Pediatric CSF diversion procedures for treatment of hydrocephalus during the COVID-19 pandemic.

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.

Nongovernmental Organizations in Global Neurosurgery: Foundation for International Education in Neurological Surgery and Solidarity Bridge.

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.

Neurosurgical management of Myelomeningocele in premature infants: a case series.

INTRODUCTION: Myelomeningocele (MMC) is the most common neural tube defect, but rarely seen in premature infants. Most centers advocate for closure of MMC within 24 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.

Predicting the presence of 4th ventricular outlet obstruction in Chiari I Malformation.

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.

Endoluminal Biopsy for Vein of Galen Malformation.

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

Selective Dorsal Rhizotomy for Spastic Cerebral Palsy: Report of 18 Cases Performed in the North of Vietnam.

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.

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

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

Ventricular catheter tissue obstruction and shunt malfunction in 9 hydrocephalus etiologies.

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.

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

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

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

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

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Patients With Chiari Malformation: Surgical Interventions.

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 .

Exploration of clinical predictors of the degree of ventricular catheter obstruction: a multicenter retrospective study.

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.

Comparison of outcomes in the management of abdominal pseudocyst in children with shunted hydrocephalus: a Hydrocephalus Clinical Research Network 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.

First-time tethered cord release among adults with myelomeningocele: an analysis of people in the National Spina Bifida Patient Registry.

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.

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

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

Improving Bowel Management in Children With Spina Bifida.

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.

Treatment of hydrocephalus following posterior fossa tumor resection: a multicenter collaboration from the Hydrocephalus Clinical Research Network.

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.

Pediatric Cranioplasty Patients With Hostile Reconstructive Environments: Split Calvarial Versus Prosthetic Implant.

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.

Management of asymptomatic cutaneous lumbosacral findings in newborns: a neurosurgical perspective.

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.

A multicenter initiative to reduce intrathecal baclofen pump surgical site infection: a Cerebral Palsy Research Network quality improvement project.

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.