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Mohini Gunnett,1 Brandon G Rocque,2 Anis Nourani,1 Guillermo Beltran-Ale1 1Department of Pediatrics, Division of Pulmonary and Sleep Medicine, University of Alabama at Birmingham (UAB), Birmingham, AL, USA; 2Department of Neurosurgery, Division of Pediatric Neurosurgery, University of Alabama at Birmingham (UAB), Birmingham, AL, USACorrespondence: Guillermo Beltran-Ale, University of Alabama at Birmingham, Department of Pediatrics, Division of Pulmonary and Sleep Medicine, 620 Jarman F. Lowder Building, 1600 7th Avenue South, Birmingham, AL, 35233-1711, USA, Tel +1 205 638 9593, Fax +1 205 638 2850, Email gjbeltranale@uabmc.eduAbstract: Spina bifida (SB) is one of the most common birth defects in children. The care for patients with SB continues to evolve, and there has been notable improvement in survival outcomes, degree of disability and quality of life for these children. However, patients with SB continue to remain at higher risk for sleep-related breathing disorders (SRBD), unexplained sudden death, and potential alterations in their sleep chronotype. Previous studies report on abnormalities in the spinal cord, brainstem function, and dysfunction of upper airway maintenance as the likely mechanisms behind SRBD that is commonly seen in SB. Most studies looking at prevalence of SRBD in SB have been retrospective studies. A recent prospective study identified a prevalence as high as 42% when a polysomnography (PSG) was completed on all patients regardless of symptomatology. Treatment options vary depending on the type and severity of SRBD and can range widely. Despite advances in care for patients with SB and SRBD, a subset of these patients with myelomeningocele (MMC) continue to experience sudden unexplained death. Studies continue to evaluate ways to stratify which of these patients may be at higher risk of this devastating outcome. Given that SRBD is potentially treatable, early assessment and intervention could become an integral part of a multidisciplinary treatment strategy to optimize long-term medical and neurodevelopmental outcomes for this patient population. By understanding the impact that SB may have on a patient’s sleep quality, their biological chronotype and their potential of developing SRBD, a provider may help to optimize the care a patient with SB receives from birth into adulthood.Keywords: sleep apnea, sleep-disordered breathing, myelomeningocele, central sleep apnea, hydrocephalus, Chiari 2 malformation

Objective: Studies have shown a high prevalence of sleep-disordered breathing (SDB) among children with myelomeningocele (MMC), but there are few published data on the longitudinal care of these patients. The objective of this study was to determine the effectiveness of standard treatments for SDB in children with MMC., Methods: The authors analyzed records from three multidisciplinary spina bifida clinics to identify all patients with both MMC and SDB diagnosed by polysomnography (PSG). The primary outcome of this study was a change in apnea-hypopnea index (AHI; the number of apneic or hypopneic events per hour of sleep) before and after clinically recommended SDB treatments. Clinical and demographic variables were recorded and evaluated for possible association with posttreatment improvement of AHI. Analysis included change in AHI (a continuous variable) and whether SDB improved (defined as an AHI < 2.5 or decrease of AHI by ≥ 50% from baseline)., Results: Seventy-one eligible patients (aged 2 days-21 years, 52% male) had an initial AHI > 2.5 and had follow-up PSG after treatment for SDB. The mean AHI decreased from 20.5 (SD 21.6) at baseline to 11.6 (SD 15.7) after treatment (p = 0.0006). Children treated with supplemental oxygen and with continuous positive airway pressure had improvement on PSG (18 of 25 and 12 of 18, respectively). Children treated with adenotonsillectomy were less likely to improve (7 of 19). Forty-one patients (58%) improved from a baseline AHI > 2.5 to an AHI < 2.5 after treatment., Conclusions: Children with MMC and SDB who undergo standard SDB treatments guided by pediatric sleep medicine physicians show improvement in PSG parameters after treatment.

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 h 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 h 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). In both cases of sepsis, patients developed signs and symptoms more than 72 h after birth. Notably, both instances of sepsis occurred unrelated to operative intervention as they occurred before permanent MMC closure. Two 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 h 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., (© 2024. The Author(s).)

Objective: Because there is not a link between COVID-19 and pediatric hydrocephalus, the COVID-19 pandemic should not have altered the incidence of pediatric hydrocephalus or the rate of CSF diversion procedures or shunt failure. Therefore, hydrocephalus-related surgical volume should have remained constant. The goal of this study was to evaluate the rates of hydrocephalus surgeries in the COVID-19 era compared with the baseline pre-COVID-19 era., Methods: Ten institutions collected information about all hydrocephalus-related surgeries performed between March 2018 and February 2022. The period after March 1, 2020, was considered the COVID-19 era; the period prior to this date was considered the baseline pre-COVID era. Four COVID surge periods were defined using the New York Times COVID-19 database. Total case volumes were compared between the COVID era and baseline, both overall and for each surge period. Sex, race, ethnicity, insurance status, Area Deprivation Index (ADI), and rural-urban commuter area were collected for each surgery. Proportions of patients were then compared overall and for each surge based on these variables., Results: Of 8056 procedures, 54% were in male patients (n = 4375), 65% in White patients (n = 5247), 18% in Hispanic patients (n = 1423), and 54% in patients with public insurance (n = 4371). There was no change in the number of surgeries per site per month in the COVID era compared with baseline (16.7 vs 17.9, p = 0.113). However, there was a significant decrease in the first surge period (April 2020; 11.5 vs 17.7, p = 0.034). Male sex (p < 0.0039) and Black race (p < 0.001) were found to be associated with a significantly higher proportion of hydrocephalus procedures during the COVID-19 era. Some surge periods showed different proportions of privately insured patient and ADI levels. However, these relationships were inconsistent between surges., Conclusions: Overall average monthly case numbers were not significantly different between the pre-COVID and COVID eras. There was a significant decrease in hydrocephalus surgery during the first COVID surge. More hydrocephalus surgeries were performed in children of male sex and Black race proportionally during the COVID period overall, but not during individual surges.

Objective: Myelomeningocele (MMC) is a lifelong condition requiring complex multidisciplinary management. Using the National Spina Bifida Patient Registry (NSBPR), the authors tested the association between sociodemographic variables and odds of undergoing neurosurgical procedures., Methods: The authors extracted sociodemographic, clinical, and neurosurgical procedure data on participants with MMC aged ≥ 1 year who visited an NSBPR clinic between 2009 and 2020. The zip code of the participant's residence at the time of the last spina bifida clinic visit was linked to the Distressed Communities Index (DCI) tier. Multivariate models were built to identify factors associated with undergoing CSF diversion, shunt revision, tethered cord release (TCR), and Chiari decompression., Results: There were 7924 participants with a median visit age of 13 years (IQR 7-20 years); 49.1% were male, 30.2% were non-Hispanic Black or Hispanic, 54.5% had public/supplemental insurance, and 16.9% were from distressed communities. CSF diversion, shunt revision, TCR, and Chiari decompression were performed in 81.8%, 47.7%, 22.9%, and 8.7% of participants, respectively. In multivariate analyses controlling for age, sex, insurance, DCI tier, lesion level, and surgical closure timing, Hispanic individuals were less likely than their non-Hispanic White counterparts to undergo shunt revision (p = 0.013). Non-Hispanic Black and Hispanic individuals were less likely to undergo TCR (p < 0.001 each) or Chiari decompression (p < 0.001 each). Compared with privately insured individuals, publicly insured individuals were more likely to undergo CSF diversion (p = 0.031). Those in distressed communities had increased odds of undergoing CSF diversion (p = 0.004) than those in prosperous communities., Conclusions: Among individuals with MMC participating in the NSBPR, there were differences in receiving neurosurgical procedures by race/ethnicity, insurance type, and DCI tier. Additional prospective studies are necessary to elucidate the reasons for these variations and their impact on long-term outcomes for this patient population in order to created targeted interventions.

Health care disparities between high-income countries (HICs) and low- and middle-income countries (LMICs) are well established. The focus of the surgical aspect of health was identified in the early twenty-first century, and efforts to provide safe surgical intervention require the shift of resources from HICs to LMICs with specialized surgeons, anesthesiologists, and equipment. This intervention may make a difference on the short run; however, to achieve a long-term self-sustaining surgical service in the region of need, education and training of local physicians is key., Competing Interests: Disclosure The authors have no disclosures., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Introduction: A subset of children with Chiari 1 malformation (CM-1) have a 4th ventricle arachnoid veil-a thin membrane covering the outlet of the 4th ventricle. Studies suggest that failure to disrupt this veil during posterior fossa decompression can reduce the likelihood of syringomyelia resolution. However, there is no reliable method for predicting the presence of the veil without direct surgical exploration. This study aims to evaluate the association between pre-operative symptoms, radiographic measurements, and the arachnoid veil., Methods: A retrospective review of an institutional database of children evaluated for CM-I was conducted. For patients treated with surgery, operative notes were reviewed to determine if an arachnoid veil was present. Logistic regression was used to test for relationship of clinical variables and radiographic measurements with the presence of an arachnoid veil., Results: Out of 997 children with CM-1, 226 surgical patients were included in the analysis after excluding those with inadequate documentation. An arachnoid veil was found in 23 patients (10.2%). Larger syrinx, spinal canal, and thecal sac diameters were significantly associated with the presence of a veil, with odds ratios of 1.23 (95% CI 1.2-1.48; p = 0.03), 1.27 (95% CI 1.02-1.59; p = 0.03), and 1.35 (95% CI 1.03-1.77; p = 0.03), respectively. No significant associations were found with any signs or symptoms., Conclusions: Arachnoid veil was present in 10% of cases. Radiographic measurements indicating larger syrinx size were the only variables found to be significantly associated with an arachnoid veil. Exploration of the 4th ventricular outlet is recommended for CM-I decompression in the setting of expansile syringomyelia., (© 2024. The Author(s).)

Objective: The purpose of this report is to describe a case series of children undergoing selective dorsal rhizotomy (SDR) for the treatment of spastic cerebral palsy in Vietnam. Also described is an international cooperation model to facilitate the development of a new, multidisciplinary team for the evaluation and treatment of these children., Methods: Details of international collaboration are described, including in-person travel and virtual interactions. All cases of children younger than 18 years undergoing SDR for treatment of spastic cerebral palsy at a single center in Hanoi, Vietnam are described, including preoperative evaluation of spasticity and gait as well as results at 6 and 12 months. Results are summarized using descriptive statistics., Results: Since the beginning of cooperation in training and transferring SDR techniques by experts from the United States, in the period from June 2016 to December 2022, 18 SDR surgeries were performed in Hanoi. Patients' ages ranged from 2 to 14 years; 13 were male and 5 were female. Overall, approximately 60% of nerve rootlets were cut. Modified Ashworth Scale scores at 6 and 12 months after surgery in the hip, knee, and ankle joints showed improvement from preoperative values. There were 2 recorded complications: intracranial hypotension causing subdural hemorrhage and a case of skin infection at the incision site., Conclusions: The ongoing international cooperation between Vietnamese and American physicians has helped improve the surgical treatment of spasticity in children with cerebral palsy in Hanoi, providing children with a surgical treatment option with successful outcomes., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objective: Interdisciplinary care and pediatric to adult transitional programs have consistently shown medical and social value for individuals with complex medical conditions such as spina bifida (SB). Such interdisciplinary clinics are common in pediatrics but are rarely offered for adults. This survey-based study reports information related to transition, daily pain burden, and satisfaction with care delivery in an adult SB clinic., Methods: A 23-question survey that was based on empirical observations from the adult SB clinic was formulated, IRB approved, and distributed to adult patients. Many respondents had previously received care at the institution's pediatric SB clinic and completed transition to the adult program. Responses were de-identified, categorized, stored in a secure database, and statistically analyzed using SPSS., Results: Of 245 patients approached, 116 (47%) surveys were completed and analyzed. Those who had a direct transition (defined as a less than 24-month gap in care) from the pediatric to the adult clinic comprised 44% (n = 51) of responders. The alternative group of 56% (n = 65) had a longer gap, disorganized or absent transition, or had pediatric care elsewhere. The study population had an average age of 36 years, had mostly received childhood care at the authors' institution, regardless of whether they made a direct transition or had a gap in care (68%), and held the diagnosis of open myelomeningocele (78%). Overall satisfaction with the clinic experience was high (mean score 9.04 on a 10-point subjective scale). Differences regarding independence in activities of daily living based on transition status were not significant, but on multivariate analysis, those who reported independence in activities of daily living had an almost 4-fold higher odds of daily pain (p = 0.024; OR 3.86, 95% CI 1.19-12.5). The most frequently identified areas for improvement included improved access to care and pain control., Conclusions: Pediatric transitional processes and interdisciplinary clinics may contribute to improved patient-perceived outcomes and satisfaction with their SB care in comprehensive settings. Further elucidation of barriers to pain control is warranted, in addition to ways in which comprehensive and longitudinal care can improve them.

Objective: The transition from pediatric to adult care is challenging for patients and families with spina bifida (SB). Lifelong care relationships yield to new care environments that are typically larger, less personal, and less engaged with the nuances of SB care. Adolescence and young adulthood are often characterized by personal and psychological stresses due to factors independent of illness or chronic medical complexity. Surveys have demonstrated that transition is associated with uncertainty, anxiety, and elevated risk of adverse events for many SB patients. To help mitigate this, the authors developed a trial mentorship program between teen patients with SB and undergraduate/medical students. This study analyzes and presents the initial outcomes from this program., Methods: The authors created the Join, Unite, Motivate, and Prepare (JUMP) program to improve readiness for the transition process. The mentee target population was patients aged 13-19 years receiving care at the authors' SB clinic. Mentors were screened/approved undergraduate/medical students who volunteered to participate and successfully completed online training in mentorship. Upon enrollment, each patient set a combination of clinical, self, and parent/guardian goals using the individualized transition plan. These goals were shared with the mentor, mentee, parent/guardian, and physician. To monitor success, the SB program director routinely met with each mentor to discuss progress made and areas of growth. These included continuous quantitative and qualitative goal setting and failures that needed to be addressed for each agenda., Results: Thirteen mentor-mentee matches were created over 9 months. Of the 13 matches, 6 had more than 5 communications after the initial meeting, and 1 mentor-mentee match is still in contact today. Noted success in the program has been through mentees gaining employment, applying for scholarships, starting college, and connecting with others who are going through similar circumstances. Challenges have arisen through failure to follow-up after the initial office visit, risk with using the virtual platform, and wide geographic dispersion of both mentors and mentees across the authors' state., Conclusions: Transition from pediatric to adult care for adolescents with SB has proven to be a large hurdle. Easing this process through well-thought-out, interactive processes has the potential to improve readiness, increase patient autonomy, and provide exposure to the adult healthcare community. However, the mentorship model, in the SB setting, has not proven to be the remedy.

Introduction: A cross-sectional study retrospectively evaluating the perceived usefulness of attending a multi-disciplinary, roundtable, educational prenatal clinic for mothers expecting children with myelomeningocele is presented., Methods: Mothers who currently have children with SB completed a survey which evaluated their overall preparedness, spina bifida education, delivery plans, surgical expectations, and expectations in terms of quality of life and development. Open comments were also collected. Statistical analysis was performed to identify differences between those who attended prenatal counseling and those who did not., Results: Approximately half of these mothers received some form of prenatal SB counseling. Mothers who attended prenatal counseling reported that they felt more informed and prepared throughout their pregnancy, during the delivery of their child and during their initial hospital stay than mothers who did not. They reported that the roundtable discussions were beneficial, and the education they received was useful in helping them form accurate expectations and feel more at ease., Conclusion: This suggests that prenatal counseling and the High-Risk Pregnancy Clinic (HRPC) provides perceived utility to families and mothers and that the HRPC is an effective method of providing prenatal counseling to mothers whose unborn children have been diagnosed with myelomeningocele., (© 2024. The Author(s).)

Objective: The objective was to identify clinical and radiological factors associated with sleep-disordered breathing (SDB) in children with Chiari type I malformation (CIM) and to evaluate the efficacy of foramen magnum decompression (FMD) in resolving SDB., Methods: A retrospective chart review was conducted for all children evaluated for CIM at a single institution from 2002 to 2022, identifying all children who had undergone nocturnal polysomnography (PSG). Apnea-hypopnea index (AHI) score, sleep apnea type (obstructive, central, mixed, and unspecified), clinical manifestations, and radiological measurements were recorded. SDB was considered present when officially diagnosed in the PSG report. Logistic regression was performed to identify factors correlating with the presence of SDB. For children with SDB who underwent FMD, the Wilcoxon signed-rank test was used to assess AHI improvement., Results: Of the 997 children referred for CIM, 310 completed PSG. SDB was diagnosed in 147 patients (overall prevalence 14.7%, 95% CI 12.7%-17.1%; prevalence among children with PSG 47.4%, 95% CI 41.9%-53%). Specific SDB diagnosis consisted of 33% of patients with central sleep apnea, 27% with obstructive sleep apnea, 9% mixed, and 31% unspecified. Lower cranial nerve (CN) dysfunction (OR 3.891, p = 0.009), tonsillar position (OR 1.049, p = 0.017), Chiari type 1.5 malformation (OR 1.862, p = 0.044), and BMI (OR 1.039, p = 0.036) were significantly associated with presence of SDB. Of the 310 patients who underwent PSG, 47 were originally categorized as asymptomatic: 27 (57%) of these asymptomatic patients were diagnosed with SDB on PSG. Of children diagnosed with SDB, 34 completed PSG before and after FMD. Median AHI score decreased from 6.5 preoperatively to 1.8 postoperatively, with a median (IQR) difference of -2.3 (-11.9 to 0.1) (p = 0.001). Twelve (35%) had resolution of SDB., Conclusions: The authors' findings suggest that the prevalence of SDB in children with CIM is high (15%-47%). Furthermore, lower CN dysfunction, Chiari type 1.5, lower tonsillar position, and higher BMI may be risk factors. Notably, SDB can be present even in the absence of clinical symptoms. This study also demonstrates that surgical intervention has the potential to reduce the severity of SDB. These results could help clinicians identify CIM patients at risk for SDB and those who may benefit from surgical decompression.

Objective: When the peritoneal cavity cannot serve as the distal shunt terminus, nonperitoneal shunts, typically terminating in the atrium or pleural space, are used. The comparative effectiveness of these two terminus options has not been evaluated. The authors directly compared shunt survival and complication rates for ventriculoatrial (VA) and ventriculopleural (VPl) shunts in a pediatric cohort., Methods: The Hydrocephalus Clinical Research Network Core Data Project was used to identify children ≤ 18 years of age who underwent either VA or VPl shunt insertion. The primary outcome was time to shunt failure. Secondary outcomes included distal site complications and frequency of shunt failure at 6, 12, and 24 months., Results: The search criteria yielded 416 children from 14 centers with either a VA (n = 318) or VPl (n = 98) shunt, including those converted from ventriculoperitoneal shunts. Children with VA shunts had a lower median age at insertion (6.1 years vs 12.4 years, p < 0.001). Among those children with VA shunts, a hydrocephalus etiology of intraventricular hemorrhage (IVH) secondary to prematurity comprised a higher proportion (47.0% vs 31.2%) and myelomeningocele comprised a lower proportion (17.8% vs 27.3%) (p = 0.024) compared with those with VPl shunts. At 24 months, there was a higher cumulative number of revisions for VA shunts (48.6% vs 38.9%, p = 0.038). When stratified by patient age at shunt insertion, VA shunts in children < 6 years had the lowest shunt survival rate (p < 0.001, log-rank test). After controlling for age and etiology, multivariable analysis did not find that shunt type (VA vs VPl) was predictive of time to shunt failure. No differences were found in the cumulative frequency of complications (VA 6.0% vs VPl 9.2%, p = 0.257), but there was a higher rate of pneumothorax in the VPl cohort (3.1% vs 0%, p = 0.013)., Conclusions: Shunt survival was similar between VA and VPl shunts, although VA shunts are used more often, particularly in younger patients. Children < 6 years with VA shunts appeared to have the shortest shunt survival, which may be a result of the VA group having more cases of IVH secondary to prematurity; however, when age and etiology were included in a multivariable model, shunt location (atrium vs pleural space) was not associated with time to failure. The baseline differences between children treated with a VA versus a VPl shunt likely explain current practice patterns.

Background: Obesity prevalence in youth with spina bifida is higher than in their typically developing peers. Obesity is associated with lifelong medical, psychological, and economic burdens. Successful prevention or treatment of obesity in individuals with spina bifida is compromised by (1) the lack of valid and reliable methods to identify body fat in a clinical setting and (2) limited data on energy expenditure that are necessary to provide daily caloric recommendations., Objective: The objectives of this study will be to develop 2 algorithms for use in youth with spina bifida in a clinical setting, one to model body fat and one to predict total daily energy expenditure. In addition, physical activity and dietary intake will be described for the sample., Methods: This multisite, prospective, national clinical study will enroll 232 youth with myelomeningocele aged 5 to 18 years (stratified by age and mobility). Participants will be enrolled for 1 week. Data obtained include 4 measures of body composition, up to 5 height measures, a ramped activity protocol, and a nutrition and physical activity screener. Participants will wear an accelerometer for the week. On the final study day, 2 samples of urine or saliva, which complete the doubly labeled water protocol, will be obtained. The analysis will include descriptive statistics, Bland-Altman plots, concordance correlation, and regression analysis., Results: The study received extramural federal funding in July 2019. Data collection was initiated in March 2020. As of April 2024, a total of 143 (female participants: n=76, 53.1%; male participants: n=67, 46.9%) out of 232 participants have been enrolled. Data collection is expected to continue throughout 2024. A no-cost extension until November 2025 will be requested for data analysis and dissemination of findings., Conclusions: This study furthers previous pilot work that confirmed the acceptability and feasibility of obtaining alternate height, body composition, and energy expenditure measures. The findings from this study will enhance screening, prevention, and treatment of abnormal weight status by facilitating the accurate identification of youths' weight status category and recommendations of daily caloric needs for this population that is at higher risk of obesity. Furthermore, the findings have the potential to impact outcomes for youth diagnosed with disabilities other than spina bifida who experience similar challenges related to alterations in body composition or fat distribution or measurement challenges secondary to mobility issues or musculoskeletal problems., International Registered Report Identifier (irrid): DERR1-10.2196/52779., (©Michele Polfuss, Kathryn Smith, Betsy Hopson, Andrea Moosreiner, Chiang-Ching Huang, Michele N Ravelli, Dan Ding, Zijian Huang, Brandon G Rocque, Rosemary White-Traut, Alexander Van Speybroeck, Kathleen J Sawin. Originally published in JMIR Research Protocols (https://www.researchprotocols.org), 02.07.2024.)

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., (© 2024. The Author(s).)

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., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the Congress of Neurological Surgeons.)

Objective: Hydrocephalus is a neurological disorder with an incidence of 80-125 per 100,000 births in the United States. The most common treatment, ventricular shunting, has a failure rate of up to 85% within 10 years of placement. The authors aimed to analyze the association between ventricular catheter (VC) tissue obstructions and shunt malfunction for each hydrocephalus etiology., Methods: Patient information was collected from 5 hospitals and entered into a REDCap (Research Electronic Data Capture) database by hydrocephalus etiology. The hardware samples were fixed, and each VC tip drainage hole was classified by tissue obstruction after macroscopic analysis. Shunt malfunction data, including shunt revision rate, time to failure, and age at surgery, were correlated with the degree of tissue obstruction in VCs for each etiology., Results: Posthemorrhagic hydrocephalus was the most common etiology (48.9% of total cases). Proximal catheter obstruction was the most frequent cause of hardware removal (90.4%). Myelomeningocele (44% ± 29%), other congenital etiologies (48% ± 40%), hydrocephalus with brain tumors (45% ± 35%), and posthemorrhagic hydrocephalus (41% ± 35%) showed tissue aggregates in more than 40% of the VC holes. A total of 76.8% of samples removed because of symptoms of obstruction showed cellular or tissue aggregates. No conclusive etiological associations were detected when correlating the percentage of holes with tissue for each VC and age at surgery, shunt revision rates, or time between shunt implantation and removal., Conclusions: The proximal VC obstruction was accompanied by tissue aggregates in 76.8% of cases. However, the presence of tissue in the VC did not seem to be associated with hydrocephalus etiology.

Objectives: To report pediatric intensivists' and pediatric neurosurgeons' responses to case-based scenarios about plasma and platelet transfusions before intracranial pressure (ICP) monitor placement in children with severe traumatic brain injury (TBI)., Design: Cross-sectional, electronic survey to evaluate reported plasma and platelet transfusion decisions in eight scenarios of TBI in which ICP monitor placement was indicated., Setting: Survey administered through the Pediatric Acute Lung Injury and Sepsis Investigators and the American Association of Neurologic Surgeons., Subjects: Pediatric intensivists and pediatric neurosurgeons., Interventions: None., Measurements and Main Results: A total of 184 participants responded (85 identified as pediatric intensivists and 54 as pediatric neurosurgeons). In all eight scenarios, the majority of respondents reported that they would base their decision-making about plasma transfusion on international normalized ratio (INR) alone (60-69%), or platelet transfusion on platelet count alone (83-86%). Pediatric intensivists, as opposed to pediatric neurosurgeons, more frequently reported that they would have used viscoelastic testing in their consideration of plasma transfusion (32% vs. 7%, p < 0.001), as well as to guide platelet transfusions (29 vs. 8%, p < 0.001), for the case-based scenarios. For all relevant case-based scenarios, pediatric neurosurgeons in comparison with pediatric reported that they would use a lower median (interquartile range [IQR]) INR threshold for plasma transfusion (1.5 [IQR 1.4-1.7] vs. 2.0 [IQR 1.5-2.0], p < 0.001). Overall, in all respondents, the reported median platelet count threshold for platelet transfusion in the case-based scenario was 100 (IQR 50-100) ×10 9 /L, with no difference between specialties., Conclusions: Despite little evidence showing efficacy, when we tested specialists' decision-making, we found that they reported using INR and platelet count in pediatric case-based scenarios of TBI undergoing ICP monitor placement. We also found that pediatric intensivists and pediatric neurosurgeons had differences in decision-making about the scenarios., Competing Interests: The authors have disclosed that they do not have any potential conflicts of interest., (Copyright © 2024 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.)

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., (© 2024. The Author(s).)

Objective: The Hydrocephalus Clinical Research Network (HCRN) conducted a prospective study 1) to determine if a new, better-performing version of the Endoscopic Third Ventriculostomy Success Score (ETVSS) could be developed, 2) to explore the performance characteristics of the original ETVSS in a modern endoscopic third ventriculostomy (ETV) cohort, and 3) to determine if the addition of radiological variables to the ETVSS improved its predictive abilities., Methods: From April 2008 to August 2019, children (corrected age ≤ 17.5 years) who underwent a first-time ETV for hydrocephalus were included in a prospective multicenter HCRN study. All children had at least 6 months of clinical follow-up and were followed since the index ETV in the HCRN Core Data Registry. Children who underwent choroid plexus cauterization were excluded. Outcome (ETV success) was defined as the lack of ETV failure within 6 months of the index procedure. Kaplan-Meier curves were constructed to evaluate time-dependent variables. Multivariable binary logistic models were built to evaluate predictors of ETV success. Model performance was evaluated with Hosmer-Lemeshow and Harrell's C statistics., Results: Seven hundred sixty-one children underwent a first-time ETV. The rate of 6-month ETV success was 76%. The Hosmer-Lemeshow and Harrell's C statistics of the logistic model containing more granular age and etiology categorizations did not differ significantly from a model containing the ETVSS categories. In children ≥ 12 months of age with ETVSSs of 50 or 60, the original ETVSS underestimated success, but this analysis was limited by a small sample size. Fronto-occipital horn ratio (p = 0.37), maximum width of the third ventricle (p = 0.39), and downward concavity of the floor of the third ventricle (p = 0.63) did not predict ETV success. A possible association between the degree of prepontine adhesions on preoperative MRI and ETV success was detected, but this did not reach statistical significance., Conclusions: This modern, multicenter study of ETV success shows that the original ETVSS continues to demonstrate good predictive ability, which was not substantially improved with a new success score. There might be an association between preoperative prepontine adhesions and ETV success, and this needs to be evaluated in a future large prospective study.

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., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Chiari I malformation (CIM) is characterized by descent of the cerebellar tonsils through the foramen magnum, potentially causing symptoms from compression or obstruction of the flow of cerebrospinal fluid. Diagnosis and treatment of CIM is varied, and guidelines produced through systematic review may be helpful for clinicians., Objective: To perform a systematic review of the medical literature to answer specific questions on the diagnosis and treatment of CIM., Methods: PubMed and Embase were queried between 1946 and January 23, 2021, using the search strategies provided in Appendix I of the full guidelines., Results: The literature search yielded 567 abstracts, of which 151 were selected for full-text review, 109 were then rejected for not meeting the inclusion criteria or for being off-topic, and 42 were included in this systematic review., Conclusion: Three Grade C recommendations were made based on Level III evidence. The full guidelines can be seen online at https://www.cns.org/guidelines/browse-guidelines-detail/1-imaging ., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

Background: Chiari malformation type I (CIM) diagnoses have increased in recent years. Controversy regarding the best operative management prompted a review of the literature to offer guidance on surgical interventions., Objective: To assess the literature to determine (1) whether posterior fossa decompression or posterior fossa decompression with duraplasty is more effective in preoperative symptom resolution; (2) whether there is benefit from cerebellar tonsillar resection/reduction; (3) the role of intraoperative neuromonitoring; (4) in patients with a syrinx, how long should a syrinx be observed for improvement before additional surgery is performed; and 5) what is the optimal duration of follow-up care after preoperative symptom resolution., Methods: A systematic review was performed using the National Library of Medicine/PubMed and Embase databases for studies on CIM in children and adults. The most appropriate surgical interventions, the use of neuromonitoring, and clinical improvement during follow-up were reviewed for studies published between 1946 and January 23, 2021., Results: A total of 80 studies met inclusion criteria. Posterior fossa decompression with or without duraplasty or cerebellar tonsil reduction all appeared to show some benefit for symptom relief and syrinx reduction. There was insufficient evidence to determine whether duraplasty or cerebellar tonsil reduction was needed for specific patient groups. There was no strong correlation between symptom relief and syringomyelia resolution. Many surgeons follow patients for 6-12 months before considering reoperation for persistent syringomyelia. No benefit or harm was seen with the use of neuromonitoring., Conclusion: This evidence-based clinical guidelines for the treatment of CIM provide 1 Class II and 4 Class III recommendations. In patients with CIM with or without syringomyelia, treatment options include bone decompression with or without duraplasty or cerebellar tonsil reduction. Improved syrinx resolution may potentially be seen with dural patch grafting. Symptom resolution and syrinx resolution did not correlate directly. Reoperation for a persistent syrinx was potentially beneficial if the syrinx had not improved 6 to 12 months after the initial operation. The full guidelines can be seen online at https://www.cns.org/guidelines/browse-guidelines-detail/3-surgical-interventions ., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

Background: Chiari I malformation (CIM) is characterized by descent of the cerebellar tonsils through the foramen magnum, potentially causing symptoms from compression or obstruction of the flow of cerebrospinal fluid. Diagnosis and treatment of CIM is varied, and guidelines produced through systematic review may be helpful for clinicians., Objective: To perform a systematic review of the medical literature to answer specific questions on the diagnosis and treatment of CIM., Methods: PubMed and Embase were queried between 1946 and January 23, 2021, using the search strategies provided in Appendix I of the full guidelines., Results: The literature search yielded 430 abstracts, of which 79 were selected for full-text review, 44 were then rejected for not meeting the inclusion criteria or for being off-topic, and 35 were included in this systematic review., Conclusion: Four Grade C recommendations were made based on Class III evidence, and 1 question had insufficient evidence. The full guidelines can be seen online at https://www.cns.org/guidelines/browse-guidelines-detail/2-symptoms ., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

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.

Objectives: In individuals with spina bifida (SB), bowel incontinence is associated with lower quality of life and lower likelihood of employment. In an effort to maximize bowel continence in children and adolescents, we created a bowel management assessment and follow-up protocol in a multidisciplinary clinic. Here we report the results of this protocol using quality-improvement methodology., Methods: Continence was defined as no unplanned bowel movements. Our protocol involved: (1) a standardized 4-item questionnaire about bowel continence and consistency; (2) if the patient was not achieving continence, an intervention starting with oral medication (stimulant and/or osmotic laxatives), and/or suppositories (glycerin or bisacodyl) followed by an escalation to trans-anal irrigation, or continence surgery; and (3) follow-up phone calls at regular intervals to monitor progress and make changes as needed. Results are summarized with descriptive statistics., Results: We screened 178 eligible patients in the SB clinic. Eighty-eight agreed to participate in the bowel management program. Of those who did not participate, the majority (68/90, 76%) were already achieving continence with their bowel regimen. Of children in the program, most (68/88, 77%) had a diagnosis of meningomyelocoele. At 1 year, the proportion of patients who were bowel accident free improved to 46% (vs 22% initially, P = 0.0007)., Conclusions: A standardized bowel management protocol, primarily the use of suppositories and trans-anal irrigation to achieve social continence, as well as frequent telephone follow-up, can reduce bowel incontinence in children and adolescents with SB., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objective: The aim of this study was to explore how clinical factors, including the number of lifetime revision surgeries and the duration of implantation, affect the degree of obstruction and failure rates of ventricular catheters (VCs) used to manage hydrocephalus., Methods: A total of 343 VCs and their associated clinical data, including patient demographics, medical history, and surgical details, were collected from 5 centers and used for this analysis. Each VC was classified by the degree of obstruction after macroscopic analysis. Univariate, multivariate, and binned analyses were conducted to test for associations between clinical data and degree of VC obstruction., Results: VCs from patients with 0 to 2 lifetime revisions had a larger proportion of VC holes obstructed than VCs from patients with 10 or more revisions (p = 0.0484). VCs implanted for less than 3 months had fewer obstructed holes with protruding tissue aggregates than VCs implanted for 13 months or longer (p = 0.0225). Neither duration of implantation nor the number of lifetime revisions was a significant predictor of the degree of VC obstruction in the regression models. In the multinomial regression model, contact of the VCs with the ventricular wall robustly predicted the overall obstruction status of a VC (p = 0.005). In the mixed-effects model, the age of the patient at their first surgery emerged as a significant predictor of obstruction by protruding tissue aggregates (p = 0.002). VCs implanted through the parietal entry site were associated with more holes with nonobstructive growth and fewer empty holes than VCs implanted via other approaches (p = 0.001)., Conclusions: The number of lifetime revisions and duration of implantation are correlated with the degree of VC obstruction but do not predict it. Contact of the VC with the ventricular wall and the age of the patient at their first surgery are predictors of the degree of VC obstruction, while the entry site of the VC correlates with it.

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., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: The authors analyzed the National Spina Bifida Patient Registry (NSBPR), a national registry that tracks the outcomes for people with various forms of spinal dysraphism, to determine the ongoing longitudinal risk for tethered cord release (TCR) among adults with myelomeningocele who had not previously undergone TCR during childhood. The authors also sought to identify the impact of lesion level, ambulation status, and prior treatments for hydrocephalus or Chiari malformations on TCR rates., Methods: Adults in the registry who had not previously undergone TCR during childhood were studied. This group was compared with the remaining adults in the registry. The frequency of first-time TCR and time to TCR (using Kaplan-Meier analysis) were determined independently for males and females. Cox proportional hazards analysis identified correlations between sex, best lesion level and ambulation status prior to TCR, and previous treatments for hydrocephalus and Chiari decompression., Results: Among 967 adults in the NSBPR (422 [43.6%] males and 545 [56.4%] females) who had not undergone TCR during childhood, the authors identified 47 people (4.9%) who underwent their first TCR during adulthood. This study cohort had significantly better mean functional motor levels and ambulation compared with the remaining adult cohort (both p < 0.001). The study group included 35 females (74.5%) and 12 males (25.5%); this sex distribution was significantly different in comparison with the remaining adult cohort (p = 0.016). The Kaplan-Meier curves for first TCR for females and males were significantly different (p = 0.01, log-rank test). TCR rates were correlated with sex (males had decreased risk; OR 0.31, 95% CI 0.16-0.62, p < 0.001), prior treatment for hydrocephalus (those who underwent prior treatment had decreased risk; OR 0.21, 95% CI 0.20-0.42, p < 0.001), and prior treatment for Chiari malformation (those who underwent prior treatment had greater risk; OR 3.84, 95% CI 1.50-9.88, p = 0.005)., Conclusions: Adults with myelomeningocele who escape childhood without undergoing TCR have an ongoing, albeit decreased, risk for spinal cord tethering requiring TCR. This risk is obviously not due to spinal column growth and therefore must reflect other factors such as dynamic changes in spinal cord health over time. Among people with MMC who underwent their first TCR as adults, females seemed to be overrepresented. Similar to the authors' prior childhood study, people who underwent previous Chiari decompression seemed to be overrepresented, whereas those who underwent previous treatment for hydrocephalus seem to be underrepresented. These novel findings deserve further study.

Objective: Abdominal pseudocyst (APC) can cause distal site failure in children with ventriculoperitoneal shunts and is specifically designated as an infection in Hydrocephalus Clinical Research Network (HCRN) protocols. Specific management and outcomes of children with APCs have not been reported in a multicenter study. In this study, the authors investigated the management and outcomes of APC in children with shunted hydrocephalus who were treated at centers in the HCRN., Methods: The HCRN Registry was queried to identify children < 18 years old with shunts who were diagnosed with an APC (i.e., a loculated abdominal fluid collection containing the peritoneal catheter with abdominal distention and/or displacement of peritoneal contents). The primary outcome was shunt failure after APC treatment. The primary variable was reimplantation of the distal catheter after pseudocyst treatment back into the peritoneum versus implantation in a nonperitoneal site. Other risk factors for shunt failure after APC treatment and variability in APC management were investigated., Results: Among 141 children from 14 centers who underwent first-time management of an APC over a 14-year period, the median time from previous shunt surgery to APC diagnosis was 3.8 months. Overall, 17.7% of children had a positive culture: APC cultures were positive in 14.2% and CSF cultures in 15.6%. Six other children underwent shunt revision without removal; all underwent reoperation within 1 month. There was no difference in shunt survival (log-rank test, p = 0.42) or number of subsequent revisions within 6, 12, or 24 months for shunts reimplanted in the abdomen versus those implanted in a nonperitoneal location. Nonperitoneal implantation was associated with more noninfectious revisions (42.3% vs 22.9%, p = 0.019), whereas infection was more common after reimplantation in the abdomen (25.7% vs 7.0%, p = 0.003). Univariable analysis demonstrated that younger age at APC diagnosis (8.3 vs 12.2 years, p = 0.006) and prior shunt procedure within 12 weeks of APC diagnosis (59.5% vs 40.5%, p = 0.012) were associated with shunt failure after APC treatment. Multivariable modeling confirmed that prior shunt surgery within 12 weeks of APC diagnosis was independently associated with failure (HR 1.79 [95% CI 1.04-3.07], p = 0.035)., Conclusions: In the HCRN, APCs in the setting of CSF shunts are usually managed with externalization. Shunt surgery within 12 weeks of APC diagnosis was associated with risk of failure after APC treatment. Although no differences were found in overall shunt failure rate, noninfectious shunt revisions were more common in the nonperitoneal distal catheter sites, and infection was a more common reason for failure after reimplantation of the shunt in the abdomen.

Introduction: Pediatric cranial defects can be preceded by prior infection, radiation therapy, failed prior cranioplasty, or cerebrospinal fluid leak, leading to a complex reconstructive environment. The primary aim of this study was to investigate differences in outcomes between pediatric patients with hostile reconstructive environments who received split-calvarial autologous grafts as opposed to prosthetic grafts in cranioplasty., Methods: We performed an institutional review board-approved retrospective chart review of 51 patients younger than 18 years who underwent cranioplasty with a hostile setting between 1998 and 2020. Patients were then stratified into prosthetic (45%) and autologous groups (54%). The primary outcome measured was postoperative complication, defined as requirement of a subsequent surgery or revision., Results: Overall, there were no significant differences in age, sex, type of hostile setting, etiology of cranial defect, or side of the cranial defect between the 2 groups. Complication rate among the 2 graft groups was 18%. However, there were no significant differences in complications, defined as infection, failure or resorption of the graft, wound breakdown or necrosis, resulting bone defect, or hematoma, between the 2 populations. There was a significant difference in etiology between patients with complications, with patients who required a cranioplasty due to previous hemicraniectomy being nearly 5 times as likely to face a complication ( P = 0.045)., Conclusions: In our study, there was no significant difference observed in complications between prosthetic and split-thickness autologous grafts in pediatric patients with hostile settings. It does, however, seem that patients who had a previous hemicraniectomy are more likely to face complications as a result of cranioplasty., Competing Interests: Conflicts of interest and sources of funding: This article was funded in part by the National Center for Advancing Translational Sciences of the National Institutes of Health under award number UL1TR003096., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: Persistent hydrocephalus following posterior fossa brain tumor (PFBT) resection is a common cause of morbidity in pediatric brain tumor patients, for which the optimal treatment is debated. The purpose of this study was to compare treatment outcomes between VPS and ETV in patients with persistent hydrocephalus following surgical resection of a PFBT., Methods: A post-hoc analysis was performed of the Hydrocephalus Clinical Research Network (HCRN) prospective observational study evaluating VPS and ETV for pediatric patients. Children who experienced hydrocephalus secondary to PFBT from 2008 to 2021 were included. Primary outcomes were VPS/ETV treatment failure and time-to-failure (TTF)., Results: Among 241 patients, the VPS (183) and ETV (58) groups were similar in age, extent of tumor resection, and preoperative ETV Success Score. There was no difference in overall treatment failure between VPS and ETV (33.9% vs 31.0%, p = 0.751). However, mean TTF was shorter for ETV than VPS (0.45 years vs 1.30 years, p = 0.001). While major complication profiles were similar, compared to VPS, ETV patients had relatively higher incidence of minor CSF leak (10.3% vs. 1.1%, p = 0.003) and pseudomeningocele (12.1% vs 3.3%, p = 0.02). No ETV failures were identified beyond 3 years, while shunt failures occurred beyond 5 years. Shunt infections occurred in 5.5% of the VPS cohort., Conclusions: ETV and VPS offer similar overall success rates for PFBT-related postoperative hydrocephalus. ETV failure occurs earlier, while susceptibility to VPS failure persists beyond 5 years. Tumor histology and grade may be considered when selecting the optimal means of CSF diversion., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Advances in medicine and technology, have enabled greater numbers of children with complex illness to survive into adulthood. Adolescents with these conditions are at high risk for adverse outcomes when transitioning to adult health care. The "Staging Transition for Every Patient" (STEP) Program was developed to systematically improve the transition from pediatric to adult healthcare., Objective: This article details the development of the STEP program and the novel use of "Individualized Transition Plans" (ITP) in the clinic setting., Methods: A provider needs' assessment of the existing transition services among youth with specific diagnoses was performed, a steering committee was developed that created a transition policy, and a medical home within the adult system was established with an interdisciplinary approach. The ITP focuses on 5 individualized goals, it was developed and tested with the first-year cohort of patients., Results: In the initial needs assessment, 7 of 35 diagnoses were found to have an effective transition plan. The STEP program partnered with departments across the adult facility to conduct 267 interdisciplinary patient visits. In the first year, 169 new patients were seen in the clinic. The average age was 23.0 ± 4.1 years old. The ITP goals included referrals to adult specialists, advanced care planning, career and education, transition readiness, caregiver burden, and an emergency sick plan., Conclusion: There is a need for organized transition care for medically complex youth. The STEP program answers that need by addressing the unique needs of each patient. Individualized transition planning builds trust and addresses multiple domains of health., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Purpose: Hyponatremia after craniotomy can be associated with increased morbidity. However, the incidence of and factors associated with post-craniotomy hyponatremia in children are not known., Methods: We performed a retrospective cohort study of patients aged 0-21 years who underwent craniotomy in 2017-2019 at a single center to determine the incidence of and to identify risk factors for hyponatremia after craniotomy. Indications for craniotomy included tumors (excluding craniopharyngioma), epilepsy, intracranial infection, trauma, craniofacial, suboccipital decompression for the treatment of Chiari malformation, and cerebrovascular disease. Hyponatremia was defined as a serum sodium level ≤ 135 mEq/L any time during the postoperative hospital stay. Statistical significance was defined a priori at p < 0.05., Results: Postoperative hyponatremia occurred in 61 (25%) of 240 children. On univariate analysis, hyponatremia was associated with younger age (8.5 vs 6.3 years, p = 0.01), use of preoperative anti-epileptic drugs (p = 0.02), need for blood transfusion (p = 0.02), government/private insurance (p = 0.04), and pre-existing hydrocephalus, defined as the requirement for permanent cerebrospinal fluid (CSF) diversion (p = 0.04). On multivariate analysis, only hydrocephalus (OR 2.95, 95% CI 1.03-8.40) remained statistically significant. Hyponatremia most occurred on the first postoperative day, with normonatremia achieved in a median of 14 (IQR 9.8-24.3) h. Hyponatremia was significantly associated with longer length of stay (median 8 vs 3 days, p < 0.01)., Conclusion: Hyponatremia was present in 25% of children after craniotomy. Preoperative hydrocephalus as an independent risk factor for hyponatremia after craniotomy., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Intrathecal baclofen (ITB) therapy is an effective treatment for spasticity and dystonia in children with cerebral palsy (CP). However, ITB pump surgery is associated with one of the highest rates of surgical site infection (SSI) in medicine, leading to significant morbidity and expense. Surgical protocols have reduced the rate of SSI in children with other CNS implants, and single-center protocols have been effective in ITB surgery in pediatrics. The authors describe the first multicenter quality improvement (QI)-driven standardized protocol for ITB pump surgery in children with CP across the Cerebral Palsy Research Network (CPRN), implemented with the aim of reducing ITB-associated SSI., Methods: SSI was defined as a culture-positive infection, ITB pump system removal for suspected infection, or wound dehiscence with exposed hardware. Each center reported historical infection rates for at least 3 years before initiating the SSI protocol (preintervention phase). After initiation of a 13-step surgical protocol, a consecutive series of 130 patients undergoing 149 surgical procedures for ITB at four CPRN tertiary pediatric neurosurgery centers were prospectively enrolled at surgery during a 2-year study period (intervention phase). QI methodology was used, including development of a key driver diagram and tracking performance using run and control charts. The primary process measure goal was documented compliance with 80% of the protocol steps, and the primary outcome measure goal was a 20% reduction in 90-day infection rate. Patient characteristics were collected from the CPRN Research Electronic Data Capture registry, including age at surgery, BMI, Gross Motor Function Classification System level, and pattern of spasticity., Results: The aggregated preintervention 90-day ITB SSI rate was 4.9% (223 procedures) between 2014 and 2017. During the intervention phase, 136 of 149 ITB surgeries performed met inclusion criteria for analysis. The mean documented compliance rate with protocol steps was 75%, and the 90-day infection rate was 4.4%, with an average of 42 days from index surgery to infection., Conclusions: This is the first multicenter QI initiative designed to reduce SSI in ITB surgery in children with CP. Ongoing enrollment and expansion of the protocol to other CPRN centers will facilitate identification of patient- and procedure-specific risk factors for SSI, and iterative plan-do-study-act cycles incorporating these data will further decrease the risk of SSI for ITB surgery in children.

Objective: The purpose of this study was to find areas of agreement among pediatric neurosurgeons with respect to the clinical management of asymptomatic newborns with a variety of lumbosacral skin findings., Methods: An electronic survey containing 18 clinical images and brief vignettes was sent to pediatric neurosurgeons within the American Academy of Pediatrics Section of Neurological Surgery (AAP SONS). In total, 38% (n = 21) of AAP SONS members submitted complete responses to the survey. Respondents were asked if they would advise routine care, watchful waiting, imaging, or subspecialty consultation for each clinical case. Responses were categorized into two groups: 1) watchful waiting and/or routine care, or 2) imaging and/or subspecialty consultation. Consensus was categorized as good (> 90% of responses in the same group), modest (70%-90%), and poor (< 70%). Demographic information, local factors impacting management, and experiences with local referral patterns were also collected., Results: Among the pediatric neurosurgeons within the AAP SONS network, the authors found high levels (> 90%) of agreement that subcutaneous lipomas, faun tail nevi, large skin tags, and deep/atypical lumbosacral dimples in asymptomatic newborns should prompt an imaging study. Similarly, the authors found high agreement that simple coccygeal dimples do not need imaging. The management of some types of lumbosacral vascular marks and gluteal crease deviations had poor agreement (< 70%). When imaging was recommended, there was preference for spinal MRI in most cases (67%)., Conclusions: Pediatric neurosurgeons generally agree that imaging of the spine is indicated for asymptomatic newborns with subcutaneous lipomas, faun tail nevi, large skin tags, or deep/atypical lumbar dimples (deep or atypical appearing). They also agree that imaging is unnecessary for infants with simple coccygeal dimples. There was a notable lack of consensus on the appropriate management of certain gluteal cleft deviations and cutaneous vascular marks.

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.

Objectives: Transition of care is the planned movement of adolescents and young adults from pediatric to adult health care. Many studies have demonstrated the importance of an organized transition process. The purpose of this study is to determine the efficacy of a telemedicine intervention for improving transition readiness among adolescents with spina bifida., Methods: The present study is a randomized, controlled trial, including children 14 years of age and older with myelomeningocele from a multidisciplinary spina bifida clinic. Subjects were randomized to standard care or to an intervention, consisting of video telemedicine contacts at 3, 6, and 9 months from the clinic visit. The primary outcome measure was a change in the Transition Readiness Assessment Questionnaire score from baseline to 1-year follow-up., Results: Twenty-four patients were enrolled in the study and underwent randomization. The mean age at enrollment was 15.8 years. Ten patients (40%) were female, and the majority were White, non-Hispanic (67%). Despite enrolling 24 patients, only 1 patient in the telemedicine group completed any of the planned telemedicine sessions. No other participant completed any telemedicine counseling sessions. The study was stopped early for lack of participation in the intervention. In a single-group, as-treated analysis, there was no significant change in the Transition Readiness Assessment Questionnaire score between enrollment and 1-year follow-up (Δ = 0.36, P = 0.46). However, there were significant improvements in subscores for Managing Medications, Appointment Keeping, and Managing Daily Activities., Conclusions: The primary finding from this study was very low participation in a telemedicine video follow-up intervention among adolescents with myelomeningocele. Based on these results, this strategy alone is unlikely to significantly improve readiness for transition to adult care., (Published by Elsevier Inc.)

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2022 Savage, Hale, Parr, Hedaya, Saccomano, Tsemo, Hafeez, Tanweer, Kan, Solomon, Meila, Dirks, Blount, Johnston, Rocque, Rozzelle, Bhatia, Muthusami, Krings and Jones.)

Objective: Hydrocephalus is common among children with myelomeningocele and is most frequently treated with a ventriculoperitoneal shunt (VPS). Although much is known about factors related to first shunt failure, relatively less data are available about shunt failures after the first one. The purpose of this study was to use a large data set to explore time from initial VPS placement to first shunt failure in children with myelomeningocele and to explore factors related to multiple shunt failures., Methods: Data were obtained from the National Spina Bifida Patient Registry. Children with myelomeningocele who were enrolled within the first 5 years of life and had all lifetime shunt operations recorded in the registry were included. Kaplan-Meier survival curves were constructed to evaluate time from initial shunt placement to first shunt failure. The total number of children who experienced at least 2 shunt failures was calculated. A proportional means model was performed to calculate adjusted hazard ratios (HRs) for shunt failure on the basis of sex, race/ethnicity, lesion level, and insurance status., Results: In total, 1691 children met the inclusion criteria. The median length of follow-up was 5.0 years. Fifty-five percent of patients (938 of 1691) experienced at least 1 shunt failure. The estimated median time from initial shunt placement to first failure was 2.34 years (95% confidence interval [CI] 1.91-3.08 years). Twenty-six percent of patients had at least 2 shunt failures, and 14% of patients had at least 3. Male children had higher likelihood of shunt revision (HR 1.25, 95% CI 1.09-1.44). Children of minority race/ethnicity had a lower likelihood of all shunt revisions (non-Hispanic Black children HR 0.74, 95% CI 0.55-0.98; Hispanic children HR 0.74, 95% CI 0.62-0.88; children of other ethnicities HR 0.80, 95% CI 0.62-1.03)., Conclusions: Among the children with myelomeningocele, the estimated median time to shunt failure was 2.34 years. Forty-five percent of children never had shunt failure. The observed higher likelihood of shunt revisions among males and lower likelihood among children of minority race/ethnicity illustrate a possible disparity in hydrocephalus care that warrants additional study. Overall, these results provide important information that can be used to counsel parents of children with myelomeningocele about the expected course of shunted hydrocephalus.

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.

Objective: Little is known about the prevalence of attention-deficit/hyperactivity disorder (ADHD) in children with hydrocephalus. In this study, the authors assessed the prevalence of ADHD and its association with clinical and demographic factors, including intellectual disability (ID), a potential factor that can confound the diagnosis of ADHD., Methods: The authors conducted a cross-sectional study of children 6-12 years of age with hydrocephalus using parent telephone surveys. The Child and Adolescent Intellectual Disability Screening Questionnaire (CAIDS-Q) and the National Institute for Children's Health Quality (NICHQ) Vanderbilt Assessment Scale were used to screen for ID and ADHD, respectively. Among children without ID, the authors identified those with ADHD and calculated a prevalence estimate and 95% confidence interval (Wald method). Logistic regression analysis was conducted to compare children with ADHD with those without ADHD based on demographics, family income, parental educational, etiology of hydrocephalus, and primary treatment. As a secondary analysis, the authors compared subjects with ID with those without using the same variables. Multivariable analysis was used to identify factors with independent association with ADHD and ID., Results: A total of 147 primary caregivers responded to the telephone questionnaire. Seventy-two children (49%) met the cutoff score for ID (CAIDS-Q). The presence of ID was significantly associated with lower family income (p < 0.001). Hydrocephalus etiology (p = 0.051) and initial treatment (p = 0.06) approached significance. Of children without ID (n = 75), 25 demonstrated a likely diagnosis of ADHD on the NICHQ, yielding a prevalence estimate of 0.33 (95% CI 0.22-0.44). No clinical or demographic variable showed significant association with ADHD., Conclusions: These data indicate that the prevalence of ADHD among children with hydrocephalus (33%) is higher than among the general population (estimated prevalence in Alabama is 12.5%). ID is also common (49%). Routine screening for ADHD and ID in children with hydrocephalus may help to ensure that adequate resources are provided to optimize functional outcomes across development.

Purpose: Ultrasound is the primary method for prenatal identification of myelomeningocele and is critical to prognostication and treatment planning. No study has considered the degree of inaccuracy of prenatal US lesion level estimates and anatomic lesion level on postnatal imaging using the weighted kappa coefficient (κw), nor the impact of maternal BMI on agreement. We examined the accuracy of prenatal ultrasound lesion level estimation in a cohort of patients with myelomeningocele using κw and determined whether BMI influenced accuracy., Methods: The study is a retrospective review including patients born 2011-2019 who had prenatal imaging and primary myelomeningocele closure at a single institution. Lesion levels from prenatal ultrasound and postnatal imaging studies were analyzed for agreement at exact level, within 1 level, and within 2 levels using κw. Maternal BMI was examined for correlation with accuracy., Results: Fifty-seven patients met inclusion criteria. Mean BMI was 31.2. There was no association between maternal BMI and agreement at any level. Lesion level on prenatal ultrasound agreed with postnatal imaging to the exact level in 13 (22.8%) cases, within 1 level in 38 (66.7%) cases, and within 2 levels in 50 (87.7%) cases. Weighted kappa showed moderate agreement at exact level (κ w = 0.494) and substantial agreement within 1 (κ w = 0.761) and 2 levels (κ w = 0.902)., Conclusion: Weighted kappa adds confidence for clinical decision making by accounting for accuracy. Prenatal ultrasound is a reliable and accurate method of determining lesion level with near-perfect agreement to postnatal imaging within 2 spinal levels. Maternal BMI may not influence lesion level determination after initial diagnosis., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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.

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.

Background: Improvements in antenatal medicine and surgical management for conditions associated with spina bifida such as hydrocephalus have extended the lifespan for individuals with spina bifida (SB) into adulthood. Decisions and education regarding reproductive care and pregnancies for patients with spina bifida are increasingly important. Pregnancy in these patients can be particularly challenging due to physical limitations, previous abdominal surgery for urinary or bowel management and presence of a ventriculoperitoneal shunt. To date, little research has examined the unique challenges that women with spina bifida face during pregnancy., Objective: The purpose of this descriptive study is to characterize the successful pregnancy histories of SB women and describe how pregnancy affected their mobility as well as bladder and bowel management., Study Design: We conducted semi-structured interviews with women followed in our adult multidisciplinary SB clinic who previously had successful pregnancies. Questions regarding perinatal issues, obstetrical complications, urinary tract infections (UTI) and neurological changes were asked. Baseline mobility, bladder and bowel management were compared with changes during and after pregnancy., Results: 121 women of childbearing age were followed per year by our adult multidisciplinary spina bifida clinic between 2009 and 2016. We identified 6 women who successfully carried 8 pregnancies to term. There were no miscarriages. Four women had ventriculoperitoneal (VP) shunts. No children were born with neural tube defects. Mean age at first pregnancy was 23.5 years. Average gestational age at delivery was 37 weeks. 50% of the women had a spontaneous vaginal delivery. Five of six women intended to get pregnant; only one patient consumed folic acid regularly prior to pregnancy. Two of six women had bladder augmentation surgery, one of whom had urologic changes during pregnancy that persisted after childbirth. The other patient had a concomitant bladder neck sling procedure and did not have urologic issues during pregnancy. 50% of the patients experienced bladder-bowel dysfunction during their pregnancy. While 67% patients had full baseline ambulatory function, 4 patients had decreased mobility and required additional assistance during pregnancy. All returned to their baseline functionality afterwards., Conclusion: Six of our patients had eight successful pregnancies, with no children born with neural tube defects. New changes to mobility, bladder and bowel management were experienced by over half of the women during their pregnancies. Future studies should focus on the role of multidisciplinary teams in reproductive health education and perinatal management of changes to activities of daily living during pregnancy in this population., Competing Interests: Conflicts of interest None., (Copyright © 2021 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)