Journal of neurosurgery. Pediatrics最新文献

Objective: Intraoperative magnetic resonance imaging (iMRI) use is becoming increasingly widespread in neurosurgical practice, and most of the data reporting its use are in adult populations. There is less evidence on the use of iMRI in pediatric neurosurgery. The aim of this paper was to synthesize the available literature into a systematic review and meta-analysis to evaluate the evidence for iMRI in pediatric neurosurgery, with a particular focus on neuro-oncology and epilepsy surgery.

Methods: This review was registered on PROSPERO and conducted according to PRISMA guidelines. A comprehensive search strategy of Medline via Ovid and Embase was conducted with predetermined key terms. Studies in English reporting the outcomes of patients < 21 years of age who underwent neuro-oncological or epilepsy surgery with the use of iMRI were included in the study. The types of studies eligible for inclusion were observational case-control and cohort studies, randomized clinical trials, cross-sectional studies, editorials, case series, and commentaries. Articles were de-duplicated and abstracts independently screened for inclusion by two reviewers. Full texts were screened, and data on demographic characteristics, etiology, outcome (extent of resection for neuro-oncology and Engel class for epilepsy), and technical iMRI data were extracted.

Results: Thirty-five articles were included in the review, 25 of which were observational cohort studies. Four articles were suitable for meta-analysis. In total, 1217 patients underwent iMRI-guided neuro-oncology surgery in 26 studies, most commonly for gliomas (n = 443). A total of 148 patients underwent iMRI-guided epilepsy surgery in 9 studies, with focal cortical dysplasia being the most common diagnosis (n = 95). The mean ± SD operating time was 357 ± 94 minutes (12 studies), with a mean of 1.32 scans per patient. There was a mean re-entry rate into the operative field of 42% (across 20 studies). Complications were noted in 21% of epilepsy surgery patients and 11% of neuro-oncology surgery patients. Meta-analysis of 4 eligible studies revealed that iMRI was more likely to lead to better Engel outcomes in terms of seizure freedom (OR 3.84, 95% CI 1.38-10.68, p = 0.69) and complete tumor resection (OR 3.19, 95% CI 0.28-36.92, p = 0.06).

Conclusions: iMRI appears to be a useful adjunct in optimizing resective pediatric epilepsy and neuro-oncology surgery, with a low complication rate.

Pediatric arteriovenous malformations (AVMs) are rare but carry a risk of devastating neurological morbidity and mortality. Rupture of a cerebral AVM is the most common cause of spontaneous intracranial hemorrhage in children, with an unruptured AVM having an approximate hemorrhage risk of 2%-4% per year. The complex etiology of pediatric AVMs persists as an impediment to a comprehensive understanding of pathogenesis and subsequent targeted gene therapies. While AVMs secondary to vascular malformation syndromes have a clearer pathogenesis, a variety of gene mutations have been identified within sporadic AVM cases. The Ephrin B2/EphB4 (RASA-1, KRAS, and MEK) signaling axis, hemorrhagic telangiectasia, NOTCH, and TIE2 receptor complexes (PIK3CA and mTOR), in addition to other isolated gene variants, have been implicated in AVM pathogenesis. Furthering the understanding of the molecular mechanisms of AVM pathogenesis will lead to future novel therapies and treatment paradigms. Given the expected lifespan of a child, pediatric patients have an unacceptably high cumulative lifetime risk of hemorrhage. AVM treatment strategies are dependent on AVM grade, provider preference, and institutional resources. While open microsurgery is the mainstay of treatment for some AVMs, radiosurgery for definitive treatment and adjunctive endovascular embolization are also used extensively. There is increasing evidence indicating that all three modalities play important and potentially synergistic roles in the armamentarium for pediatric AVM treatment. This review serves to report current understanding in the genetic and molecular mechanisms of pediatric AVMs, review clinical diagnostic and classification criteria, and detail treatment options and subsequent outcomes of pediatric AVM patients.

Objective: Recently there has been an increase in pediatric neurosurgical fellowship graduates. It is important to understand the current pediatric neurosurgical workforce to help with prospective strategic workforce planning. The authors sought to determine 1) the geographic distribution and regional retention after training and 2) academic and leadership metrics by geographic location, era of training, and gender for practicing pediatric neurosurgeons in the United States.

Methods: Current practicing pediatric neurosurgeons were identified through American Board of Pediatric Neurological Surgery (ABPNS) certification status and the American Association of Neurological Surgeons directory. NIH RePORTER, Web of Science, and departmental and hospital networking websites were used to collect data on demographics, training, leadership, NIH involvement, and academic metrics.

Results: A total of 298 ABPNS-certified pediatric neurosurgeons were identified as currently practicing in the United States. Of these pediatric neurosurgeons, 26.2% were women, 74.5% were academic, and 11.7% have received current or past NIH funding. There were significant differences in the concentration of pediatric neurosurgeons per general population based on region. A total of 117 (39.3%) pediatric neurosurgeons held leadership positions; 4 (1.3%) served as neurosurgery department chairs, 67 (22.5%) served as chief of pediatric neurosurgery (9 of whom were women), 12 (4.0%) served as residency program directors, and 32 (10.7%) served as pediatric fellowship directors. Women were more likely to currently practice in the same region in which they trained for medical school (p = 0.050), have a lower academic rank (p = 0.004), and have a lower h-index (p < 0.001). Pediatric neurosurgeons practicing in the Northeast were more likely to have completed residency (p = 0.022) and medical school (p = 0.002) in the same region as their current practice.

Conclusions: There are differences in the concentration of pediatric neurosurgeons based on region. In pediatric neurosurgery, women hold fewer leadership positions, have lower academic ranks, and are less academically impactful as measured by the h-index. As the demand for pediatric neurosurgeons evolves, thoughtful monitoring of the distribution and composition of the neurosurgical workforce can help ensure equitable access to care across the country.

Objective: Determining the long-term success of endoscopic third ventriculostomy (ETV) remains challenging. This study aimed to investigate the impact of clinical and radiological factors on ETV success in pediatric patients with hydrocephalus.

Methods: The study included patients < 18 years old with hydrocephalus who underwent ETV between March 2014 and May 2021. Data including patient age, gender, history of previous shunt surgery, previous external ventricular drain placement, intraventricular hemorrhage history, and postoperative meningitis were extracted from medical records. Imaging features such as aqueductal stenosis, third ventricle floor bowing, displaced lamina terminalis, pulsatility index (PI), and maximum diameter of the cortical subarachnoid space (CSAS) were recorded for each patient using preoperative CT scans. Two independent neurosurgeons measured the CSAS maximum diameter and the PI. CSAS measurements were obtained on axial slices of the preoperative CT scans, whereas the PI was based on intraoperative third ventricle pulsatility. Patients were followed up for 1 year after surgery, with failure defined as the need for ventriculoperitoneal shunt (VPS) placement or death attributable to hydrocephalus.

Results: Ninety-eight children with a mean age of 16.39 ± 19.07 months underwent ETV for hydrocephalus. No deaths were recorded, but over 6 months and 1 year of follow-up, 12.2% and 22.4% of patients, respectively, experienced documented ETV failure requiring VPS placement. At the 6-month follow-up, a smaller maximum diameter of the CSAS was significantly associated with ETV failure; multivariate analysis revealed that CSAS maximum diameter was a predictor of 6-month ETV failure. At the 1-year follow-up, a lower PI was significantly associated with ETV failure, and multivariate analysis confirmed the PI as a significant predictor of ETV failure within 1 year after surgery. CSAS and PI measurements were repeated to assess interrater reliability: the intraclass correlation coefficients were 0.897 and 0.669 for CSAS and PI, respectively.

Conclusions: This study found that the CSAS maximum diameter and the PI are predictors of ETV failure at 6 months and 1 year, respectively. These findings highlight the importance of considering specific factors such as the CSAS and PI when assessing the likelihood of ETV success in pediatric patients with hydrocephalus. Further research and consideration of these factors may help optimize patient selection and improve outcomes for those undergoing ETV as a treatment for hydrocephalus.

Objective: The goal of this study was to survey the members of the American Society of Pediatric Neurosurgeons (ASPN) to assess the prevalence and associated risks of burnout among pediatric neurosurgeons. The authors aimed to identify the factors that most significantly contributed to this risk to provide a baseline group of characteristics to improve physician well-being.

Methods: Institutional Review Board approval from the University of Arizona was obtained, and the 7-question and 9-question Mayo Physician Well-Being Index (WBI) was distributed to members of the ASPN (n = 275). This index screens for many different aspects of distress for physicians, including burnout risk, stress, depression, fatigue, suicidal ideation, and low career satisfaction.

Results: An analysis of 111 pediatric neurosurgeons (111/275 [40% response rate]) was completed. Respondent ages were distributed, with those aged 56-60 years representing the highest proportion (20%). University practice represented a majority (72%). One-third (32%) of respondents reported practicing greater than 25 years, and most physicians in the survey were married (76%). One-third of surgeons spend 61-70 hours working per week (33%), and a plurality are on call between 6 and 10 days per month (42%). Most surgeons reported treating fewer than 200 cases per year (37% reported 100-150 cases; 23%, 151-200). Most pediatric neurosurgeons (63%) stated their annual salary was sufficient. Analysis of each WBI question was performed to identify which factors specifically contributed to the risk of burnout. An overwhelming majority of respondents reported that they make significant efforts to do at least one thing each week that brings them joy (97%), and they either agree or strongly agree that they perform meaningful work (98% of all participants, 97% of females, and 98% of men, p = 0.010). Nearly half of all respondents (49%) reported feelings of burnout and a majority of them were female (67% of women and 42% of men, p = 0.021). Time, environment, case volumes, and quality-of-life concerns are all factors that significantly contribute to the overall risk of burnout and well-being.

Conclusions: This survey study of the ASPN membership revealed a 49% rate of burnout with females at higher risk (67%). Factors associated with burnout were salary, more than 10 days on call per month, electronic medical record stressors, and work-life incongruity. The aforementioned notwithstanding, respondents believe pediatric neurosurgery is a meaningful career. This study provides evidence supporting a moral imperative toward recognition of burnout symptoms and a pivot point toward implementing change.

Objective: Hydrocephalus is one of the neurological risks occurring in patients with achondroplasia. Ventriculoperitoneal shunt (VPS) insertion is the most common treatment. However, reports of successful endoscopic third ventriculostomy (ETV) suggest that ETV may be a good alternative to VPS insertion in achondroplasia. However, it has been stated that ETV in achondroplasia patients is technically demanding to perform. The current study examined the anatomical variations of the third ventricle and the brainstem in achondroplasia patients and correlated the findings with the difficulty of performing ETV.

Methods: A retrospective analysis of 51 patients with achondroplasia and 138 hydrocephalus patients without achondroplasia (48 patients had tumor-related hydrocephalus and 90 patients had hydrocephalus of nontumorous origin) who have visited the authors' institution since 2012 was performed. Preoperative T2-weighted sagittal MR images were used to measure α (steepness of the third ventricle floor), β (endoscopic angle of incidence), d1 (vertical distance between the dorsum sellae and basilar bifurcation), and d2 (horizontal distance between the dorsum sellae and basilar artery). Each value was compared using the Tukey multicomparison test.

Results: Achondroplasia patients showed significantly smaller α (p < 0.001) and β (p < 0.001) angles, while there were no significant differences between the control groups (p = 0.947 for α, p = 0.836 for β). The d1 value was significantly larger in achondroplasia patients (p < 0.001), and d2 was smaller (p < 0.001). The control groups showed similar d1 and d2 values (p = 0.415 for d1, p = 0.154 for d2). Smaller α and β values meant that in achondroplasia patients the third ventricle floor stood more vertically than in other patients with hydrocephalus, and the endoscopic contact angles were small, increasing the risk of ventriculostomy devices slipping down into the infundibular recess. Additionally, a large d1 meant that the basilar artery was shifted upward and a small d2 indicated that the basilar artery was located closer to the dorsum sellae, potentially increasing the risk of basilar artery damage.

Conclusions: Achondroplasia patients' skull and brain anatomies were significantly different from those of other hydrocephalus patients, with steeper third ventricle floors and basilar arteries closer to the dorsum sellae. Because these anatomical differences lead to difficulties in performing ETVs in achondroplasia patients, such differences should be considered when ETV is planned for the patients.

Objective: Achondroplasia is associated with foramen magnum stenosis (FMS), which can lead to sudden unexpected death in infants. There is no wide consensus regarding the best management of FMS. This study aimed to analyze the prevalence of FMS in a cohort of children with achondroplasia and to evaluate screening and neurosurgical interventions of FMS regarding its effects and complications.

Methods: The authors conducted a retrospective cohort study including all children with achondroplasia assessed or treated at Karolinska University Hospital between September 2005 and June 2020. The severity of FMS was graded using the MRI Achondroplasia Foramen Magnum Score (AFMS). The AFMS was correlated with neurological examinations and polysomnography (PSG) results.

Results: Severe FMS (AFMS 3-4) was present in 35% of the 51 children included in the study. As many as 65% of the children in the cohort underwent foramen magnum decompression (FMD). Neurological examination had a high specificity (94%) but a low sensitivity (28%) for severe FMS. Signs of central apnea on PSG did not correlate with severity of FMS (p = 0.735). Surgery improved FMS (p < 0.001) with a nonsignificant trend of decreased central apnea (p = 0.070), but carried a 9% risk of severe surgery- and anesthesia-related complications.

Conclusions: This study confirmed previous reports that severe FMS is common in children with achondroplasia, that neurological symptoms may be absent even in severe FMS, and that FMD improves FMS and may improve central apnea. The finding that neurological examination had a low sensitivity for severe FMS supports the recommendation that all children with achondroplasia should undergo early MRI.