Endoluminal Biopsy for Vein of Galen Malformation.

BACKGROUND AND OBJECTIVES: Vein of Galen malformation (VOGM), the result of arteriovenous shunting between choroidal and/or subependymal arteries and the embryologic prosencephalic vein, is among the most severe cerebrovascular disorders of childhood. We hypothesized that in situ analysis of the VOGM lesion using endoluminal tissue sampling (ETS) is feasible and may advance our understanding of VOGM genetics, pathogenesis, and maintenance. METHODS: We collected germline DNA (cheek swab) from patients and their families for genetic analysis. In situ VOGM "endothelial" cells (ECs), defined as CD31+ and CD45-, were obtained from coils through ETS during routine endovascular treatment. Autologous peripheral femoral ECs were also collected from the access sheath. Single-cell RNA sequencing of both VOGM and peripheral ECs was performed to demonstrate feasibility to define the transcriptional architecture. Comparison was also made with a published normative cerebrovascular transcriptome atlas. A subset of VOGM ECs was reserved for future DNA sequencing to assess for somatic and second-hit mutations. RESULTS: Our cohort contains 6 patients who underwent 10 ETS procedures from arterial and/or venous access during routine VOGM treatment (aged 12 days to ∼6 years). No periprocedural complications attributable to ETS occurred. Six unique coil types were used. ETS captured 98 ± 88 (mean ± SD; range 17-256) experimental ECs (CD31+ and CD45-). There was no discernible correlation between cell yield and coil type or route of access. Single-cell RNA sequencing demonstrated hierarchical clustering and unique cell populations within the VOGM EC compartment compared with peripheral EC controls when annotated using a publicly available cerebrovascular cell atlas. CONCLUSION: ETS may supplement investigations aimed at development of a molecular-genetic taxonomic classification scheme for VOGM. Moreover, results may eventually inform the selection of personalized pharmacologic or genetic therapies for VOGM and cerebrovascular disorders more broadly.

Endovascular balloon usage in endoscopic third ventriculostomy for hydrocephalus during a national shortage: case series and technical note.

Endoscopic third ventriculostomy (ETV) is a well-established surgical technique for treating hydrocephalus. Many providers have transitioned to utilizing the specialized Neuroballoon for the stoma dilation in ETV; however, these devices are intermittently unavailable during supply chain shortages. We present the experience of employing cardiac angioplasty and neurovascular balloons as substitutes for the Neuroballoon in 3 patients. The scepter balloon (Microvention), priced at $1800 compared to the standard $300 Neuroballoon (Integra), proved effective, but its pliability presented technical challenges. The substantial cost differential compared to a Neuroballoon ($300) raises economic considerations. The Cardiac TREK balloon (Abbott) was similarly effective, while also being easier to manage endoscopically and cheaper at $158. These experiences support the viability of non-neuroendoscopic specialized balloons as alternatives for ETV dilation of the floor of tuber cinereum.

Neurosurgical Management of Myelomeningocele in Premature Infants: A Case Series.

Introduction: Myelomeningocele (MMC) is the most common neural tube defect, but rarely seen in premature infants. Most centers advocate for closure of MMC within 24 hours of birth. However, this is not always possible in severely premature infants. Given the rarity of this patient population, we aimed to share our institutional experience and outcomes of severely premature infants with MMC. Methods: We performed a retrospective, observational review of premature infants (≤ 32 weeks gestational age) identified through our multidisciplinary spina bifida clinic (1995-2021) and surgical logs. Descriptive statistics were compiled about this sample including timing of MMC closure and incidence of adverse events such as sepsis, CSF diversion, meningitis, and death. Results: Eight patients were identified (50% male) with MMC who were born ≤ 32 weeks gestational age. Mean gestational age of the population was 27.3 weeks (SD 3.5). Median time to MMC closure was 1.5 days (IQR = 1 -80.8). Five patients were taken for surgery within the recommended 48 hours of birth; 2 patients underwent significantly delayed closure (107 and 139 days); and one patient's defect epithelized without surgical intervention. Six of eight patients required permanent cerebrospinal fluid (CSF) diversion (2 patients were treated with ventriculoperitoneal shunting (VPS), three were treated with endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) and 1 patient treated with ETV; mean of 3 years after birth, ranging from 1 day to 16 years). Two patients required more than one permanent CSF diversion procedure. Two patients developed sepsis (defined as meeting at least 2/4 SIRS criteria), and 2 patients had intraventricular hemorrhage (both grade III). No patients developed meningitis (defined as positive CSF cultures) prior to MMC closure. Median follow up duration was 9.7 years. During this time epoch, 3 patients died: Two before 2 years of age of causes unrelated to surgical intervention. One of the two patients with grade III IVH died within 24 hours of MMC closure. Conclusions: In our institutional experience with premature infants with MMC, some patients underwent delayed MMC closure. The overall rate of meningitis, sepsis, and mortality for preterm children with MMC was similar to MMC patients born at term.

Pediatric non-galenic pial arteriovenous fistula's characteristics and outcomes: a systematic review.

INTRODUCTION: Pediatric non-galenic pial arteriovenous fistulas (pAVFs) are rare vascular malformations that are characterized by a pial arterial-venous connection without an intervening capillary bed. Outcomes and treatment strategies for pAVFs are highly individualized, owing to the rarity of the disease and lack of large-scale data guiding optimal treatment approaches. METHODS: We performed a systematic review of pediatric patients (< 18 years at diagnosis) diagnosed with a pAVF by digital subtraction angiogram (DSA). The demographics, treatment modalities, and outcomes were documented for each patient and clinical outcome data was collected. Descriptive information stratified by outcome scores were classified as follows: 1 = excellent (no deficit and full premorbid activity), 2 = good (mild deficit and full premorbid activity), 3 = fair (moderate deficit and impaired activity), 4 = poor (severe deficit and dependent on others), 5 = death. RESULTS: A total of 87 studies involving 231 patients were identified. Median age at diagnosis was 3 years (neonates to 18 years). There was slight male preponderance (55.4%), and 150 subjects (81.1%*) experienced excellent outcomes after treatment. Of the 189 patients treated using endovascular approaches, 80.3% experienced excellent outcomes and of the 15 patients surgically treated subjects 75% had an excellent outcome. The highest rate of excellent outcomes was achieved in patients treated with Onyx (95.2%) and other forms of EvOH (100%). High output heart failure and comorbid vascular lesions tended to result in worse outcomes, with only 54.2% and 68% of subjects experiencing an excellent outcome, respectively. *Outcomes were reported in only 185 patients. CONCLUSION: pAVFs are rare lesions, necessitating aggregation of patient data to inform natural history and optimal treatment strategies. This review summarizes the current literature on pAVF in children, where children presenting with heart failure as a result of high flow through the lesion were less likely to experience an excellent outcome. Prospective, large-scale studies would further characterize pediatric pAVFs and enable quantitative analysis of outcomes to inform best treatment practices.

The genetic basis of hydrocephalus: genes, pathways, mechanisms, and global impact.

Hydrocephalus (HC) is a heterogenous disease characterized by alterations in cerebrospinal fluid (CSF) dynamics that may cause increased intracranial pressure. HC is a component of a wide array of genetic syndromes as well as a secondary consequence of brain injury (intraventricular hemorrhage (IVH), infection, etc.) that can present across the age spectrum, highlighting the phenotypic heterogeneity of the disease. Surgical treatments include ventricular shunting and endoscopic third ventriculostomy with or without choroid plexus cauterization, both of which are prone to failure, and no effective pharmacologic treatments for HC have been developed. Thus, there is an urgent need to understand the genetic architecture and molecular pathogenesis of HC. Without this knowledge, the development of preventive, diagnostic, and therapeutic measures is impeded. However, the genetics of HC is extraordinarily complex, based on studies of varying size, scope, and rigor. This review serves to provide a comprehensive overview of genes, pathways, mechanisms, and global impact of genetics contributing to all etiologies of HC in humans.

Applicant Perceptions of Postinterview Communication During the 2022 to 2023 Neurosurgery Recruitment Cycle: A Cross-Sectional Survey Study.

OBJECTIVES: To evaluate and describe neurosurgery applicant perceptions of the postinterview communication (PIC) process during the US residency match. METHODS: A voluntary and anonymous postmatch web-based survey was developed and sent to 209 candidates who applied to 1 academic neurosurgery practice during the 2022-2023 recruitment cycle, approximately 1 week following match day. Survey questions focused on their perceptions of and participation behaviors with PIC and how this impacted their final rank list. RESULTS: Seventy-eight (37.3%) of the 209 candidates responded to the survey. Sixty-four (84.2%) respondents reported submitting a letter of intent (LOI) to their number 1 ranked program. Sixty-one (82%) felt pressured to send a LOI to improve their rank status, fearing that it may harm them if they did not. Fifty-four (73.0%) respondents felt pressured to send an early LOI despite not seeing the program in person to communicate interest before programs certified their rank lists. Fourteen (18.9%) respondents agreed that a second look experience impacted their rank list enough to where they regretted an early LOI. Fifty-five (76.4%) respondents disagreed that second-look attendance had no impact on their rank status with a program. Fifty (71.4%) respondents agreed that PIC causes undue stress during the match process. Sixty-one (84.7%) respondents agreed that aspects of PIC require universal guidelines. CONCLUSIONS: This is the first study to describe the perceptions of PIC and behaviors of neurosurgery applicants during the US residency match process. Standardized PIC practices may help to ensure transparency and relieve stress for applicants during the match process.

Characteristics and outcomes of pediatric dural arteriovenous fistulas: a systematic review.

BACKGROUND: Dural arteriovenous fistulas (dAVF) are arteriovenous shunts in communication with the dural vasculature in the brain or spine. Apart from single-center series, risk factors and treatment outcomes for pediatric dAVFs are largely undescribed. METHODS: We performed a systematic literature review of pediatric (< 18 years at diagnosis) intracranial and spinal dAVF according to PRISMA guidelines. We queried PubMed, CINAHL, SCOPUS, and Embase databases without time/date restriction. Search strings included a variety of MeSH keywords relating to dural AV fistulas in combination with MeSH keywords related to pediatric cases (see Appendix). Manuscripts describing patients diagnosed with dural sinus malformations or pial AVF were excluded. RESULTS: We identified 61 studies describing 69 individual patients. Overall, dAVF were more common in males (55.1%) with a mean age of diagnosis (5.17 ± 4.42 years). Approximately 20.2% of patients presented with cardiovascular disease (CVD), and 31.9% were discovered incidentally on neuroimaging studies. Transverse-sigmoid junction was the most common location (17.3%). Ninety-three percent (64 patients) were treated, most commonly using endovascular embolization (68.1%) followed by surgery (8.7%) and radiosurgery (2.9%). Almost half (43.8%) of dAVFs were completely obliterated. Of the 64 procedures, there were 19 neurological complications (29.7%) of varying severity where 12.5% were considered transient (i.e., pseudomeningocele) and 17.2% permanent (i.e., mortality secondary to acute sinus thrombosis, etc.). CONCLUSION: There is a paucity of information on pediatric dAVFs. This systematic review summarizes the published cases of dAVFs in the pediatric population. While the rate of missing data is high, there is publication bias, and precise details regarding complications are difficult to ascertain, this review serves as a descriptive summary of pediatric dAVFs.

Does subtotal resection ameliorate hypothalamic morbidity in pediatric craniopharyngioma? A 30-year retrospective cohort study.

OBJECTIVE: The optimal extent of resection of craniopharyngiomas to minimize the long-term risks of hypothalamic and endocrine dysfunction (obesity and panhypopituitarism) in children remains uncertain. The purpose of this study was to report long-term outcomes of pediatric patients with craniopharyngioma undergoing surgical treatment and to study rates of endocrinological and hypothalamic dysfunction in association with extent of resection. METHODS: This retrospective study was performed in a cohort of children who underwent resection for craniopharyngioma at Children's of Alabama between 1990 and 2020. The primary outcome was hypothalamic dysfunction defined as a 0.5 increase in body mass index (BMI) Z-score and as a BMI > 2 SDs with or without psychiatric disturbances. Univariable analysis was performed using ANOVA, Wilcoxon rank-sum test, Pearson's chi-square test, and Fisher's exact test as appropriate. Missing data on the primary outcome were handled via multiple imputations. Relative risks were estimated using a multivariable generalized linear model with a priori variables selected using a modified Poisson regression approach with robust error variance to estimate risk ratios. RESULTS: The cohort includes 39 patients (24 girls and 15 boys; age range 1 month-16 years) who underwent resection of craniopharyngioma at the authors' center between 1990 and 2020. The preoperative goal of treatment was cyst decompression (CD) in 5, subtotal resection (STR) in 13, and gross-total resection (GTR) in 21 patients. The median long-term follow-up after surgery was 8.11 years (average 8.21, range 0.4-24.33 years). Univariate analysis demonstrated a statistically significant increase in hypothalamic dysfunction in patients undergoing GTR when compared to those undergoing STR or CD at 1 month postoperatively (p = 0.006) and 6-11 months postoperatively (p = 0.010), but with this difference not persisting beyond 1 year. Multivariable analysis showed patients older than 10 years at time of surgery to be the most affected and at highest risk of developing significant hypothalamic dysfunction. There was no significant difference in pituitary or neurological function between the STR/CD and GTR groups at 12-24 months or at most recent follow-up. There was no significant difference in BMI Z-scores between the STR/CD and GTR groups at 6-12 months or at most recent follow-up. CONCLUSIONS: Both STR and GTR of craniopharyngioma were associated with significant endocrinological sequelae after 1 year. These potential complications should be discussed with patients and their families, and postoperative protocols should include early nutritional and endocrinological interventions with endocrinologist consultation.

Hemispherectomy Outcome Prediction Scale: a validity study.

OBJECTIVE: Hemispherectomy is highly effective for patients with medically refractory epilepsy (MRE) arising from a single hemisphere. Recently, the Hemispherectomy Outcome Prediction Scale (HOPS) was developed as a prediction tool for seizure freedom after hemispherectomy. The authors' goal was to perform a validation study to determine the generalizability of the HOPS score. METHODS: The authors present an observational, retrospective, 20-year, single-institution, two-surgeon experience using the lateral peri-insular hemispherectomy approach to validate the HOPS score. Variables used to derive the HOPS score included seizure onset age, semiology, PET hypometabolism, seizure substrate, and history of prior epilepsy resection. Multivariable logistic regression, multiple imputation, and Bayesian analyses were used to determine validity. RESULTS: The authors' cohort comprised 60 patients; 55% of patients were male and 78% were Caucasian. The median age at first hemispherectomy surgery was 72 months. At 1 year postoperatively, 80% of patients had Engel class I outcomes, analogous to most contemporary series. All patients who experienced seizure recurrence after hemispherectomy did so within the first 2 years postoperatively. Sixteen (27%) and 10 (17%) patients had contralateral MRI findings and hypometabolism on PET, respectively. Both a multivariable logistic regression model using HOPS score variables (model p = 0.2588) and a revised model that included contralateral MRI findings (model p = 0.4715) were not statistically significant in this cohort. Bayesian analysis also did not validate the HOPS score. CONCLUSIONS: While seizure outcome prediction tools may be helpful for counseling patients about postoperative outcomes, rigorous validity and reliability testing are required. Prospective, standardized, and longitudinal evaluation of patients undergoing hemispherectomy are needed.

What has changed in pediatric neurosurgical care in spina bifida? A 30-year UAB/Children's of Alabama observational overview.

Spina bifida (SB) remains the most serious and most common congenital anomaly of the human nervous system that is compatible with life. The open myelomeningocele on the back is perhaps the most obvious initial problem, but the collective impact of dysraphism upon the entirety of the nervous system and innervated organs is an equal or greater longitudinal threat. As such, patients with myelomeningocele (MMC) are best managed in a multi-disciplinary clinic that brings together experienced medical, nursing, and therapy teams that provide high standards of care while studying outcomes and sharing insights and experiences. Since its inception 30 years ago, the spina bifida program at UAB/Children's of Alabama has remained dedicated to providing exemplary multi-disciplinary care for affected children and their families. During this time, there has been great change in the care landscape, but many of the neurosurgical principles and primary issues have remained the same. In utero myelomeningocele closure (IUMC) has revolutionized initial care and has favorable impact on several important co-morbidities of SB including hydrocephalus, the Chiari II malformation, and the functional level of the neurologic deficit. Hydrocephalus however is not solved by IUMC, and hydrocephalus management remains at the center of neurosurgical care in SB. Ventricular shunts were long the cornerstone of treatment for hydrocephalus, but we came to assess and incorporate endoscopic third ventriculostomy with choroid plexus coagulation (ETV-CPC). Educated and nurtured by an experienced senior mentor, we dedicated ourselves to fundamental concepts but persistently evaluated our care outcomes and evolved our protocols and paradigms for improvement. Active conversations amidst networks of treasured colleagues were central to this development and growth. While hydrocephalus support and treatment of tethered spinal cord remained our principal neurosurgical charges, we evolved to embrace a holistic perspective and approach that is reflected and captured in the Lifetime Care Plan. Our team engaged actively in important workshops and guideline initiatives and was central to the development and support of the National Spina Bifida Patient Registry. We started and developed an adult SB clinic to support our patients who aged out of pediatric care. Lessons there taught us the importance of a model of transition that emphasized personal responsibility and awareness of health and the crucial role of dedicated support over time. Support for sleep, bowel health, and personal intimate cares are important contributors to overall health and care. This paper details our growth, learning, and evolution of care provision over the past 30 years.

Building consensus for the medical management of children with moderate and severe acute spinal cord injury: a modified Delphi study.

OBJECTIVE: The focus of this modified Delphi study was to investigate and build consensus regarding the medical management of children with moderate and severe acute spinal cord injury (SCI) during their initial inpatient hospitalization. This impetus for the study was based on the AANS/CNS guidelines for pediatric SCI published in 2013, which indicated that there was no consensus provided in the literature describing the medical management of pediatric patients with SCIs. METHODS: An international, multidisciplinary group of 19 physicians, including pediatric neurosurgeons, orthopedic surgeons, and intensivists, were asked to participate. The authors chose to include both complete and incomplete injuries with traumatic as well as iatrogenic etiologies (e.g., spinal deformity surgery, spinal traction, intradural spinal surgery, etc.) due to the overall low incidence of pediatric SCI, potentially similar pathophysiology, and scarce literature exploring whether different etiologies of SCI should be managed differently. An initial survey of current practices was administered, and based on the responses, a follow-up survey of potential consensus statements was distributed. Consensus was defined as ≥ 80% of participants reaching agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree). A final meeting was held virtually to generate final consensus statements. RESULTS: Following the final Delphi round, 35 statements reached consensus after modification and consolidation of previous statements. Statements were categorized into the following eight sections: inpatient care unit, spinal immobilization, pharmacological management, cardiopulmonary management, venous thromboembolism prophylaxis, genitourinary management, gastrointestinal/nutritional management, and pressure ulcer prophylaxis. All participants stated that they would be willing or somewhat willing to change their practices based on consensus guidelines. CONCLUSIONS: General management strategies were similar for both iatrogenic (e.g., spinal deformity, traction, etc.) and traumatic SCIs. Steroids were recommended only for injury after intradural surgery, not after acute traumatic or iatrogenic extradural surgery. Consensus was reached that mean arterial pressure ranges are preferred for blood pressure targets following SCI, with goals between 80 and 90 mm Hg for children at least 6 years of age. Further multicenter study of steroid use following acute neuromonitoring changes was recommended.

Understanding and identifying the needs of parent caregivers of children with hydrocephalus: a qualitative study.

OBJECTIVE: Hydrocephalus is inherently unpredictable. Most parents whose child is diagnosed with hydrocephalus do not anticipate the diagnosis, nor can anyone predict if or when a child's shunt will fail and require emergency surgery. Previous research has shown that children with hydrocephalus and their caregivers experience significant posttraumatic stress symptoms secondary to the diagnosis. This study aims to understand caregiver experiences and needs, identify gaps in resources/support, and determine opportunities to improve care. METHODS: Semistructured interviews were conducted with parent caregivers of children with hydrocephalus to learn about their experiences with the hydrocephalus diagnosis, hospitalizations, surgeries, coping and support, challenges of caring for a child with hydrocephalus, and logistics for a proposed support program. De-identified interviews were audio-recorded, transcribed, and analyzed for themes. RESULTS: Thematic saturation was reached after 17 interviews. Five major themes emerged: 1) coping with the diagnosis, 2) received support, 3) hydrocephalus management, 4) implications for intervention, and 5) psychosocial stressors for caregivers. A top priority was balanced, trustworthy information delivered with compassion and updated throughout the child's life. Caregivers described a variety of coping strategies, but a majority reported a need for support in processing complex emotions and dealing with the uncertainty of their child's hydrocephalus. Most agreed that having a caregiver support network, medical professionals available for referrals and questions, and referrals to support services and therapies would facilitate feeling supported and providing the best care for their children. CONCLUSIONS: Parent caregivers are critical to the health and well-being of children with hydrocephalus, and it is essential to understand their experiences to improve care. Providing well-defined information, psychosocial support, and resources will help to equip parent caregivers to be advocates for their children and to improve both the caregiver and the child's quality of life.

Outcomes of endovascular embolization for Vein of Galen malformations: An individual participant data meta-analysis.

Introduction: Understanding outcomes after Vein of Galen malformation (VOGM) embolization has been limited by small sample size in reported series and predominantly single center studies. To address these limitations, we perform an individual-participant meta-analysis (IPMA) to identify risk factors associated with all-cause mortality and clinical outcome after VOGM endovascular embolization. Methods: We performed a systematic review and IPMA of VOGM endovascular outcomes according to PRISMA guidelines. Individual patient characteristics including demographic, intra/post-operative adverse events, treatment efficacy (partial or complete occlusion), and clinical outcome were collected. Mixed-effects logistic regression with random effects modeling and Bonferroni correction was used (p ≤ 0.003 threshold for statistical significance). The primary and secondary outcomes were all-cause mortality and poor clinical outcome (moderate/severe developmental delay or permanent disabling injury), respectively. Data are expressed as (mean ± standard deviation (SD)) or (odds ratio (OR), 95% confidence interval (CI), I 2, p-value). Results: Thirty-five studies totaling 307 participants quantifying outcomes after endovascular embolization for VOGM were included. Follow up time was 42 (±57) months. Our analysis contained 42% neonates (<1 month) at first embolization, 45% infants (1 month ≤2 years), and 13% children (>2 years). Complete occlusion was reported in 48% of participants. Overall all-cause mortality was 16%. Overall, good clinical outcome was achieved in 68% of participants. First embolization as a neonate [OR = 6.93; 95% CI (1.99-24.08); I 2 < 0.01; p < 0.001] and incomplete embolization [OR = 10.87; 95% CI (1.86-63.55); I 2 < 0.01; p < 0.001] were associated with mortality. First embolization as a neonate [OR = 3.24; 95% CI (1.47-7.15); I 2 < 0.01; p < 0.001], incomplete embolization [OR = 5.26; 95% CI (2.06-13.43); I 2 < 0.01; p < 0.001], and heart failure at presentation [OR = 3.10; 95% CI (1.03-9.33); I 2 < 0.01; p = 0.002] were associated with poor clinical outcomes. Sex, angioarchitecture of lesion, embolization approach (transvenous vs. transarterial), and single or multistage embolization were not associated with mortality or clinical outcome. Conclusions: We identify incomplete VOGM embolization independently associated with mortality and poor clinical outcome. While this study provides the highest level of evidence for VOGM embolization to date, prospective multicenter studies are needed to understand the optimal treatment strategies, outcomes, and natural history after VOGM embolization.

Quantifying and reporting outcome measures in pediatric epilepsy surgery: A systematic review.

Several instruments and outcomes measures have been reported in pediatric patients undergoing epilepsy surgery. The objective of this systematic review is to summarize, evaluate, and quantify outcome metrics for the surgical treatment of pediatric epilepsy that address seizure frequency, neuropsychological, and health-related quality of life (HRQL). We performed a systematic review according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines to identify publications between 2010 and June 2021 from PubMed, Embase, and the Cochrane Database of Systematic Reviews that report clinical outcomes in pediatric epilepsy surgery. Eighty-one articles were included for review. Overall, rates of postoperative seizure frequency were the most common metric reported (n = 78 studies, 96%). Among the seizure frequency metrics, the Engel Epilepsy Surgery Outcome Scale (n = 48 studies, 59%) was most commonly reported. Neuropsychological outcomes, performed in 32 studies (40%) were assessed using 36 different named metrics. HRQL outcomes were performed in 16 studies (20%) using 13 different metrics. Forty-six studies (57%) reported postoperative changes in antiepileptic drug (AED) regimen, and time-to-event analysis was performed in 15 (19%) studies. Only 13 outcomes metrics (1/5 seizure frequency, 6/13 HRQL, 6/36 neuropsychological) have been validated for use in pediatric patients with epilepsy and only 13 have been assessed through reliability studies (4/5 seizure frequency, 6/13 HRQL, and 3/36 neuropsychological). Of the 81 included studies, 17 (21%) used at least one validated metric. Outcome variable metrics in pediatric epilepsy surgery are highly variable. Although nearly all studies report seizure frequency, there is considerable variation in reporting. HRQL and neuropsychological outcomes are less frequently and much more heterogeneously reported. Reliable and validated outcomes metrics should be used to increase standardization and accuracy of reporting outcomes in pediatric patients undergoing epilepsy surgery.

Endoscopic third ventriculostomy in previously shunt-treated patients.

OBJECTIVE: Endoscopic third ventriculostomy (ETV) is an option for treatment of hydrocephalus, including for patients who have a history of previous treatment with CSF shunt insertion. The purpose of this study was to report the success of postshunt ETV by using data from a multicenter prospective registry. METHODS: Prospectively collected data in the Hydrocephalus Clinical Research Network (HCRN) Core Data Project (i.e., HCRN Registry) were reviewed. Children who underwent ETV between 2008 and 2019 and had a history of previous treatment with a CSF shunt were included. A Kaplan-Meier survival curve was created for the primary outcome: time from postshunt ETV to subsequent CSF shunt placement or revision. Univariable Cox proportional hazards models were created to evaluate for an association between clinical and demographic variables and subsequent shunt surgery. Postshunt ETV complications were also identified and categorized. RESULTS: A total of 203 children were included: 57% male and 43% female; 74% White, 23% Black, and 4% other race. The most common hydrocephalus etiologies were postintraventricular hemorrhage secondary to prematurity (56, 28%) and aqueductal stenosis (42, 21%). The ETV Success Score ranged from 10 to 80. The median patient age was 4.1 years. The overall success of postshunt ETV at 6 months was 41%. Only the surgeon's report of a clear view of the basilar artery was associated with a lower likelihood of postshunt ETV failure (HR 0.43, 95% CI 0.23-0.82, p = 0.009). None of the following variables were associated with postshunt ETV success: age at the time of postshunt ETV, etiology of hydrocephalus, sex, race, ventricle size, number of previous shunt operations, ETV performed at time of shunt infection, and use of external ventricular drainage. Overall, complications were reported in 22% of patients, with CSF leak (8.6%) being the most common complication. CONCLUSIONS: Postshunt ETV was successful in treating hydrocephalus, without subsequent need for a CSF shunt, in 41% of patients, with a clear view of the basilar artery being the only variable significantly associated with success. Complications occurred in 22% of patients. ETV is an option for treatment of hydrocephalus in children who have previously undergone shunt placement, but with a lower than expected likelihood of success.

Expedited epilepsy surgery prior to drug resistance in children: a frontier worth crossing?

Epilepsy surgery is an established safe and effective treatment for selected candidates with drug-resistant epilepsy. In this opinion piece, we outline the clinical and experimental evidence for selectively considering epilepsy surgery prior to drug resistance. Our rationale for expedited surgery is based on the observations that (i) a high proportion of patients with lesional epilepsies (e.g. focal cortical dysplasia, epilepsy-associated tumours) will progress to drug resistance; (ii) surgical treatment of these lesions, especially in non-eloquent areas of brain, is safe; and (iii) earlier surgery may be associated with better seizure outcomes. Potential benefits beyond seizure reduction or elimination include less exposure to antiseizure medications, which may lead to improved developmental trajectories in children and optimize long-term neurocognitive outcomes and quality of life. Further, there exists emerging experimental evidence that brain network dysfunction exists at the onset of epilepsy, where continuing dysfunctional activity could exacerbate network perturbations. This in turn could lead to expanded seizure foci and contribution to the comorbidities associated with epilepsy. Taken together, we rationalize that epilepsy surgery, in carefully selected cases, may be considered prior to drug resistance. Last, we outline the path forward, including the challenges associated with developing the evidence base and implementing this paradigm into clinical care.

Hydrocephalus surveillance following CSF diversion: a modified Delphi study.

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.

Endoscopic third ventriculostomy revision after failure of initial endoscopic third ventriculostomy and choroid plexus cauterization.

OBJECTIVE: Primary treatment of hydrocephalus with endoscopic third ventriculostomy (ETV) and choroid plexus cauterization (CPC) is well described in the neurosurgical literature, with wide reported ranges of success and complication rates. The purpose of this study was to describe the safety and efficacy of ETV revision after initial ETV+CPC failure. METHODS: Prospectively collected data in the Hydrocephalus Clinical Research Network Core Data Project registry were reviewed. Children who underwent ETV+CPC as the initial treatment for hydrocephalus between 2013 and 2019 and in whom the initial ETV+CPC was completed (i.e., not abandoned) were included. Log-rank survival analysis (the primary analysis) was used to compare time to failure (defined as any other surgical treatment for hydrocephalus or death related to hydrocephalus) of initial ETV+CPC versus that of ETV revision by using random-effects modeling to account for the inclusion of patients in both the initial and revision groups. Secondary analysis compared ETV revision to shunt placement after failure of initial ETV+CPC by using the log-rank test, as well as shunt failure after ETV+CPC to that after ETV revision. Cox regression analysis was used to identify predictors of failure among children treated with ETV revision. RESULTS: The authors identified 521 ETV+CPC procedures that met their inclusion criteria. Ninety-one children underwent ETV revision after ETV+CPC failure. ETV revision had a lower 1-year success rate than initial ETV+CPC (29.5% vs 45%, p < 0.001). ETV revision after initial ETV+CPC failure had a lower success rate than shunting (29.5% vs 77.8%, p < 0.001). Shunt survival after initial ETV+CPC failure was not significantly different from shunt survival after ETV revision failure (p = 0.963). Complication rates were similar for all examined surgical procedures (initial ETV+CPC, ETV revision, ventriculoperitoneal shunt [VPS] placement after ETV+CPC, and VPS placement after ETV revision). Only young age was predictive of ETV revision failure (p = 0.02). CONCLUSIONS: ETV revision had a significantly lower 1-year success rate than initial ETV+CPC and VPS placement after ETV+CPC. Complication rates were similar for all studied procedures. Younger age, but not time since initial ETV+CPC, was a risk factor for ETV revision failure.

The Hydrocephalus Clinical Research Network quality improvement initiative: the role of antibiotic-impregnated catheters and vancomycin wound irrigation.

OBJECTIVE: Two previous Hydrocephalus Clinical Research Network (HCRN) studies have demonstrated that compliance with a standardized CSF shunt infection protocol reduces shunt infections. In this third iteration, a simplified protocol consisting of 5 steps was implemented. This analysis provides an updated evaluation of protocol compliance and evaluates modifiable shunt infection risk factors. METHODS: The new simplified protocol was implemented at HCRN centers on November 1, 2016, for all shunt procedures, excluding external ventricular drains, ventricular reservoirs, and subgaleal shunts. Procedures performed through December 31, 2019, were included (38 months). Compliance with the protocol, use of antibiotic-impregnated catheters (AICs), and other variables of interest were collected at the index operation. Outcome events for a minimum of 6 months postoperatively were recorded. The definition of infection was unchanged from the authors' previous report. RESULTS: A total of 4913 procedures were performed at 13 HCRN centers. The overall infection rate was 5.1%. Surgeons were compliant with all 5 steps of the protocol in 79.4% of procedures. The infection rate for the protocol alone was 8.1% and dropped to 4.9% when AICs were added. Multivariate analysis identified having ≥ 2 complex chronic conditions (odds ratio [OR] 1.76, 95% confidence interval [CI] 1.26-2.44, p = 0.01) and a history of prior shunt surgery within 12 weeks (OR 1.84, 95% CI 1.37-2.47, p < 0.01) as independent risk factors for shunt infection. The use of AICs (OR 0.70, 95% CI 0.50-0.97, p = 0.05) and vancomycin irrigation (OR 0.36, 95% CI 0.21-0.62, p < 0.01) were identified as independent factors protective against shunt infection. CONCLUSIONS: The authors report the third iteration of their quality improvement protocol to reduce the risk of shunt infection. Compliance with the protocol was high. These updated data suggest that the incorporation of AICs is an important, modifiable infection prevention measure. Vancomycin irrigation was also identified as a protective factor but requires further study to better understand its role in preventing shunt infection.