Magnetic Resonance Fingerprinting to Characterize Childhood and Young Adult Brain Tumors.

OBJECT: Magnetic resonance fingerprinting (MRF) allows rapid, simultaneous mapping of T1 and T2 relaxation times and may be an important diagnostic tool to measure tissue characteristics in pediatric brain tumors. We examined children and young adults with primary brain tumors to determine whether MRF can discriminate tumor from normal-appearing white matter and distinguish tumor grade. METHODS: MRF was performed in 23 patients (14 children and 9 young adults) with brain tumors (19 low-grade glioma, 4 high-grade tumors). T1 and T2 values were recorded in regions of solid tumor (ST), peritumoral white matter (PWM), and contralateral white matter (CWM). Nonparametric tests were used for comparison between groups and regions. RESULTS: Median scan time for MRF and a sequence for tumor localization was 11 min. MRF-derived T1 and T2 values distinguished ST from CWM (T1: 1,444 ± 254 ms vs. 938 ± 96 ms, p = 0.0002; T2: 61 ± 22 ms vs. 38 ± 9 ms, p = 0.0003) and separated high-grade tumors from low-grade tumors (T1: 1,863 ± 70 ms vs. 1,355 ± 187 ms, p = 0.007; T2: 90 ± 13 ms vs. 56 ± 19 ms, p = 0.013). PWM was distinct from CWM (T1: 1,261 ± 359 ms vs. 933 ± 104 ms, p = 0.0008; T2: 65 ± 51 ms vs. 38 ± 8 ms, p = 0.008), as well as from tumor (T1: 1,261 ± 371 ms vs. 1,462 ± 248 ms, p = 0.047). CONCLUSIONS: MRF is a fast sequence that can rapidly distinguish important tissue components in pediatric brain tumor patients. MRF-derived T1 and T2 distinguished tumor from normal-appearing white matter, differentiated tumor grade, and found abnormalities in peritumoral regions. MRF may be useful for rapid quantitative measurement of tissue characteristics and distinguish tumor grade in children and young adults with brain tumors.

Vagal nerve stimulation for medically refractory epilepsy in Angelman syndrome: a series of three cases.

BACKGROUND: We describe three children with Angelman syndrome and medically refractory epilepsy. METHODS: Case series of three pediatric patients with Angelman syndrome and medically refractory epilepsy. All three patients failed medical treatment and were recommended for vagal nerve stimulator (VNS) implantation. RESULTS: Following VNS implantation, all three patients experienced reduction in seizure frequency greater than that afforded by medication alone. CONCLUSION: We present vagal nerve stimulator implantation as a viable treatment option for medically refractory epilepsy associated with Angelman syndrome.

Acute Management of Tension Pneumocephalus in a Pediatric Patient: A Case Report.

BACKGROUND: Tension pneumocephalus is a rare but life-threatening condition in which air gains entry into the cranium and exerts mass effect on the brain, resulting in increased intracranial pressure. It occurs most frequently secondary to head trauma, particularly to the orbits or sinuses. CASE REPORT: A 13-year-old male sustained facial trauma from a motor vehicle collision and was found to have tension pneumocephalus on computer tomography. The patient underwent immediate rapid sequence intubation without preceding positive pressure ventilation in the emergency department. At the time of his craniotomy, the tension pneumocephalus was found to have resolved and he went on to have a complete recovery. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Any patient with facial or head trauma and pneumocephalus is at risk for the potential development of tension pneumocephalus. When present, we advocate that aggressive definitive airway management by rapid sequence intubation without preceding positive pressure ventilation and early surgical management should be prioritized.

The Evolution of Cerebrospinal Fluid Shunts: Advances in Technology and Technique.

Over the past few centuries, there have been many advances in shunt technology, from the evolution of shunt tubing materials, to advances in valves for regulating the flow of cerebrospinal fluid (CSF). Additional medical advances have enabled the antibiotic impregnation of catheters. Finally, advances in technology have expanded the options for minimally invasive techniques and improved the management of complicated cases of hydrocephalus. The evolution of technology and technique in the management of hydrocephalus with CSF shunts will be discussed here.

Successful delayed non-operative management of C2 neurosynchondrosis fractures in a pediatric patient: a case report and review of management strategies and considerations for treatment.

Upper cervical synchondrosis fractures are the most common types of cervical fractures in the pediatric population less than 7 years of age (Blauth et al (Eur Spine J 5:63-70, 1996); Connolly et al (Pediatr Radiol 25(Suppl1):S129-133, 1995); Mandabach et al (Pediatr Neurosurg 19(5):225-232, 1993); Schippers et al (Acta Neurochir 138:524-530, 1990)) The vast majority occur through the dentocentral or basilar synchondrosis. We present the second reported case in recent literature of a unilateral neurosynchondrosis fracture. The patient, a 4-year-old male, was initially managed conservatively with a rigid cervical collar for a period of 3 months. Despite multiple counseling sessions with family, the patient remained poorly compliant with cervical immobilization. After 3 months, imaging demonstrated partial fusion with persistent anterolisthesis of C2 on C3. The decision was made to place the child in external halo fixation as an attempt to achieve fusion prior to committing to internal surgical fixation and the associated sequelae. Immobilization with a hard cervical collar is often first line treatment. In the case of failed fusion, debate exists regarding surgical fixation in children. Occipito-atlanto-axial fusion leads to permanent loss of a significant degree of flexion, extension, and rotatory movement. The pediatric population has a strong propensity to fuse; however, compliance is often a barrier to conservative treatment due to age-related behavioral practices. We demonstrate that even with initial failed fusion and progression of deformity while in a cervical collar, conservative management with external halo fixation can potentially obviate the need for internal fixation.

Postnatal Pancraniosynostosis in a Patient With Infantile Hypophosphatasia.

Hypophosphatasia is a rare metabolic bone disorder that predisposes patients to craniosynostosis. Typically, patients born with hypophosphatasia will exhibit fused cranial sutures at birth. This is the first reported case of delayed onset of pancraniosynostosis in a patient with infantile hypophosphatasia. The severity of onset and delayed presentation in this patient are of interest and should give pause to those care providers who treat and evaluate patients with hypophosphatasia.

The association of COVID-19 with increased pediatric ventriculoatrial shunt failures: a national retrospective cohort.

OBJECTIVE: During the COVID-19 pandemic, the authors' institution managed ventriculoatrial (VA) shunt complications in 2 teenage patients in close proximity to a symptomatic COVID-19 infection. Systemic thrombotic events are an established complication of COVID-19 infection due to a hypercoagulable state. Thrombotic complications, particularly elevated central venous pressure, can cause VA shunt failure. The true effect of COVID-19 on patients with intravascular devices is currently unknown. In this study, the authors aimed to determine if there was an association between COVID-19 infection and VA shunt failure. METHODS: TriNetX, an aggregated electronic health record platform, was used to analyze data of more than 13 million US pediatric patients. Two matched cohorts of patients < 18 years of age with a VA shunt were defined. Group 1 (n = 311) had a positive laboratory test for COVID-19 from March 1, 2020, to March 31, 2022. Group 2 (n = 311), a control group, had any medical appointment from March 1, 2020, to March 31, 2022, and never had a positive laboratory test for COVID-19. The authors analyzed outcomes 1 year after testing positive for COVID-19 in group 1, and after the medical appointment in group 2. Outcomes included shunt complications, shunt revisions or replacements, and thromboembolic complications. To protect patient privacy, individual results of fewer than 10 patients are not specified in TriNetX. RESULTS: Group 1 had a greater odds of mechanical shunt complication than group 2 (20% vs 4%, OR 5.71, 95% CI 3.07-10.62). Group 1 had a greater odds of shunt reoperation than group 2 (11% vs < 3%, OR > 3.7, 95% CI 1.72-7.62). There were 1-10 patients in group 1 (≤ 3% of group 1) who experienced a thromboembolism due to the shunt, compared with no patients in group 2 who had a thromboembolism due to the shunt. CONCLUSIONS: This analysis demonstrates an association of shunt complications, reoperations, and thromboembolic events in patients with VA shunts following COVID-19 infection.

Survey of United States neurosurgeons on firearm injury prevention.

OBJECTIVE: Firearm-related injuries and deaths are an endemic problem in the US, posing a burden on the healthcare system with significant social and economic consequences. As front-line care providers for these patients, neurosurgeons are both knowledgeable about these injuries and credible messengers in the public discussion of ways to reduce firearm injuries. The purpose of this study was to explore US-based neurosurgeons' views and behaviors regarding firearms to understand and define a potential role for neurosurgical organizations in advocacy efforts to reduce firearm death and injuries. METHODS: The authors conducted an anonymous survey of US neurosurgeons using the American Association of Neurological Surgeons (AANS) member database from April to June 2023. The 22-question survey included questions related to firearm ownership, personal views on firearms, and support for both general and policy-specific advocacy efforts to reduce firearm deaths and injuries. RESULTS: The survey response rate was 20.7%, with 1568 of the 7587 members invited completing the survey. The survey completion rate was 93.4%, with 1465 of the 1568 surveys completed and included in this analysis. The majority of respondents were male (raw: 81.7%; weighted 81.1%), White (raw: 69.7%; weighted 70.2%), and older than 50 years (raw: 56.2%; weighted: 54%). Most respondents reported treating patients with firearm injuries (raw: 83.3%; weighted: 82%), 85.5% (weighted: 85.1%) had used a firearm, and 42.4% (weighted: 41.5%) reported owning a firearm. Overall, 78.8% (weighted: 78.7%) of respondents felt that organized neurosurgery should participate in advocacy efforts. When examining individual policies, those that restrict the acquisition of firearms garnered the support of at least 65% of respondents, while nonrestrictive policies were supported by more than 75% of respondents. Free-text responses provided insight into both motivations for and objections to organizational advocacy. CONCLUSIONS: The majority of US-based neurosurgeons support involvement in advocacy efforts to reduce firearm deaths and injuries. Themes expressed by members both supporting and objecting to advocacy provide insight into approaches that could ensure broad support. Neurosurgical organizations such as the AANS and Congress of Neurological Surgeons may use the results of this survey to make informed decisions regarding involvement in advocacy efforts on behalf of their membership to lessen the burden of firearm injury in the US.

Spontaneous hemorrhage of a facial neurofibroma: endovascular embolization before surgical intervention.

A 50-year-old man with neurofibromatosis type 1 presented to the emergency department after a spontaneous hemorrhage into a facial plexiform neurofibroma. An emergent selective angiography of the external carotid artery was performed with Onyx embolization of the origin of the internal maxillary artery as well as the superficial temporal artery to minimize arterial bleeding during the subsequent operative hematoma evacuation. This technique was aimed to control blood loss during the surgical procedure and reduce the amount of transfusion product required for resuscitation. This technique provides a viable option to control potentially life-threatening arterial bleeding in emergent settings.

Complete regression of papillary tumor of the pineal region after radiation therapy: case report and review of the literature.

Papillary tumor of the pineal region (PTPR) is a rare neuroepithelial tumor that arises in the pineal region. The optimal treatment for PTPR remains controversial, as no definitive treatment strategy exists for this lesion. It is not clear whether aggressive surgical removal is superior to biopsy followed by radiotherapy. The majority of cases in the literature have undergone attempted gross total resection with a supracerebellar-infratentorial or a transcallosal-transventricular approach. In this report, we describe a case of PTPR in a 23 year-old male that presented as a third ventricular mass causing obstructive hydrocephalus. An endoscopic third ventriculostomy was performed followed by an endoscopic biopsy. Postoperative radiotherapy resulted in complete regression of the tumor with no evidence of tumor recurrence at 25 months. This case highlights a minimally invasive strategy for a rare neoplasm that resulted in a favorable response to radiation therapy, thereby avoiding the risks of aggressive surgical removal. We also review the radiographic and histopathologic features of PTPR and discuss various options of treatment reported in the literature.

Comparative analysis of state-level concussion legislation and review of current practices in concussion.

OBJECT: Forty-two states and the District of Columbia have passed legislation based on the Lystedt law of Washington state, enacted in 2009 to protect young athletes who have sustained a concussion. The aim of this study was to note the several similarities and differences among the various laws. METHODS: Concussion legislation was compared for 50 states and the District of Columbia. Evaluation parameters of this study included stipulations of concussion education, criteria for removal from play, requirements for evaluation and return to play after concussion, and individuals required to assess young athletes. Other parameters that were not consistent across states were also noted. RESULTS: Forty-three states and the District of Columbia have passed concussion legislation, and an additional 4 states have pending legislation. All states with existing legislation support concussion education for coaches; however, only 48% require coaches to undergo formal education. Athletes must be educated on concussion in 86% of states and parents in 88.7%. Suspicion of concussion is a criterion for removal from play in 75% of states; signs and symptoms of concussion are criteria for removal from play in 16% of states. The individuals allowed to evaluate and clear an athlete for return to play differ greatly among states. CONCLUSIONS: Injury prevention legislation holds historical precedent, and given the increasing attention to long-term sequelae of repeated head trauma and concussion, concussion legislation has been rapidly passed in 43 states and the District of Columbia. Although the exact stipulations of these laws vary among states, the overall theme is to increase recognition of concussion in young athletes and ensure that they are appropriately cleared for return to play after concussion.

Lipomyelomeningocele: pathology, treatment, and outcomes.

Lipomyelomeningocele represents a rare but complex neurological disorder that may present with neurological deterioration secondary to an inherent tethered spinal cord. Radiological testing is beneficial in determining the morphology of the malformation. Specialized testing such as urodynamic studies and neurophysiological testing may be beneficial in assessing for neurological dysfunction secondary to the lipomyelomeningocele. Early surgical intervention may be beneficial in preventing further neurological decline.

Management of urological dysfunction in pediatric patients with spinal dysraphism: review of the literature.

An intact, fully functional spine is the result of a complex sequence of embryological events involving both nervous and musculoskeletal system precursors. Deviations from this highly ordered system can result in congenital abnormalities ranging from clinically insignificant cosmetic changes to CNS malformations that are incompatible with life. Closure of the neural tube, which is believed to be the embryological event gone awry in these cases, is complete by just 28 days' gestation, often before pregnancy is detected. Although progress has been made to help prevent neural tube defects in the children of those attempting to conceive, these congenital deformities unfortunately continue to affect a startling number of infants worldwide each year. Furthermore, the precise mechanisms governing closure of the neural tube and how they might be interrupted remain elusive. What is known is that there are a large number of individuals who must deal with congenital spine dysraphism and the clinical sequelae on a daily basis. Bladder and urinary dysfunction are frequently encountered, and urological care is a critical, often neglected, component in the lifelong multidisciplinary approach to treatment. Although many treatment strategies have been devised, a need remains for evidence-based interventions, analysis of quality of life, and preemptive education of both caregivers and patients as they grow older. Pediatric neurosurgeons in particular have the unique opportunity to address these issues, often in the first few days of life and throughout pre- and postoperative evaluation. With proper management instituted at birth, many patients could potentially delay or avoid the potential urological complications resulting from congenital neurogenic bladder.

Best practices for the pregnant neurosurgical resident: balancing safety and education.

Establishment of a diverse neurosurgical workforce includes increasing the recruitment of women in neurosurgery. The impact of pregnancy on the training and career trajectory of female neurosurgeons poses a barrier to recruitment and retention of women in neurosurgery. A recent Women in Neurosurgery survey evaluated female neurosurgeons' perception and experience regarding childbearing of female neurosurgeons and identified several recommendations regarding family leave policies. Additionally, pregnancy may carry higher risk in surgical fields, yet little guidance exists to aid both the pregnant resident and her training program in optimizing the safety of the training environment with specific considerations to risks inherent in neurosurgical training. This review of current literature aims to address best practices that can be adopted by pregnant neurosurgery residents and their training programs to improve the well-being of these residents while considering the impact on their education and the educational environment for their colleagues.

Beyond Training the Next Generation of Physicians: The Unmeasured Value Added by Residents to Teaching Hospitals and Communities.

Following medical school, most newly graduated physicians enter residency training. This period of graduate medical education (GME) is critical to creating a physician workforce with the specialized skills needed to care for the population. Completing GME training is also a requirement for obtaining medical licensure in all 50 states. Yet, crucial federal and state funding for GME is capped, creating a bottleneck in training an adequate physician workforce to meet future patient care needs. Thus, additional GME funding is needed to train more physicians. When considering this additional GME funding, it is imperative to take into account not only the future physician workforce but also the value added by residents to teaching hospitals and communities during their training. Residents positively affect patient care and health care delivery, providing intrinsic and often unmeasured value to patients, the hospital, the local community, the research enterprise, and undergraduate medical education. This added value is often overlooked in decisions regarding GME funding allocation. In this article, the authors underscore the value provided by residents to their training institutions and communities, with a focus on current and recent events, including the global COVID-19 pandemic and teaching hospital closures.

Telehealth in Neurosurgery: 2021 Council of State Neurosurgical Societies National Survey Results.

OBJECTIVE: Telehealth was rapidly adopted during the COVID-19 pandemic. A survey was distributed to neurosurgeons in the United States (U.S.) to understand its use within neurosurgery, what barriers exist, unique issues related to neurosurgery, and opportunities for improvement. METHODS: A survey was distributed via email and used the SurveyMonkey platform. The survey was sent to 3828 practicing neurosurgeons within the U.S., 404 responses were collected between October 30, 2021 and December 4, 2021. RESULTS: During the pandemic, telehealth was used multiple times per week by 60.65% and used daily by an additional 12.78% of respondents. A supermajority (89.84%) of respondents felt that evaluating patients across state lines with telemedicine is beneficial. Most respondents (95.81%) believed that telehealth improves patient access to care. The major criticism of telehealth was the inability to perform a neurological exam. CONCLUSIONS: Telehealth has been widely implemented within the field of neurosurgery during the COVID-19 pandemic and has increased access to care. It has allowed patients to be evaluated remotely, including across state lines. While certain aspects of the neurological exam are suited for video evaluation, sensation and reflexes cannot be adequately assessed. Neurosurgeons believe that telehealth adds value to their ability to deliver care.

Noninvasive evaluation of craniovertebral junction instability in 2 patients following Chiari decompression with rigid C-collar immobilization: illustrative cases.

BACKGROUND: The complex Chiari malformation has been identified in a subset of Chiari patients at higher risk for worsening symptoms following Chiari decompression. Although parameters such as the clivoaxial angle and the perpendicular distance of the dens to the line from the basion to the inferoposterior part of the C2 body (pBC2) have been evaluated to help with the prediction of risk, the decision to pursue an occipitocervical fusion in lower-risk patients does not come without inherent risk. OBSERVATIONS: The authors present 2 patients who had symptoms of worsening ventral brainstem compression following Chiari decompression, neither of whom was categorized in the highest risk category for occipitocervical instability. In addition, neither patient had gross instability on radiographic imaging. A trial with rigid C-collar immobilization provided relief of symptoms in both patients and allowed reassurance of the likelihood of success of occipitocervical fusion. LESSONS: In patients without clear radiographic instability following Chiari decompression, a C-collar trial may provide a noninvasive option for assessing the potential success of occipitocervical fusion.

Evaluation of nonaccidental trauma in infants presenting with skull fractures: a retrospective review.

OBJECTIVE: Nonaccidental trauma (NAT) is one of the leading causes of serious injury and death among young children in the United States, with a high proportion of head injury. Numerous studies have demonstrated the safety of discharge of infants with isolated skull fractures (ISFs); however, these same studies have noted that those infants with suspected abuse should not be immediately discharged. The authors aimed to create a standardized protocol for evaluation of infants presenting with skull fractures to our regional level I pediatric trauma center to best identify children at risk. METHODS: A protocol for evaluation of NAT was developed by our pediatric trauma committee, which consists of evaluation by neurosurgery, pediatric surgery, and ophthalmology, as well as the pediatric child protection team. Social work evaluations and a skeletal survey were also utilized. Patients presenting over a 2-year period, inclusive of all infants younger than 12 months at the time of presentation, were assessed. Factors at presentation, protocol compliance, and the results of the workup were evaluated to determine how to optimize identification of children at risk. RESULTS: A total of 45 infants with a mean age at presentation of 5.05 months (SD 3.14 months) were included. The most common stated mechanism of injury was a fall (75.6%), followed by an unknown mechanism (22.2%). The most common presenting symptoms were swelling over the fracture site (25 patients, 55.6%), followed by vomiting (5 patients, 11.1%). For the entire population of patients with skull fractures, there was suspicion of NAT in 24 patients (53.3% of the cohort). Among the 30 patients with ISFs, there was suspicion of NAT in 13 patients (43.3% of the subgroup). CONCLUSIONS: Infants presenting with skull fractures with intracranial findings and ISFs had a substantial rate of concern for the possibility of nonaccidental skull fracture. Although prior studies have demonstrated the relative safety of discharging infants with ISFs, it is critical to establish an appropriate standardized protocol to evaluate for infants at risk of abusive head trauma.

Impact of multidisciplinary engagement in a quality improvement blood conservation protocol for craniosynostosis.

OBJECTIVE: Patients undergoing open cranial vault remodeling for craniosynostosis frequently experience substantial blood loss requiring blood transfusion. Multiple reports in the literature have evaluated the impact of individual blood conservation techniques on blood transfusion rates during craniosynostosis surgery. The authors engaged a multidisciplinary team and assessed the impact of input from multiple stakeholders on the evolution of a comprehensive quality improvement protocol aimed at reducing or eliminating blood transfusion in patients undergoing open surgery for craniosynostosis. METHODS: Over a 4-year period from 2012 to 2016, 39 nonsyndromic patients were operated on by a single craniofacial plastic surgeon. Initially, no clear blood conservation protocol existed, and specific interventions were individually driven. In 2014, a new pediatric neurosurgeon joined the craniofacial team, and additional stakeholders in anesthesiology, transfusion medicine, critical care, and hematology were brought together to evaluate opportunities for developing a comprehensive blood conservation protocol. The initial version of the protocol involved the standardized administration of intraoperative aminocaproic acid (ACA) and the use of a cell saver. In the second version of the protocol, the team implemented the preoperative use of erythropoietin (EPO). In addition, intraoperative and postoperative resuscitation and transfusion guidelines were more clearly defined. The primary outcomes of estimated blood loss (EBL), transfusion rate, and intraoperative transfusion volume were analyzed. The secondary impact of multidisciplinary stakeholder input was inferred by trends in the data obtained with the implementation of the partial and full protocols. RESULTS: Implementing the full quality improvement protocol resulted in a 66% transfusion-free rate at the time of discharge compared to 0% without any conservation protocol and 27% with the intermediate protocol. The administration of EPO significantly increased starting hemoglobin/hematocrit (11.1 g/dl/31.8% to 14.7 g/dl/45.6%, p < 0.05). The group of patients receiving ACA had lower intraoperative EBL than those not receiving ACA, and trends in the final-protocol cohort, which had received both preoperative EPO and intraoperative ACA, demonstrated decreasing transfusion volumes, though the decrease did not reach statistical significance. CONCLUSIONS: Patients undergoing open calvarial vault remodeling procedures benefit from the input of a multidisciplinary stakeholder group in blood conservation protocols. Further research into comprehensive protocols for blood conservation may benefit from input from the full surgical team (plastic surgery, neurosurgery, anesthesiology) as well as additional pediatric subspecialty stakeholders including transfusion medicine, critical care, and hematology.

A Precursor to Multiloculated Hydrocephalus: Case Report and Review of Literature.

BACKGROUND: Multiloculated hydrocephalus (MH) is a challenging pathology for pediatric neurosurgeons, arising from various etiologies including intraventricular hemorrhage, infection, and overshunting. Although previous publications have discussed the potential etiology of this pathological process, including fibroglial webbing, no clear precursor has been proven. We present a case of MH developing after both intraventricular hemorrhage and intraventricular infection, with visualization of the precursor via endoscopy and a confirmed glial scar on pathological examination. CASE DESCRIPTION: Our patient is an preterm-born (at 24 weeks of gestation) male with a grade III intraventricular hemorrhage treated with reservoir placement and serial taps. He did not develop posthemorrhagic hydrocephalus but presented back at approximately 4 months of age with Escherichia coli meningitis that necessitated multiple interventions for intraventricular abscesses, including an endoscopic exploration. He ultimately developed MH requiring placement of a ventriculoperitoneal shunt. CONCLUSIONS: MH is a complex pathology with multiple risk factors. To date, only theories regarding the etiology have been proposed. Our case represents the first known direct visualization of intraventricular fibroglial webbing with magnetic resonance imaging correlation. Improved understanding of the pathophysiology of this entity may improve our ability to treat this pathology before loculations develop.