The impact of social determinants of health on utilization of pediatric epilepsy surgery: a systematic review.

OBJECTIVE: A minority of pediatric patients who may benefit from epilepsy surgery receive it. The reasons for this utilization gap are complex and not completely understood. Patient and caregiver social determinants of health (SDOH) may impact which patients undergo surgery and when. The authors conducted a systematic review examining SDOH and surgical intervention in children with drug-resistant epilepsy (DRE). They aimed to understand which factors influenced time to surgical program referral or receipt of epilepsy surgery among children with DRE, as well as identify areas to characterize the SDOH impacting epilepsy surgery in children and guide efforts aimed to promote health equity in epilepsy. METHODS: A systematic review was conducted using the PubMed, Embase, and Scopus databases in January 2022. Studies were analyzed by title and abstract, then full text, to identify all studies examining the impact of SDOH on utilization of epilepsy surgery. Studies meeting inclusion criteria were analyzed for SDOH examined, outcomes, and key findings. Quality was assessed using the Grading of Recommendations Assessment, Development and Evaluation system. RESULTS: Of 4545 resultant articles, 18 were included. Studies examined social, cultural, and environmental factors that contributed to SDOH impacting epilepsy surgery. Patients who underwent surgical evaluation were found to be most commonly White and privately insured and have college-educated caregivers. Five studies found differences in time to referral/surgery or rates of surgery by racial group, with most finding an increased time to referral/surgery or lower rates of surgery for those who were Hispanic and/or non-White. Four studies found that private insurance was associated with higher surgical utilization. Three studies found higher household income was related to surgical utilization. No studies examined biological, psychological, or behavioral factors that contributed to SDOH impacting epilepsy surgery. CONCLUSIONS: The authors conducted a systematic review exploring the impact of SDOH in DRE surgery utilization. They found that race, ethnicity, insurance type, caregiver educational attainment, and household income demonstrate relationships with pediatric epilepsy surgery. Further study is necessary to understand how these factors, and others not identified in this study, contribute to the low rates of utilization of epilepsy surgery and potential target areas for interventions aiming to increase equity in access to epilepsy surgery in children.

Unraveling the Complexity of the Senescence-Associated Secretory Phenotype in Adamantinomatous Craniopharyngioma Using Multi-Modal Machine Learning Analysis.

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 (SASP). 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.

Visual outcomes after treatment of craniopharyngioma in children: A systematic review.

OBJECTIVE: Craniopharyngiomas (CP) are rare brain tumors that often result in visual impairment due to their proximity to the optic pathway. The optimal management approach to preserve visual function in these patients remains controversial. We sought to investigate visual outcomes of children with craniopharyngiomas based on treatment modality. METHODS: A systematic review was performed according to PRISMA guidelines. PubMed, Embase, and Scopus databases were searched in December 2022 for relevant articles. Articles were screened by title/abstract for relevance, then by full-text. Relevant demographic, intervention, and outcome data were extracted from included studies. RESULTS: A total of 59 studies were included, representing 2655 patients. The overall visual status (OVS) of patients receiving surgery alone was improved in 27.6% of reported outcomes, unchanged in 50.3%, and deteriorated in 22.1%. The OVS for patients receiving radiation alone was improved in 21.1%, unchanged in 42.1%, and deteriorated in 36.8%. Patients receiving surgery plus adjuvant radiotherapy had OVS improvement in 27.4%, unchanged in 63.2%, and deteriorated in 9.4%. Of those receiving intracystic bleomycin, 23.1% had improvement in OVS, 46.2% remained unchanged, and 30.8% deteriorated. Of patients receiving interferon-α, 34.8% improved, 54.5% remained unchanged, and 10.6% deteriorated. CONCLUSION: OVS most frequently remained unchanged regardless of intervention. The greatest improvement in OVS was seen in those receiving interferon-α or surgery alone. The greatest OVS deterioration was noted with radiation alone. Future standardized, randomized, large-scale studies with focused assessment of ophthalmologic findings are key to further understanding the impact different interventions have on visual outcomes in these children.

Machine learning models for predicting seizure outcome after MR-guided laser interstitial thermal therapy in children.

OBJECTIVE: MR-guided laser interstitial thermal therapy (MRgLITT) is associated with lower seizure-free outcome but better safety profile compared to open surgery. However, the predictors of seizure freedom following MRgLITT remain uncertain. This study aimed to use machine learning to predict seizure-free outcome following MRgLITT and to identify important predictors of seizure freedom in children with drug-resistant epilepsy. METHODS: This multicenter study included children treated with MRgLITT for drug-resistant epilepsy at 13 epilepsy centers. The authors used clinical data, diagnostic investigations, and ablation features to predict seizure-free outcome at 1 year post-MRgLITT. Patients from 12 centers formed the training cohort, and patients in the remaining center formed the testing cohort. Five machine learning algorithms were developed on the training data by using 10-fold cross-validation, and model performance was measured on the testing cohort. The models were developed and tested on the complete feature set. Subsequently, 3 feature selection methods were used to identify important predictors. The authors then assessed performance of the parsimonious models based on these important variables. RESULTS: This study included 268 patients who underwent MRgLITT, of whom 44.4% had achieved seizure freedom at 1 year post-MRgLITT. A gradient-boosting machine algorithm using the complete feature set yielded the highest area under the curve (AUC) on the testing set (AUC 0.67 [95% CI 0.50-0.82], sensitivity 0.71 [95% CI 0.47-0.88], and specificity 0.66 [95% CI 0.50-0.81]). Logistic regression, random forest, support vector machine, and neural network yielded lower AUCs (0.58-0.63) compared to the gradient-boosting machine but the findings were not statistically significant (all p > 0.05). The 3 feature selection methods identified video-EEG concordance, lesion size, preoperative seizure frequency, and number of antiseizure medications as good prognostic features for predicting seizure freedom. The parsimonious models based on important features identified by univariate feature selection slightly improved model performance compared to the complete feature set. CONCLUSIONS: Understanding the predictors of seizure freedom after MRgLITT will assist with prognostication.

Patient family engagement and partnership: Pilot survey results in assessing behavior, communication, and quality of life in children with Lennox-Gastaut syndrome and other drug-resistant epilepsy.

OBJECTIVES: Lennox-Gastaut Syndrome (LGS) and other drug-resistant epilepsy (DRE) can impact behavior, communication, and quality of life (QoL). In collaboration with community engagement efforts with the Lennox-Gastaut Syndrome Foundation (LGSF), we aimed to gain an initial snapshot of patient and family perspectives and experiences with evaluation of behavior, communication, and QoL. METHODS: A cross-sectional survey was conducted to collect self-reported information from caregivers of children with LGS and other DRE regarding their perspectives and experiences with healthcare providers' evaluation of behavior, communication, and QoL. The survey tool was developed by the study investigators in partnership with the LGS Foundation and had diffused to caregivers online by epilepsy advocacy groups including the Pediatric Epilepsy Surgery Alliance (PESA). Responses were analyzed. Descriptive statistics were calculated. The survey asked for caregiver perspectives and assessed which instruments the caregivers had previously been given for measuring these domains. RESULTS: Responses from 245 caregivers were included, with 132 (54%) caregivers of an individual with LGS and 113 (46%) caregivers of an individual with non-LGS related DRE. Respondents reported that 66% of their loved ones had undergone epilepsy-related surgery. Over 90% agreed that measuring behavior, communication, and QoL was important, but fewer than half felt that their healthcare providers evaluated these domains well. LGS caregivers largely shared non-LGS caregivers' perspectives; however, they reported more frequently that communication was not evaluated enough. Barriers to measuring these domains included a lack of good surveys (developmentally appropriate and specific to the type of epilepsy) or not receiving any survey instruments for these domains during clinic appointments. Caregivers play a crucial role for individuals with DRE, and their input is essential in identifying challenges and needs. Caregivers believe that measuring behavior, communication, and quality of life is important, and most of them feel that their loved ones are not adequately evaluated during their healthcare encounters. There is a need for appropriately scaled survey instruments to measure areas of importance for patients and caregivers, as well as incorporation of these outcomes in the healthcare discussion.

Survival and neurological outcomes after stereotactic biopsy of diffuse intrinsic pontine glioma: a systematic review.

OBJECTIVE: Diffuse intrinsic pontine gliomas (DIPGs) are aggressive and malignant tumors of the brainstem. Stereotactic biopsy can obtain molecular and genetic information for diagnostic and potentially therapeutic purposes. However, there is no consensus on the safety of biopsy or effect on survival. The authors aimed to characterize neurological risk associated with and the effect of stereotactic biopsy on survival among patients with DIPGs. METHODS: A systematic review was performed in accordance with PRISMA guidelines to identify all studies examining pediatric patients with DIPG who underwent stereotactic biopsy. The search strategy was deployed in PubMed, Embase, and Scopus. The quality of studies was assessed using the Grading of Recommendations, Assessment, Development and Evaluation system, and risk of bias was evaluated with the Cochrane Risk of Bias in Nonrandomized Studies-of Interventions tool. Bibliographic, demographic, clinical, and outcome data were extracted from studies meeting inclusion criteria. RESULTS: Of 2634 resultant articles, 13 were included, representing 192 patients undergoing biopsy. The weighted mean age at diagnosis was 7.5 years (range 0.5-17 years). There was an overall neurosurgical complication rate of 13.02% (25/192). The most common neurosurgical complication was cranial nerve palsy (4.2%, 8/192), of which cranial nerve VII was the most common (37.5%, 3/8). The second most common complication was perioperative hemorrhage (3.6%, 7/192), followed by hemiparesis (2.1%, 4/192), speech disorders (1.6%, 3/192) such as dysarthria and dysphasia, and movement disorders (1.0%, 2/192). Hydrocephalus was less commonly reported (0.5%, 1/192), and there were no complications relating to wound infection/dehiscence (0%, 0/192) or CSF leak (0%, 0/192). No mortality was specifically attributed to biopsy. Diagnostic yield of biopsy revealed a weighted mean of 97.4% (range 91%-100%). Of the studies reporting survival data, 37.6% (32/85) of patients died within the study follow-up period (range 2 weeks-48 months). The mean overall survival in patients undergoing biopsy was 9.73 months (SD 0.68, median 10 months, range 6-13 months). CONCLUSIONS: Children with DIPGs undergoing biopsy have mild to moderate rates of neurosurgical complications and no excessive morbidity. With reasonably acceptable surgical risk and high diagnostic yield, stereotactic biopsy of DIPGs can allow for characterization of patient-specific molecular and genetic features that may influence prognosis and the development of future therapeutic strategies.

Rett Syndrome-Associated Scoliosis: Analysis of National Trends and Treatment Patterns of a Rare Indication for Posterior Instrumented Fusion.

STUDY DESIGN: Retrospective database cohort study. OBJECTIVE: To evaluate U.S. treatment trends and inpatient outcomes for children undergoing posterior spinal fusion (PSF) for Rett syndrome (RTT)-associated scoliosis (RAS). SUMMARY OF BACKGROUND DATA: RTT is a rare, sporadic neurodevelopmental disorder presenting in childhood with developmental regression, ataxia, and seizures. RAS occurs in 50% to 80% of cases of RTT, but little is known about the case volume and perioperative experience for children undergoing PSF. MATERIALS AND METHODS: Using the International Classification of Diseases Ninth and 10th revision codes in the national Kids' Inpatient Database, we identified children with RTT who underwent PSF from 2000 to 2019. Annual case volumes were analyzed. Clinical characteristics and outcomes were compared with those of a cohort of patients with neuromuscular scoliosis (NMS). RESULTS: Among 220 patients with RAS, 216 (98.2%) were females (mean age at surgery: 12.3±3.3 yr). Surgical case incidence steadily increased over 19 years, with more RAS admissions in the South (31.4%). Overall, patients with RAS demonstrated a higher mean Elixhauser Comorbidity Index score (2 vs . 1, P < 0.001) and had more perioperative complications (41.4% vs . 18%, P < 0.001) than patients with NMS. RTT diagnosis independently predicted higher odds of any complications (odds ratio: 1.98, P < 0.001) and increased length of stay (odds ratio: 1.18, P = 0.009) for admissions for PSF. CONCLUSIONS: Surgical treatment for RAS is rare but increased over a 19-year period. Cases appear to be clustering by region, with the highest proportion in the South. The higher Elixhauser Comorbidity Index in RAS patients predicted higher costs, longer hospital stays, more complications (particularly respiratory), and more nonroutine discharge disposition than in other patients with NMS. RTT was independently associated with higher odds of complications and longer length of stay. Because RAS cases appear to be increasing in number, future studies should emphasize methods to reduce morbidity and investigate deformity-specific metrics to help better understand this population.

Your Brain on Reddit: Exploring Neurosurgical Concerns on a Popular Social Media Site.

OBJECTIVE: Social media has irreversibly impacted global interpersonal connectivity. This study aims to understand how information discussed in condition-specific communities, called "subreddits," on the major social media platform Reddit, can improve education by medical providers. METHODS: The top 50 Reddit posts in each subreddit's "hot" tab were retroactively screened from June 1, 2022. Posts written by those 18 and older who had the condition or were engaging in a better understanding of the condition were included in the analysis. In addition, posts were placed in 1 of 6 categories based on the redditors' primary questions: social advice, health advice, providing health education or suggesting equipment, personal experience/progress, researchers recruiting patients for research, or others. RESULTS: We identified 605 posts within 15 subreddits related to cranial neurosurgical topics including epilepsy, traumatic brain injury, stroke, brain tumors/cancer, seizures, hydrocephalus, cerebrospinal fluid leaks, Chiari malformations, aneurysms, pineal cysts, ataxia, and moyamoya. Asking for health advice was the most common form of information seeking across subreddits (53.6%), specifically treatment-related questions (41.0%). Redditors from r/epilepsy and r/seizures constituted 84.6% of nonsurgical treatment questions relating to antiepileptic dosing or side effects. CONCLUSIONS: Cranial neurosurgical disease can overwhelm patients, leaving them with unaddressed questions. Reddit provides anonymity and camaraderie within condition-specific communities not found at the doctor's office. The ease of access to an Internet community may encourage redditors to seek health information online.

Post-Surgical Cognitive Outcomes of Moyamoya Disease: A Systematic Review.

BACKGROUND: Moyamoya disease (MMD) is an occlusive arteriopathy leading to stroke. Progressive if left untreated, revascularization surgery has become the mainstay of treatment. Although clinical and radiographic outcomes of MMD after intervention are well-characterized, cognitive outcomes in pediatric patients remain unclear. We aimed to examine postoperative cognitive outcomes in children with MMD, examine factors associated with cognitive changes after intervention, and define the effect of revascularization surgery on cognitive outcomes. METHODS: A systematic review was conducted following PRISMA guidelines searching PubMed, Embase, and Scopus databases. Articles met inclusion criteria if they studied pediatric patients undergoing revascularization surgery for MMD and examined cognitive outcomes either qualitatively or quantitatively. All data extracted from included articles was examined descriptively. RESULTS: Of 1091 resultant articles, 12 articles containing 446 patients were included. Surgery was associated with maintained or improved full scale intellectual quotient (IQ), performance IQ, perceptual IQ, memory quotient and verbal memory. However, 70% of patients had impaired cognitive function, with associated poor school performance. Improvements in cognition were associated with increased cerebral blood flow, particularly to the middle cerebral artery, due to the development of collaterals. Female sex, shorter duration from symptom onset to surgery, and surgery after age 7 were predictive of cognitive improvement. Completed ischemic stroke prior to surgery was associated with poorer cognitive outcomes. CONCLUSIONS: Although children with MMD have improved cognitive outcomes following revascularization overall, a distinct subset experience cognitive impairment. Consideration of patient-specific and treatment-related factors is important to enable proper risk stratification and inform management approaches.

Comparison of long-term survival with continued medical therapy, vagus nerve stimulation, and cranial epilepsy surgery in paediatric patients with drug-resistant epilepsy in the USA: an observational cohort study.

BACKGROUND: Long-term survival in paediatric epilepsy is incompletely characterised. A better understanding of treatment effects on mortality in paediatric patients with drug-resistant epilepsy is needed for health-care decision making. We aimed to compare the long-term survival rates associated with antiseizure medications only, antiseizure medications plus vagus nerve stimulation (VNS), and antiseizure medications plus cranial epilepsy surgery in paediatric patients with drug-resistant epilepsy using a large national administrative database in the USA. METHODS: In this observational cohort study, patients aged 0-17 years who were diagnosed with drug-resistant epilepsy using International Classificiaton of Diseases codes between Jan 1, 2004, and Dec 31, 2020, were identified from the Pediatric Health Information System, an administrative database that contains inpatient, emergency department, ambulatory, and observation unit encounter-level data from more than 49 children's hospitals in the USA. Patients treated with at least three types of antiseizure medications were included in the medical therapy cohort, those treated with antiseizure medications plus VNS were included in the VNS cohort, and those treated with antiseizure medications plus cranial epilepsy surgery were included in the surgery cohort. Participants were followed up until the date of their last clinical encounter, in-hospital death, or Dec 31, 2020. Inverse probability of treatment weighting (IPTW) was used to balance baseline demographics and clinical characteristics between treatment groups. The unconditional probabilities of survival were estimated by weighted Kaplan-Meier analysis. A weighted Cox proportional hazards model was used to investigate the association between risk of overall death and age, sex, geographical region, race and ethnicity, comorbidity, primary diagnosis, insurance, and treatment. FINDINGS: This study included 10 240 patients treated with antiseizure medications only, 5019 patients treated with antiseizure medications plus VNS, and 3033 patients treated with antiseizure medications plus cranial epilepsy surgery. The median age of paediatric patients was 7 years (IQR 4-12) in the medical therapy cohort, 9 years (6-13) in the VNS cohort, and 9 years (5-13) in the surgery cohort. The IPTW-adjusted probabilities of surviving beyond 10 years were 89·27% (95% CI 87·71-90·85) for the medical therapy cohort, 92·65% (90·62-94·72) for the VNS cohort, and 98·45% (97·53-99·38) for the surgery cohort. The difference in survival probabilities was significant (log-rank p<0·0001). Compared with the medical therapy cohort, the IPTW-adjusted hazard ratio for overall death was 0·60 (95% CI 0·50-0·74) for the VNS cohort and 0·19 (0·10-0·33) for the surgery cohort. INTERPRETATION: Paediatric patients with drug-resistant epilepsy who underwent cranial epilepsy surgery or VNS had a higher survival rate than those who received only medical treatment. These findings highlight the importance of a multidisciplinary comprehensive team approach to the treatment of epilepsy, which includes tailored evaluation and deployment of medical and surgical treatment options for patients with this challenging disease. FUNDING: None.

Cronobacter brain abscess and refractory epilepsy in a newborn: role of epilepsy surgery. Illustrative case.

BACKGROUND: Neonatal meningitis due to Cronobacter is associated with powdered infant formula. Prompt recognition of this rare but aggressive infection is critical. OBSERVATIONS: The authors report a unique case of neonatal Cronobacter meningoencephalitis complicated by brain abscess and status epilepticus, requiring surgical intervention in a preterm 4-week-old male and related to contaminated powdered infant formula. They discuss the medical and surgical management in this patient, as well as the role of epilepsy surgery in acute drug-resistant epilepsy. This is paired with a literature review examining Cronobacter infections in infants to provide a summative review of the existing literature. LESSONS: Cronobacter contamination in powdered infant formula and breast pumps is rare but can cause life-threatening infections. When evaluating patients with Cronobacter central nervous system infections, serial neuroimaging, infection control, and prompt surgical management are essential. Future studies are needed regarding the role of epilepsy surgery in the acute infectious period.

Endoscopic third ventriculostomy and biopsy of a tectal lesion using flexible neuroendoscopy and urological cup forceps: illustrative case.

BACKGROUND: Tectal region tumors often clinically present as obstructive hydrocephalus due to mass effect on the outflow of the third ventricle and cerebral aqueduct. Pathology in this region varies; thus, biopsy can be of great value in the management decision making. Appropriate instrumentation remains an area of interest to further advance flexible neuroendoscopic techniques and applications. OBSERVATIONS: The authors report an illustrative case using flexible neuroendoscopy through a single burr hole for simultaneous endoscopic third ventriculostomy (ETV) and tectal tumor biopsy using urological cup forceps in a 13-year-old boy who had presented with obstructive hydrocephalus. LESSONS: The authors demonstrate the feasibility of simultaneous ETV and tectal lesion biopsy via flexible neuroendoscopy to address obstructive hydrocephalus and obtain a tissue biopsy in a single-site procedure. They found that the use of flexible cup forceps designed for uroscopy is an important complement to flexible neuroendoscopy. Given the evolving applications of flexible neuroendoscopy, this has implications for instrumentation adaptation and future design.

Thoracolumbar Scoliosis in Pediatric Patients With Loeys-Dietz Syndrome: A Case Series.

Background Loeys-Dietz syndrome (LDS) is a genetic connective tissue disorder that predominantly affects cardiovascular, skeletal, and craniofacial structures. Associated thoracolumbar scoliosis in LDS can be challenging to manage, though other etiologies of pediatric scoliosis have better-defined management guidelines. We examined our institutional experience regarding the treatment of pediatric patients with LDS and scoliosis. Methodology In this retrospective study, all patients seen at our pediatric tertiary care center from 2004 through 2018 with a diagnosis of LDS were reviewed, and those with radiographic diagnoses of scoliosis (full-length scoliosis X-rays) were included. Demographic, clinical, and radiographic parameters were collected, and management strategies were reported. Results A total of 39 LDS patients whose ages ranged between seven and 13 years were identified. A total of nine patients were radiographically diagnosed with scoliosis, but three patients were excluded due to incomplete medical records, leaving six patients. The median age at scoliosis diagnosis was 11.5 years, with a median follow-up of 51 months. Two patients were successfully managed with observation (average initial Cobb angle (CA): 14°, average final CA: 20.5°). Two were braced, one successfully (initial CA: 15°, final CA: 30°) and one with a progressive disease requiring surgery (initial CA: 40°, final CA: 58°). Of the two who were offered surgical correction, one underwent surgery with a durable correction of spinal deformity (CA: 33° to 19°). One patient underwent a recent correction of aortic root dilatation and was not a candidate for scoliosis surgery. Conclusions Principles of adolescent idiopathic scoliosis management such as bracing for CA of 20-50° and surgery for CA of >50° can be applied to LDS patients with good outcomes. This augments our understanding of the treatment algorithm for pediatric patients with LDS.

The past, present and future of ACS NSQIP-Pediatric: Evolution from a quality registry to a comparative quality performance platform.

Quality and process improvement (QI/PI) in children's surgical care require reliable data across the care continuum. Since 2012, the American College of Surgeons' (ACS) National Surgical Quality Improvement Program-Pediatric (NSQIP-Pediatric) has supported QI/PI by providing participating hospitals with risk-adjusted, comparative data regarding postoperative outcomes for multiple surgical specialties. To advance this goal over the past decade, iterative changes have been introduced to case inclusion and data collection, analysis and reporting. New datasets for specific procedures, such as appendectomy, spinal fusion for scoliosis, vesicoureteral reflux procedures, and tracheostomy in children less than 2 years old, have incorporated additional risk factors and outcomes to enhance the clinical relevance of data, and resource utilization to consider healthcare value. Recently, process measures for urgent surgical diagnoses and surgical antibiotic prophylaxis variables have been developed to promote timely and appropriate care. While a mature program, NSQIP-Pediatric remains dynamic and responsive to meet the needs of the surgical community. Future directions include introduction of variables and analyses to address patient-centered care and healthcare equity.

The impact of voluntary folate fortification of corn masa flour on US pregnancies complicated by neural tube defects.

INTRODUCTION: In 1996, the US Food and Drug Administration (FDA) mandated folic acid fortification for all enriched cereal grains. This resulted in a reduction of neural tube defect (NTD)-affected pregnancies. However, Hispanic women continued to be twice as likely to give birth to a child affected by NTD compared to non-Hispanic White women. Some hypotheses explaining this difference focus on cultural variation in dietary intake of cereal grains. In 2016, the FDA approved voluntary folic acid fortification for corn masa flour products to focus on the Hispanic diet staple. This study investigates rates of NTDs in predominantly Hispanic-populated zip codes before and after the voluntary fortification of corn masa flour with folic acid. METHODS: Normal pregnancies and those complicated by NTDs between 1/1/2016 and 9/30/2020 were identified using ICD-9 and ICD-10 codes in an all-payor claims database. The post-fortification period began 12 months after the fortification recommendation. The US Census data was used to stratify pregnancies in predominantly Hispanic zip codes (≥ 75% of households) vs. non-Hispanic zip codes. The causal impact of the FDA's recommendation was assessed by means of a Bayesian structural time series model. RESULTS: A total of 2,584,366 pregnancies were identified among females aged 15-50 years. Of these, 365,983 took place in predominantly Hispanic zip codes. Mean quarterly NTDs per 100,000 pregnancies did not significantly differ between predominantly Hispanic zip codes and predominantly non-Hispanic zip codes pre-FDA recommendation (184.5 vs. 175.6; p = 0.427), nor post-recommendation (188.2 vs. 185.9; p = 0.713). Rates of NTDs predicted to occur if no FDA recommendation had been made were compared to the actual rate post-recommendation: no significant difference was observed in predominantly Hispanic zip codes (p = 0.245) or overall (p = 0.116). CONCLUSIONS: Rates of neural tube defects were not significantly reduced in predominantly Hispanic zip codes following the 2016 FDA approval of voluntary folic acid fortification of corn masa flour. Further research and implementation of comprehensive approaches to advocacy, policy, and public health are necessary to decrease preventable congenital disease rates. Mandatory rather than voluntary fortification of corn masa flour products may achieve more substantial prevention of neural tube defects in at-risk US populations.

Disability or Death: A Focused Review of Informed Consent in Pediatric Neurosurgery.

The management of pediatric neurosurgical disease often requires families to choose between long-term disability and premature death. This decision-making is codified by informed consent. In practice, decision-making is heavily weighted toward intervening to prevent death, often with less consideration of the realities of long-term disability. We analyze long-term disability in pediatric neurosurgical disease from the perspectives of patients, families, and society. We then present a pragmatic framework and conversational approach for addressing informed consent discussions when the outcome is expected to be death or disability. We performed a focused review of literature regarding informed consent in pediatric neurosurgery by searching PubMed and Google Scholar with search terms including "pediatric neurosurgery," "informed consent," and "disability." The literature was focused on patients with diagnoses including spina bifida, neuro-oncology, trauma, and hydrocephalus. Patient perspective elements were physical/mental disability, lack of autonomy, and role in community/society. The family perspective involves caregiver burden, emotional toll, and financial impact. Societal considerations include the availability of public resources for disabled children, large-scale financial cost, and impacts on global health. Practical conversational steps with patients/caregivers include opening the discussion, information provision and acknowledgement of uncertainty, assessment of understanding and clarifying questions, decision-making, and decision maintenance, all while remaining sensitive to the emotional burden commensurate with these decisions. The "death or disability" paradigm represents a common challenge to informed consent in pediatric neurosurgery. Patient, family, and societal factors that inform surrogate decisions vary and sometimes conflict. Pediatric neurosurgeons must use a comprehensive approach to address the informational and relational needs of caregivers during the informed consent process.

Morning glory disc anomaly and its implications in moyamoya arteriopathy: a retrospective case series.

OBJECTIVE: Morning glory disc anomaly (MGDA), a congenital abnormality of the optic nerve, may be associated with moyamoya arteriopathy, a cerebrovascular abnormality. In this study, the authors aimed to define the temporal evolution of cerebrovascular arteriopathy in patients with MGDA to characterize a rational strategy for screening and management over time. METHODS: The records of pediatric neurosurgical patients at two academic institutions were retrospectively reviewed to identify cases of cerebral arteriopathy and MGDA, including radiographic and clinical records documenting patient outcomes of medical and surgical management. RESULTS: Thirteen cases of moyamoya syndrome (MMS) associated with MGDA were identified in 13 children aged 0.6-17 years. The pattern of arteriopathy resembled that of non-MGDA MMS, with predominantly anterior circulation involvement. The arteriopathy lateralized with the MGDA, although 3 patients also had contralateral involvement. The overall group was followed for a median of 3.2 years. Radiological biomarkers of cerebral ischemia were applied to guide surgical decisions, and more than half of the patients (7 of 13) had evidence of stroke or progression on serial imaging. Nine patients underwent revascularization surgery, and 4 were managed medically. CONCLUSIONS: Cerebral arteriopathy observed in association with MGDA resembles MMS seen in patients without MGDA and is dynamic, with progression observed over months to years and an associated risk of cerebral ischemia that indicates a role for surgical revascularization. Radiological biomarkers may augment clinical data to identify candidates for revascularization surgery.

Disparities in pediatric drug-resistant epilepsy care.

INTRODUCTION: Epilepsy affects millions of children worldwide, with 20-40% experiencing drug-resistant epilepsy (DRE) who are recommended for epilepsy surgery evaluation and may benefit from surgical management. However, many patients live with DRE for multiple years prior to surgical epilepsy referral or treatment or are never referred at all. OBJECTIVE: We aimed to describe factors associated with referral for epilepsy surgery in the USA, in order to identify disparities in DRE, characterize why they may exist, and recognize areas for improvement. METHODS: Pediatric patients diagnosed with DRE between January 1, 2004 and December 31, 2020 were identified from the Pediatric Health Information System (PHIS) Database. Patients treated with antiseizure medications (ASMs) only, ASMs plus vagus nerve stimulation (VNS), and ASMs plus cranial epilepsy surgery were studied regarding access to epilepsy surgery and disparities in care. This study used chi-square tests to determine associations between treatment time and preoperative factors. Preoperative factors studied included epilepsy treatment type, age, sex, race/ethnicity, insurance type, geographic region, patient type, epilepsy type, and presence of pediatric complex chronic conditions (PCCCs). RESULTS: A total of 18,292 patients were identified; 10,240 treated with ASMs, 5019 treated with ASMs + VNS, and 3033 treated with ASMs + cranial epilepsy surgery. Sex was not found to significantly vary among groups. There was significant variation in age, census region, race/ethnicity, patient type, presence of PCCCs, diagnosis, and insurance (p < 0.001). Those treated surgically, either with VNS or cranial epilepsy surgery, were 2 years older than those medically treated. Additionally, those medically treated were less likely to be living in the Midwest (25.46%), identified as non-Hispanic white (51.78%), have a focal/partial epilepsy diagnosis (8.74%), and be privately insured (35.82%). CONCLUSIONS: We studied a large administrative US database examining variables associated with surgical epilepsy evaluation and management. We found significant variation in treatment associated with age, US census region, race/ethnicity, patient type, presence of PCCCs, diagnosis, and health insurance type. We believe that these disparities in care are related to access and social determinants of health, and we encourage focused outreach strategies to mitigate these disparities to broaden access and improve outcomes in children in the USA with DRE.

Flexible neuroendoscopy for endoscopic third ventriculostomy and fourth ventricular arachnoid cyst fenestration in an infant.

Arachnoid cysts of the fourth ventricle are rarely reported. Management options include CSF diversion, cyst fenestration, or cyst excision. Fenestration can be done via open microsurgical technique or endoscopically with or without simultaneous third ventriculostomy; and both rigid and flexible endoscopy have been used successfully. However, application of this treatment modality in pediatric patients is not well described. Therefore, to their knowledge, the authors report the first successful treatment of a fourth ventricular arachnoid cyst with a single frontal burr hole entry point for third ventriculostomy and fourth ventricular arachnoid cyst fenestration performed using flexible neuroendoscopy. The patient was a 13-month-old boy presenting with progressive macrocephaly. The authors review their technique, discuss special considerations when using this approach, and include an annotated intraoperative video for demonstration to help instruct and guide management. The authors demonstrate with an example that a single frontal burr hole entry point for flexible endoscopic third ventriculostomy and navigation through a dilated cerebral aqueduct for fourth ventricular arachnoid cyst fenestration is a viable treatment for symptomatic fourth ventricular arachnoid cysts in children.