with USP6 rearrangements being a characteristic finding. We present two cases in which genomic analysis was critical in the diagnosis of SVABCs and revealed unique gene fusions that may provide insight into SVABC pathogenesis., Case Descriptions: Two 13-year old male children presented to our institution with new mass lesions involving the craniofacial skeleton. Magnetic resonance imaging (MRI) in both cases revealed predominantly solid, avidly enhancing masses, one of the squamous portion of the temporal bone, and the other arising from the sphenopalatine foramen with extension into the ipsilateral maxillary and ethmoid sinuses. Histopathology displayed predominantly solid morphology, and next generation sequencing (NGS) revealed a FAT1-USP6 gene fusion in the temporal lesion, and a MIR22HG-USP6 gene fusion in the maxillofacial lesion, the latter of which was not identified on fluorescence in situ hybridization (FISH). These findings were most consistent with a diagnosis of SVABC in each case., Conclusions: These two cases highlight novel gene fusions in atypically located SVABCs and emphasize the ability of NGS to more accurately and consistently identify USP6 gene fusions, particularly in SVABCs that may otherwise be indistinguishable from alternative pathologies., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Pediatric glioblastoma is relatively rare compared with its adult counterpart but is associated with a similarly grim prognosis. Available data indicate that pediatric glioblastomas are molecularly distinct from adult tumors, and relatively little is known about the pediatric glioblastoma tumor microenvironment (TME). Cancer immunotherapy has emerged as a new pillar of cancer treatment and is revolutionizing the care of patients with many advanced solid tumors, including melanoma, non-small cell lung cancer, head and neck cancer, and renal cell carcinoma. Unfortunately, attempts to treat adult glioblastoma with current immunotherapies have had limited success to date. Nevertheless, the immune milieu in pediatric glioblastoma is distinct from that found in adult tumors, and evidence suggests that pediatric tumors are less immunosuppressive. As a result, immunotherapies should be specifically evaluated in the pediatric context. The purpose of this review is to explore known and emerging mechanisms of immune evasion in pediatric glioblastoma and highlight potential opportunities for implementing immunotherapy in the treatment of these devastating pediatric brain tumors., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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: Telemedicine can be an effective tool for the evaluation of the pediatric patient with a cranial deformity, but it increases the reliance of neurosurgical providers on data provided by patients and families. Family-acquired photographs, in particular, can be used to augment the evaluation of pediatric head shape abnormalities via telemedicine, but photographs of sufficient quality are necessary. Here, the authors systematically reviewed the quality and utility of family-acquired photographs for patients referred to their pediatric neurosurgery clinic for telemedicine-based head shape evaluations., Methods: All telemedicine encounters that were completed for head shape abnormalities at the authors' institution between May 2020 and December 2021 were retrospectively reviewed. Instructions were sent to families prior to each visit with examples of ideal photographs. Three orthogonal views of the patient's head-frontal, lateral, and vertex-were requested. Data were collected regarding demographics, diagnosis, follow-up, and photograph quality. Quality variables included orthogonality of each requested view, appropriate distance, appropriate lighting, presence of distracting elements, and whether hair obscured the head shape., Results: Overall, 565 patients had 892 visits during the study period. A total of 1846 photograph requests were made, and 3335 photographs were received for 829 visits. Of 2676 requested orthogonal views, 1875 (70%) were received. Of these, 1826 (97%) had adequate lighting, 1801 (96%) had appropriate distance, and 1826 (97%) had no distracting features. Hair did not obscure the head shape on the vertex view in 557 visits with orthogonal vertex views (82%). In-person follow-up was requested for further medical evaluation in 40 visits (5%)., Conclusions: The family-acquired photographs in this series demonstrated high rates of adequate lighting and distance, without distracting features. Lack of orthogonality and obscuration of the head shape by hair, however, were more common issues. Family education prior to the visit may improve the quality of family-acquired photographs but requires an investment of time by medical staff. Efforts to further improve photographic quality will facilitate efforts to perform craniometric evaluations through telemedicine visits.

Background: The authors have developed pretrained machine learning (ML) models to evaluate neonatal head shape deformities using top-down and facial orthogonal photographs of the patient's head. In previous preliminary analysis, this approach was tested with images from an open-source data bank., Objective: To determine the accuracy of pretrained ML models in identifying craniosynostosis among patients seen in our outpatient neurosurgery clinic., Methods: We retrospectively reviewed top-down and facial orthogonal images of each patient's head and provider clinical diagnosis from the same encounters. Head shape classifications generated from 3 pretrained ML models (random forest, classification and regression tree, and linear discriminant analysis) were applied to each patient's photograph data set after craniometric extraction using a predefined image processing algorithm. Diagnoses were codified into a binary scheme of craniosynostosis vs noncraniosynostosis. Sensitivity, specificity, and Matthew correlation coefficient were calculated for software vs provider classifications., Results: A total of 174 patients seen for abnormal head shape between May 2020 and February 2021 were included in the analysis. One hundred seven patients (61%) were seen in-person and 67 (39%) through telemedicine. Twenty-three patients (13%) were diagnosed with craniosynostosis. The best-performing model identified craniosynostosis with an accuracy of 94.8% (95% CI 90.4-97.6), sensitivity of 87.0% (95% CI 66.4-97.2), specificity of 96.0% (95% CI 91.6-98.5), and Matthew correlation coefficient of 0.788 (95% CI 0.725-0.839)., Conclusion: Machine learning-driven image analysis represents a promising strategy for the identification of craniosynostosis in a real-world practice setting. This approach has potential to reduce the need for imaging and facilitate referral by primary care providers., (Copyright © Congress of Neurological Surgeons 2022. All rights reserved.)

Background/Purpose: Telehealth has evolved over the past several decades to address varying medical needs. Most recently, it has been widely utilized as a result of the COVID-19 pandemic. Most previous studies have not performed in-depth analyses of patient satisfaction with telehealth technologies. This project investigated patient satisfaction with the abrupt implementation of telemedicine within pediatric surgical subspecialties during the COVID-19 pandemic. Materials and Methods: We conducted a single-center review to determine patients' satisfaction with telemedicine during the early stages of the pandemic. Surveys were distributed to patients who completed a telemedicine video visit within a pediatric surgical subspecialty at Connecticut Children's Medical Center between March 16 and May 16, 2020. Results: A total of 1,608 patients were contacted, with 457 surveys completed (28.4%). Of all respondents, 95.6% were "very satisfied" or "satisfied" with their overall telemedicine appointment. Seventy-three percent reported no difficulty with completing their visit. Eighty-seven percent would consider another telemedicine visit again in the future. No significant difference was found between the payor mix for telemedicine and in-person appointments. Conclusions: The implementation of telemedicine at Connecticut Children's Medical Center during the COVID-19 pandemic was successful despite numerous barriers to use. Families not only showed high rates of satisfaction with the overall visit and provider interaction, but also an overwhelming openness to future use of telehealth.

Objective: Central diabetes insipidus (DI) is frequently identified preoperatively and/or postoperatively in patients with sellar or parasellar lesions. Early diagnosis and effective perioperative management of central DI is critical to minimize disruptions in fluid homeostasis. In particular, although venous thromboembolism (VTE) is generally less common in pediatric patients than their adult counterparts, isolated reports suggest that VTE occurs at a higher frequency in pediatric patients with central DI., Methods: Using the PubMed, Scopus, and Springer Link databases, the authors performed a systematic review of the literature with regard to the incidence of VTE in pediatric patients with central DI. Inclusion criteria were availability of the full text in English, diagnosis of central DI and VTE in the same patient, and pediatric age defined as ≤ 21 years. Data were reported as median and interquartile range for continuous variables and as frequencies and percentages for categorical variables. Risk of bias assessments of the individual studies were performed using the Joanna Briggs Institute Critical Appraisal Checklists for case series and case reports., Results: Of 2094 search results, 12 articles met the inclusion criteria and described a total of 17 cases of VTE in pediatric patients with central DI. Two additional patients from the authors' institution were added to this cohort. The underlying pathologies included craniopharyngioma (n = 6), suprasellar germinoma (n = 4), epileptic encephalopathy (n = 2), pilocytic astrocytoma (n = 2), prolactinoma (n = 2), Cushing disease (n = 1), failure to thrive (n = 1), and congenital hypothalamic syndrome (n = 1). Thrombotic complications included deep vein thrombosis (n = 10 [53%]), cerebral venous sinus thrombosis (n = 6 [32%]), pulmonary embolism (n = 4 [21%]), inferior vena cava thrombosis (n = 2 [11%]), and disseminated intravascular coagulation (n = 1 [5%]). There was a 26% mortality rate., Conclusions: VTE is a rare but potentially devastating postoperative complication that appears to have a higher incidence among patients with central DI. Although this review was limited by heterogeneous information across limited reports, pediatric neurosurgical patients with DI may benefit from more aggressive VTE surveillance and prophylaxis.

Craniosynostosis involves the premature fusion of 1 or more cranial sutures and commonly presents as an isolated, nonsyndromic diagnosis. A subset of patients have syndromic craniosynostosis. Several unique considerations must be taken into account when managing patients with syndromic craniosynostosis. A multidisciplinary craniofacial team with a central coordinator is particularly useful for coordinating care among various specialists, and close monitoring is mandatory owing to the increased risk of intracranial hypertension. Surgical management varies among centers, but core options include fronto-orbital advancement with cranial vault remodeling, posterior vault expansion, endoscopic-assisted suturectomy with postoperative orthotic therapy, and midface advancement., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Introduction: The optimal protocol for diagnostic workup of craniosynostosis and the role of specific imaging modalities remain controversial. Skull X-rays and 3-dimensional head CTs are options when physical exam is equivocal but involve ionizing radiation. Ultrasound has emerged as an alternative modality for visualization of cranial sutures, but its use is not widespread., Methods: The authors performed a systematic review of the literature on the use of ultrasound for the diagnosis of craniosynostosis., Results: A total of 12 studies involving 1062 patients were included. Overall, 300 patients (28.2%) were diagnosed with craniosynostosis. A total of 369 (34.7%) patients had their diagnosis (craniosynostosis vs. patent sutures) confirmed with another imaging modality in addition to ultrasound. Among studies, the specificity of ultrasound ranged from 86 to 100%, and the sensitivity from 71 to 100%., Conclusions: Ultrasonography of cranial sutures is a feasible and accurate tool for the diagnosis of single-suture craniosynostosis when physical exam findings are insufficient. Although technical aspects of ultrasonography and its interpretation have an associated learning curve, ultrasound can achieve high sensitivity and specificity among patients with suspected craniosynostosis., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Widespread use of modern neuroimaging has led to a surge in diagnosing pediatric brain incidentalomas. Thalamic lesions have unique characteristics such as deep location, surgical complexity, and proximity to eloquent neuronal structures. Currently, the natural course of incidental thalamic lesions is unknown. Therefore, the authors present their experience in treating such lesions., Methods: A retrospective, international multicenter study was carried out in 8 tertiary pediatric centers from 5 countries. Patients were included if they had an incidental thalamic lesion suspected of being a tumor and were diagnosed before the age of 20 years. Treatment strategy, imaging characteristics, pathology, and the outcome of operated and unoperated cases were analyzed., Results: Overall, 58 children (23 females and 35 males) with a mean age of 10.8 ± 4.0 years were included. The two most common indications for imaging were nonspecific reasons (n = 19; e.g., research and developmental delay) and headache unrelated to small thalamic lesions (n = 14). Eleven patients (19%) underwent early surgery and 47 were followed, of whom 10 underwent surgery due to radiological changes at a mean of 11.4 ± 9.5 months after diagnosis. Of the 21 patients who underwent surgery, 9 patients underwent resection and 12 underwent biopsy. The two most frequent pathologies were pilocytic astrocytoma and WHO grade II astrocytoma (n = 6 and n = 5, respectively). Three lesions were high-grade gliomas., Conclusions: The results of this study indicate that pediatric incidental thalamic lesions include both low- and high-grade tumors. Close and long-term radiological follow-up is warranted in patients who do not undergo immediate surgery, as tumor progression may occur.

Objective: Craniosynostosis is a congenital disorder resulting from the premature fusion of cranial sutures in the infant skull. This condition results in significant cosmetic deformity and can impede neurodevelopment, if left untreated. Currently, rates of craniometric change following minimally invasive surgery have only been examined for sagittal craniosynostosis. A better understanding of postoperative skull adaptations in other craniosynostosis subtypes is needed to objectively categorize surgical outcomes and guide length of cranial orthosis therapy., Methods: Eleven patients with sagittal and 8 with metopic craniosynostosis treated using endoscopic strip craniectomy and postoperative helmet orthoses were retrospectively reviewed. Using semiautomated image analysis of top-down orthogonal 2D photographs, the following craniometrics were recorded before surgery and at postoperative visits: cephalic index (CI), cranial vault asymmetry index (CVAI), anterior arc angle (AAA), posterior arc angle (PAA), anterior-middle width ratio (AMWR), anterior-posterior width ratio (APWR), left-right height ratio (LRHR), sagittal Hu moment (Sag-Hu), and brachycephaly Hu moment (Brachy-Hu). These craniometrics were then normalized to photograph-based measurements of normocephalic patients and the rates of change between metopic and sagittal craniosynostoses were compared., Results: Patients with sagittal craniosynostosis exhibited significantly lower CI, lower PAA, higher AMWR, higher APWR, lower Sag-Hu, and higher Brachy-Hu preoperatively compared to patients with normocephalic craniosynostosis. Patients with metopic craniosynostosis exhibited lower AAA and AMWR preoperatively compared to normocephalic subjects. Sagittal and metopic patients had a rapid initial change in normalized CI or AAA, respectively. Craniometric rates of change that significantly differed between metopic and sagittal patients were found in AAA (p < 0.001), AMWR (p < 0.001), and APWR (p < 0.0001). Metopic patients had a prolonged AAA change with a significantly different rate of change up to 6 months postoperatively (median at 3 months = 0.027 normalized units/day, median at 6 months = 0.017 normalized units/day, and median at > 6 months = 0.007 normalized units/day), while sagittal CI rate of change at these time points was not significantly different., Conclusions: Patients with metopic craniosynostosis have a prolonged rate of change compared to patients with sagittal craniosynostosis and may benefit from longer helmet use and extended postoperative follow-up. Categorizing craniometric changes for other craniosynostosis subtypes will be important for evaluating current treatment guidelines.

Objective: In the wake of the COVID-19 pandemic, telemedicine has become rapidly adopted by the neurosurgical community; however, few studies have examined predictors of telemedicine utilization. Here, we analyze patient variables associated with the acceptance of a telemedicine encounter by a pediatric neurosurgical population during the early phases of the COVID-19 pandemic., Methods: All patients seen in a single institution's outpatient pediatric neurosurgery clinic between April 1, 2020 and July 31, 2020 were retrospectively reviewed. Demographic variables were collected for each patient's first completed encounter. Patients participating in telemedicine were compared with those seen in person. Univariate analysis was performed using the Wilcoxon rank sum test for continuous variables and Fischer exact test for categorical variables. A logistic regression multivariable analysis was then performed., Results: We included 682 patients (374 telemedicine and 308 in person). Univariate analysis demonstrated that telemedicine visits were more likely to occur at earlier study dates (P < 0.001) and that patients participating in telemedicine visits were more likely to be established rather than new patients (P < 0.001), White or Caucasian (P < 0.001), not Hispanic or Latino (P < 0.001), English-speaking (P < 0.001), non-Medicare/Medicaid recipients (P < 0.001), have lower no-show rates (P = 0.006), and live farther from the hospital (P = 0.005). Multivariable analysis demonstrated older age (P = 0.031), earlier appointment date (P < 0.01), established patient status (P < 0.001), English-speaking (P < 0.02), and non-Medicare/Medicaid insurance (P < 0.05) were significant predictors of telemedicine utilization., Conclusions: Significant demographic differences exist among pediatric patients who participated in telemedicine versus those who requested an in-person visit at our institution. Addressing barriers to access will be crucial for promoting health equity in continued utilization of telemedicine., (Copyright © 2021 Elsevier Inc. All rights reserved.)

The cranial fontanelles and sutures have several benign variations, including most cases of "early" or "late" closure of the anterior fontanelle, bathrocephaly, overriding sutures, and benign metopic ridging. However, recognizing true craniosynostosis and referring the patient to a craniofacial specialist in a timely fashion are imperative, as minimally invasive options can be offered to most patients younger than 6 months of age. Gaining comfort with the physical examination of an infant with an abnormal head shape is best achieved through experience and pattern recognition and will frequently facilitate an accurate diagnosis without the need for ionizing radiation., (Copyright © 2021 Elsevier Inc. All rights reserved.)

The authors describe the unusual case of a 6-year-old boy presenting with decorticate posturing, diminished hearing, and an inability to open his eyes, despite being verbally responsive. He underwent a posterior interhemispheric transcallosal intervenous approach for resection of a pineal region mature teratoma, which recurred 2 years postoperatively. This video demonstrates his initial surgery and reresection, illustrating the value of this approach for more complex lesions that involve the internal cerebral veins (ICVs). At the time of recurrence, microsurgical dissection of the scarred interhemispheric fissure was required to facilitate removal of the multifocal recurrent teratoma, resulting in gross-total resection. The video can be found here: https://stream.cadmore.media/r10.3171/2021.4.FOCVID2134., Competing Interests: Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this publication., (© 2021, The Authors.)

Background: Juvenile myelomonocytic leukemia (JMML) is a rare childhood hematopoietic disorder typically presenting with hepatosplenomegaly, lymphadenopathy, pallor, fever, and cutaneous findings. The authors report the first case, to our knowledge, of JMML presenting in a pediatric patient with a subdural hematoma., Case Description: A 7-month old male with recurrent respiratory infections and a low-grade fever presented with a full fontanelle and an increasing head circumference and was found to have chronic bilateral subdural collections. Abusive head trauma, infectious, and coagulopathy workups were unremarkable, and the patient underwent bilateral burr holes for evacuation of the subdural collections. The postoperative course was complicated by the development of thrombocytopenia, anemia, and an acute subdural hemorrhage which required evacuation. Cytologic analysis of the subdural fluid demonstrated atypical cells, which prompted flow cytometric analysis, a bone marrow biopsy, and ultimately a diagnosis of JMML. Following chemotherapy, the patient's counts improved, and he subsequently underwent a hematopoietic stem cell transplant., Conclusion: Subdural collections may rarely represent the first presenting sign of hematologic malignancies. In patients with a history of recurrent infections and a negative workup for abusive head trauma, clinicians should include neoplastic etiologies in the differential for chronic subdural collections and have a low threshold for fluid analysis.

Objective: Late failure is a well-documented complication of cerebrospinal fluid shunt placement and, less commonly, endoscopic third ventriculostomy (ETV). However, standards regarding the frequency of clinical and radiological follow-up in these patients have not been defined. Here, the authors report on their survey of surgeons at sites for the Hydrocephalus Clinical Research Network (HCRN) or its implementation/quality improvement arm (HCRNq) to provide a cross-sectional overview of practice patterns., Methods: A 24-question survey was developed using the Research Electronic Data Capture (REDCap) platform and was distributed to the 138 pediatric neurosurgeons across 39 centers who participate in the HCRN or HCRNq. Survey questions were organized into three sections: 1) Demographics (5 questions), 2) Shunt Surveillance (12 questions), and 3) ETV Surveillance (7 questions)., Results: A total of 122 complete responses were obtained, for an overall response rate of 88%. The majority of respondents have been in practice for more than 10 years (58%) and exclusively treat pediatric patients (79%). Most respondents consider hydrocephalus to have stabilized 1 month (21%) or 3 months (39%) after shunt surgery, and once stability is achieved, 72% then ask patients to return for routine clinical follow-up annually. Overall, 83% recommend lifelong clinical follow-up after shunt placement. Additionally, 75% obtain routine imaging studies in asymptomatic patients, although the specific imaging modality and frequency of imaging vary. The management of an asymptomatic increase in ventricle size or an asymptomatic catheter fracture also varies widely. Many respondents believe that hydrocephalus takes longer to stabilize after ETV than after shunt placement, reporting that they consider hydrocephalus to have stabilized 3 (28%), 6 (33%), or 12 (28%) months after an ETV. Although 68% of respondents have patients return annually for routine clinical follow-up after an ETV, only 56% recommend lifelong follow-up. The proportion of respondents who perform lifelong follow-up increases with greater practice experience (p = 0.01). Overall, 67% of respondents obtain routine imaging studies in asymptomatic patients after an ETV, with "rapid" MRI the study of choice for most respondents., Conclusions: While there is a general consensus among pediatric neurosurgeons across North America that hydrocephalus patients should have long-term follow-up after shunt placement, radiological surveillance is characterized by considerable variety, as is follow-up after an ETV. Future work should focus on evaluating whether any one of these surveillance protocols is associated with improved outcomes.

Following a decompressive craniectomy, the autologous bone flap is generally considered the reconstructive material of choice in pediatric patients. Replacement of the original bone flap takes advantage of its natural biocompatibility and the associated low risk of rejection, as well as the potential to reintegrate with the adjacent bone and subsequently grow with the patient. However, despite these advantages and unlike adult patients, the replaced calvarial bone is more likely to undergo delayed bone resorption in pediatric patients, ultimately requiring revision surgery. In this review, we describe the materials that are currently available for pediatric cranioplasty, the advantages and disadvantages of autologous calvarial replacement, the incidence and classification of bone resorption, and the clinical risk factors for bone flap resorption that have been identified to date., (© Congress of Neurological Surgeons 2021.)

Objective: Neurosurgical residents receive exposure to the subspecialty of pediatric neurosurgery during training. The authors sought to determine resident operative experience in pediatric neurosurgery across Accreditation Council for Graduate Medical Education (ACGME)-accredited neurosurgical programs., Methods: During 2018-2019, pediatric neurosurgical case logs for recent graduates or current residents who completed their primary pediatric exposure were collected from US continental ACGME training programs. Using individual resident reports and procedure designations, operative volumes and case diversity were analyzed collectively, according to training site characteristics, and also correlated with the recently described Resident Experience Score (RES)., Results: Of the 114 programs, a total of 316 resident case logs (range 1-19 residents per program) were received from 86 (75%) programs. The median cumulative pediatric case volume per resident was 109 (IQR 75-161). Residents at programs with a pediatric fellowship reported a higher median case volume (143, IQR 96-187) than residents at programs without (91, IQR 66-129; p < 0.0001). Residents at programs that outsource their pediatric rotation had a lower median case volume (84, IQR 52-114) compared with those at programs with an in-house experience (117, IQR 79-170; p < 0.0001). The case diversity index among all programs ranged from 0.61 to 0.80, with no statistically significant differences according to the Accreditation Council for Pediatric Neurosurgery Fellowships designation or pediatric experience site (p > 0.05). The RES correlated moderately (r = 0.44) with median operative volumes per program. A program's annual pediatric operative volume and duration of pediatric experience were identified as significant predictive factors for median resident operative volume., Conclusions: Resident experience in pediatric neurosurgery is variable within and between programs. Case volumes are generally higher for residents at programs with in-house exposure and an accredited fellowship, but case diversity is relatively uniform across all programs. RES provides some insight on anticipated case volume, but other unexplained factors remain.

Surgical options for metopic craniosynostosis include the traditional open approach or a minimally invasive approach that typically involves an endoscopy-assisted strip craniectomy. The minimally invasive approach has been associated with less blood loss and operative time, a lower transfusion rate, and a shorter length of stay. Additionally, it is more cost-effective than open reconstruction, despite the need for a postoperative cranial orthosis and multiple follow-up visits. The authors describe a variation of the minimally invasive approach using a lighted retractor to perform a strip craniectomy of the metopic suture in a 2-month-old patient with metopic craniosynostosis. The video can be found here: https://vimeo.com/511237503., Competing Interests: Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this publication., (© 2021, The Authors.)

Objective: Surgical education has increasingly relied on electronic learning. In particular, online operative videos have become a core resource within neurosurgery. We analyze the forums for neurosurgical operative videos., Methods: Operative videos from 5 sources were reviewed: 1) the NEUROSURGERY Journal YouTube channel; 2) the American Association of Neurological Surgeons Neurosurgery YouTube channel; 3) The Neurosurgical Atlas Operative Video Cases; 4) Operative Neurosurgery; and 5) Neurosurgical Focus: Video. Title, year of publication, senior author, institution, country, and subspecialty were documented for each video., Results: A total of 1233 videos showing 1247 surgeries were identified. Ten videos included >1 surgery; of those, there was a median of 2 surgeries (interquartile range, 2.0-2.5) per video. The most frequently represented subspecialties included vascular (48.3%), tumor (35.2%), and skull base surgery (27.5%), with almost 40% of videos showing >1 category. Videos were submitted by investigators from 28 countries, but 82.1% of the videos originated in the United States., Conclusions: Neurosurgical operative videos have become increasingly common through a variety of online platforms. Future efforts may benefit from collecting videos from underrepresented regions and subspecialties, providing long-term follow-up data and showing techniques for managing complications., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: The spectrum of injury severity for abusive head trauma (AHT) severity is broad, but outcomes are unequivocally worse than accidental trauma. There are few publications that analyze different outcomes of AHT., Objective: To determine variables associated with different outcomes of AHT., Methods: Patients were identified using our AHT database. Three different, but not mutually exclusive, outcomes of AHT were modeled: (1) death or hemispheric stroke (diffuse loss of grey-white differentiation); (2) stroke(s) of any size; and (3) need for a neurosurgical operation. Demographic and clinical variables were collected and correlations to the 3 outcomes of interest were identified using bivariate and multivariable analysis., Results: From January 2009 to December 2017, 305 children were identified through a prospectively maintained AHT database. These children were typically male (60%), African American (54%), and had public or no insurance (90%). A total of 29 children (9.5%) died or suffered a massive hemispheric stroke, 57 (18.7%) required a neurosurgical operation, and 91 (29.8%) sustained 1 or more stroke. Death or hemispheric stroke was statistically associated with the pupillary exam (odds ratio [OR] = 45.7) and admission international normalized ratio (INR) (OR = 17.3); stroke was associated with the pupillary exam (OR = 13.2), seizures (OR = 14.8), admission hematocrit (OR = 0.92), and INR (9.4), and need for surgery was associated with seizures (OR = 8.6)., Conclusion: We have identified several demographic and clinical variables that correlate with 3 clinically applicable outcomes of abusive head injury., (Copyright © 2020 by the Congress of Neurological Surgeons.)

Background: Endoscopic third ventriculostomy (ETV) is an effective primary treatment for certain forms of hydrocephalus. However, its use in children with an existing shunt is less well known., Objective: To report a multicenter experience in attempting to convert patients from shunt dependence to a third ventriculostomy and to determine predictors of success., Methods: Three participating centers provided retrospectively collected information on patients with an attempted conversion from a shunt to an ETV between December 1, 2008, and April 1, 2018. Demographic, clinical, and radiological data were recorded. Success was defined as shunt independence at the last follow-up., Results: Eighty patients with an existing ventricular shunt underwent an ETV. The median age at the time of the index ETV was 9.9 yr, and 44 (55%) patients were male. The overall success rate was 64% (51/80), with a median duration of follow-up of 2.0 yr (range, 0.1-9.4 yr). Four patients required a successful repeat ETV at a median of 1.7 yr (range, 0.1-5.7 yr) following the index ETV. Only age was predictive of ETV failure on multivariate analysis (odds ratio 0.86 [95% CI 0.78-0.94], P = .005). No patient less than 6 mo of age underwent an ETV, and of the 5 patients between 6 and 12 mo of age, 4 failed., Conclusion: Although not every shunted patient will be a candidate for an ETV, nor will they be successfully converted, an ETV should at least be considered in every child who presents with a shunt malfunction or who has an externalized shunt., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Objective: Biopsies of brainstem lesions are performed to establish a diagnosis in the setting of an atypical clinical or radiological presentation, or to facilitate molecular studies. A better understanding of the safety and diagnostic yield of brainstem biopsies would help guide appropriate patient selection., Methods: All patients who underwent biopsy of a brainstem lesion during the period from January 2011 to June 2019 were reviewed. Demographic, radiological, surgical, and outcome data were collected., Results: A total of 58 patients underwent 65 brainstem biopsies during the study period. Overall, the median age was 7.6 years (IQR 3.9-14.2 years). Twenty-two of the 65 biopsies (34%) were open, 42 (65%) were stereotactic, and 1 was endoscopic. In 3 cases (5%), a ventriculoperitoneal shunt was placed, and in 9 cases (14%), a posterior fossa decompression was performed during the same operative session as the biopsy. An intraoperative MRI (iMRI) was performed in 28 cases (43%). In 3 of these cases (11%), the biopsy was off target and additional samples were obtained during the same procedure. New neurological deficits were noted in 5 cases (8%), including sensory deficits, ophthalmoparesis/nystagmus, facial weakness, and hearing loss; these deficits persisted in 2 cases and were transient in 3 cases. A pseudomeningocele occurred in 1 patient; no patients developed a CSF leak or infection. In 8 cases (13%) an additional procedure was needed to obtain a diagnosis., Conclusions: Brainstem biopsies are safe and effective. Target selection and approach should be a collaborative effort. iMRI can be used to assess biopsy accuracy in real time, thereby allowing any adjustment if necessary.

Background: Incontrovertible predictors of shunt malfunction remain elusive., Objective: To determine predictors of shunt failure within 30 d of index surgery., Methods: This was a single-center retrospective cohort study from January 2010 through November 2016. Using a ventricular shunt surgery research database, clinical and procedural variables were procured. An "index surgery" was defined as implantation of a new shunt or revision or augmentation of an existing shunt system. The primary outcome was shunt failure of any kind within the first 30 days of index surgery. Bivariate models were created, followed by a final multivariable logistic regression model using a backward-forward selection procedure., Results: Our dataset contained 655 unique patients with a total of 1206 operations. The median age for the cohort at the time of first shunt surgery was 4.6 yr (range, 0-28; first and third quartile, .37 and 11.8, respectively). The 30-day failure rates were 12.4% when analyzing the first-index operation only (81/655), and 15.7% when analyzing all-index operations (189/1206). Small or slit ventricles at the time of index surgery and prior ventricular shunt operations were found to be significant covariates in both the "first-index" (P < .01 and P = .05, respectively) and "all-index" (P = .02 and P < .01, respectively) multivariable models. Intraventricular hemorrhage at the time of index surgery was an additional predictor in the all-index model (P = .01)., Conclusion: This study demonstrates that only 3 variables are predictive of 30-day shunt failure when following established variable selection procedures, 2 of which are potentially under direct control of the surgeon., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Objective: In pediatric patients, the development of a postoperative pseudomeningocele after an elective craniotomy is not unusual. Most will resolve with time, but some may require intervention. In this study, the authors analyzed patients who required intervention for a postoperative pseudomeningocele following an elective craniotomy or craniectomy and identified factors associated with the need for intervention., Methods: An institutional operative database of elective craniotomies and craniectomies was queried to identify all surgeries associated with development of a postoperative pseudomeningocele from January 1, 2010, to December 31, 2017. Demographic and surgical data were collected, as were details regarding postoperative events and interventions during either the initial admission or upon readmission. A bivariate analysis was performed to compare patients who underwent observation with those who required intervention., Results: Following 1648 elective craniotomies or craniectomies, 84 (5.1%) clinically significant pseudomeningoceles were identified in 82 unique patients. Of these, 58 (69%) of the pseudomeningoceles were diagnosed during the index admission (8 of which persisted and resulted in readmission), and 26 (31%) were diagnosed upon readmission. Forty-nine patients (59.8% of those with a pseudomeningocele) required one or more interventions, such as lumbar puncture(s), lumbar drain placement, wound exploration, or shunt placement or revision. Only race (p < 0.01) and duraplasty (p = 0.03, OR 3.0) were associated with the need for pseudomeningocele treatment., Conclusions: Clinically relevant pseudomeningoceles developed in 5% of patients undergoing an elective craniotomy, with 60% of these pseudomeningoceles needing some form of intervention. The need for intervention was associated with race and whether a duraplasty was performed.