Journal of neurosurgery. Pediatrics最新文献

Objective: Improving outcomes for pediatric patients with hydrocephalus in low- and middle-income countries (LMICs) requires research on ventriculoperitoneal shunt (VPS) complications and outcomes that may be comparable to studies conducted in high-income countries (HICs). The authors aimed to address this gap by conducting a systematic review to analyze VPS complications and outcomes among pediatric patients in LMICs.

Methods: This review adhered to PRISMA guidelines and the Assessing the Methodological Quality of Systematic Reviews (AMSTAR 2) quality assessment. The authors screened English-language publications on pediatric VPS surgeries in LMICs, excluding studies from HICs and adult populations (> 18 years), using 6 databases: PubMed/Medline, Embase, Cochrane Library, Web of Science, Global Index Medicus, and Google Scholar. The search was completed on August 31, 2023. Data extraction included patient demographics, diagnosis, management, complications, and outcomes. Descriptive analyses were performed using Google Spreadsheets and R.

Results: A total of 590 studies were included, revealing trends in VPS utilization across 6 continents. Among 25,950 patients, the majority were aged 0-5 years (13,044/15,008, 86.9%), with a slight male predominance (11,043/19,971, 55.3%). Key complications included infections (mainly involving Staphylococcus spp.), shunt failure, and obstruction. Outcomes were reported for 7185 patients, representing 27.7% of the total cohort. Favorable outcomes were observed in 68.1% (4893 patients) and unfavorable outcomes in 13.1% (938 patients), and the overall mortality rate was 18.8% (1354 patients).

Conclusions: This review provides a comprehensive profile of VPS complications and outcomes in pediatric patients in LMICs. Despite a predominance of observational studies, these findings offer critical insights that may inform health policy and practice in LMICs. Future research should prioritize longitudinal studies to explore long-term outcomes, develop cost-effective approaches to reduce complications, and foster international collaborations to strengthen global neurosurgical capacity.

Objective: Stereotactic gene therapy in children is challenging due to the fragility of the infant skull and long hours of infusion. The thalamus, an integrative hub for the entire cortex, has been shown to facilitate widespread gene/protein delivery via axonal transport. The aim of this study was to evaluate the safety and accuracy of bilateral thalamic convection-enhanced delivery (CED) of adeno-associated virus (AAV) vectors for GM2 gangliosidoses in children and to assess outcomes based on post-infusion imaging.

Methods: This clinical trial enrolled 10 pediatric patients, including 7 infants and toddlers and 3 children ranging in age from 5 to 12 years, who underwent bilateral thalamic rAAVrh8-HEXA/HEXB delivery via CED for GM2 gangliosidoses. The approximate trajectory entered the middle frontal gyrus, lateral to the frontal horn and caudate, passing through the anterior limb of the internal capsule to the thalamus. Injection volumes ranged from 180 μL to 1250 μL at a rate of 4 μL/min. The surgical technique for trajectory planning, cranial stabilization, operating room setup, stereotactic cannula placement, infusion parameters, and gene delivery was reviewed. The accuracy of catheter placement was calculated, and the volume of distribution (Vd) versus the volume of infusate (Vi) based on thalamic signal intensity on MRI was quantified.

Results: Ten children (age range 6 months to 12 years) with GM2 gangliosidosis were included. Twenty infusion cannulas were successfully placed for bilateral thalamic drug delivery. Intrathalamic cannula distance measurements ranged from 12.4 to 15.3 mm, and all the cannula steps were inside the thalamic boundaries to prevent backflow. There were no significant intraoperative complications. The mean targeting error was 1.3 ± 0.8 mm. The calculated volume of thalamic coverage by the infusate signal ranged from 5.9% to 53.3% (mean 26.9% ± 15.5%) in a dose escalating pattern. The Vd/Vi ratio ranged from 0.60 to 2.8 (mean 2.0 ± 0.6), depending on the infusate volume. The diffusate shape was circular to slightly ellipsoid in all patients.

Conclusions: In this study, the surgical technique used in the first in-human intrathalamic drug infusion gene therapy trial in young children with GM2 gangliosidosis was reviewed. The innovative stereotactic setup used robotic surgical assistance and ensured high-precision targeting and secure cannula placement during prolonged infusions, even in infants as young as 6 months of age. Post-infusion MRI confirmed that the infusate remained well contained within the thalamus. The mean Vd/Vi ratio of 2.0 is consistent with the literature. Overall, bilateral thalamic delivery of AAV transgene in children was safe and well tolerated.

Objective: Traumatic brain injury (TBI) is a leading cause of mortality and morbidity among children in the United States, with nearly a half million pediatric TBI-related emergency visits annually. The authors aimed to investigate geospatial disparities in pediatric TBI across ZIP Code Tabulation Areas (ZCTAs) and to assess the association of neighborhood sociodemographic factors with pediatric TBI incidence rate and outcomes.

Methods: A retrospective cross-sectional study was conducted to examine the electronic medical records of pediatric patients treated at a level I pediatric trauma center between June 2016 and June 2023. Data were linked with ZCTA-level socioeconomic indicators from the American Community Survey 5-year estimates. Neighborhood-level social disadvantage, including the Social Deprivation Index (SDI), median household income, housing characteristics, and health coverage patterns, was assessed. Pediatric TBI incidence rates were calculated using spatial Bayesian smoothing techniques. Global Moran's I test was used to assess spatial autocorrelation, while the local indicators of spatial association test was used to identify TBI hot spots and cold spots. Incidence rate ratios (IRRs) were derived using zero-inflated negative binomial regression. Injury severity (via the Injury Severity Score [ISS]), hospital length of stay (LOS), discharge disposition, and mortality were examined.

Results: Among 2809 patients (median age 6 years [IQR 1-12 years], 36.4% female), 47 ZCTAs were identified as hot spots and 143 as cold spots. Compared with cold spots, hot spot ZCTAs had a higher child population density, greater proportions of renter-occupied housing units, lower median household incomes, shorter mean travel times to work, higher rates of public health insurance coverage, and higher SDI scores (all p < 0.001). In multivariable regression, higher vacant housing units (IRR 1.032, 95% CI 1.014-1.051; p < 0.001), lower proportions of individuals working from home (IRR 0.941, 95% CI 0.921-0.963; p < 0.001), lower private health insurance coverage (IRR 0.979, 95% CI 0.969-0.990; p < 0.001), and higher poverty (IRR 1.073, 95% CI 1.047-1.110; p < 0.001) were associated with increased TBI incidence rates. Compared with other areas, patients from hot spots had a lower median ISS (5 vs 6, p < 0.001) and fewer prolonged hospital LOS events (25.1% vs 32.0%, p < 0.001), but no significant differences in discharge disposition or mortality (both p > 0.05).

Conclusions: In this cross-sectional study, pediatric TBI rates clustered disproportionately in socioeconomically disadvantaged areas. These findings underscore the need for targeted, neighborhood-level prevention strategies and policies addressing social determinants to mitigate the rising burden of pediatric TBI.

Objective: Myelomeningocele (MMC) is a severe neural tube defect frequently associated with vertebral anomalies, including scoliosis and kyphosis, which can significantly impact mobility and quality of life. This study aimed to evaluate the incidence and clinical correlations of scoliosis and other vertebral anomalies in children with MMC.

Methods: A retrospective analysis of the hospital records of 422 pediatric MMC patients diagnosed between 2013 and 2020 at two tertiary care centers was conducted. Patients were evaluated for scoliosis, kyphosis, hemivertebra, butterfly vertebra, block vertebra, and diastematomyelia using radiographic and MRI findings. The severity of scoliosis was assessed using Cobb angles, and statistical analyses were performed to determine associations between vertebral anomalies.

Results: Scoliosis was identified in 55.9% of patients (mean Cobb angle 35.65°), while kyphosis was present in 41.2%. The presence of hemivertebra and butterfly vertebra was strongly associated with scoliosis progression. Additionally, patients with split cord malformations exhibited a higher incidence of scoliosis and kyphosis. Age was found to be a key factor in scoliosis severity, with curve progression observed over time.

Conclusions: Scoliosis and other vertebral anomalies are highly prevalent in MMC patients, necessitating early diagnosis and multidisciplinary management. These findings underscore the importance of long-term monitoring and individualized treatment approaches to optimize spinal health and functional outcomes.

Objective: This study aimed to investigate the effect of intrathecal baclofen (ITB) on BMI over time in a large pediatric cohort of patients with cerebral palsy (CP).

Methods: The medical records of pediatric patients diagnosed with CP who underwent ITB pump placement at Texas Children's Hospital between 2007 and 2024 were retrospectively reviewed. Pre- and postoperative BMI, demographic information, and clinical characteristics were collected. Multiple BMI velocities were calculated. A linear mixed-effects model was used to account for interpatient variability.

Results: Among 237 patients, the average BMI was 17.90 (SD 4.00) kg/m2 preoperatively and 19.13 (SD 4.58) kg/m2 postoperatively, showing a significant difference (p = 0.001, Kruskal-Wallis test) but a small effect size (η2 = 0.02, 95% CI 0.001-0.05). The average BMI velocity was 0.55 (SD 3.5) kg/m2/yr presurgery and 0.46 (SD 3.5) kg/m2/yr postsurgery, showing no significant difference (p = 0.52, t-test). The mixed-effects model found no statistically significant effect of ITB surgery on BMI rate of change by catheter level. Specifically, cervical (p = 0.97), high thoracic (p = 0.41), midthoracic (p = 0.63), and low thoracic (p = 0.84) catheter levels were nonsignificant in effect on BMI.

Conclusions: Although there was an absolute increase in BMI postoperatively, the small effect size and results of the linear mixed-effects model-accounting for clinical confounders, within-patient variability, and catheter level-demonstrated that ITB surgery does not significantly affect BMI. The authors conclude that improved tone control may not substantially impact BMI, necessitating further nutritional intervention to ensure optimal BMI.

Objective: Economic constraints as well as viral surges straining hospital capacity drive shorter hospital lengths of stay. There is a paucity of data examining the safety of outpatient minimally invasive craniosynostosis repair. The authors aimed to examine hospital events of patients undergoing minimally invasive craniosynostosis repair who were admitted and discharged on postoperative day (POD) 1 compared with those who were discharged on POD 0 to develop criteria for same-day discharge.

Methods: This study was a retrospective review of consecutive patients with single-suture craniosynostosis evaluated between January 2020 and December 2022. All patients underwent minimally invasive strip craniectomy with postoperative helmet therapy at a single institution. Concurrently, a prospective analysis of patients discharged on POD 0 was performed from October 2022 to May 2024. Patients with postoperative hemoglobin levels > 7 g/dl, no intraoperative complications, stable vital signs, and tolerating a diet were discharged following surgery on the same day.

Results: Forty-six patients (30 males, 16 females) were included in this study. Thirteen patients (8 male, 5 female) were discharged on POD 0 with an average age at repair of 2.8 (SD 0.7) months. No complications or blood transfusions were noted in this group. The retrospectively reviewed inpatient group included 33 patients (22 male, 11 female). The majority of patients in this group were discharged on POD 1. Most patients undergoing minimally invasive strip craniectomy for single-suture craniosynostosis did not require blood transfusions and had minimal perioperative events. Most patients in the retrospectively reviewed inpatient group had an uncomplicated hospital course and were discharged on POD 1. There were no complications in the prospectively reviewed outpatient group who were discharged on POD 0.

Conclusions: Outpatient minimally invasive craniosynostosis repair is safe in appropriately selected patients. It is feasible to develop criteria for same-day discharge.

Objective: Implant infection is a significant contributor to morbidity and mortality for children with hydrocephalus and other neurosurgical conditions requiring shunts and other neurosurgical implants. To standardize pediatric neurosurgical care and minimize the risk of infections related to implanted shunts and neurosurgical devices, the authors introduced a 23-point checklist for all pediatric neurosurgical implant procedures at Stanford University in March 2019. This protocol minimizes operating room traffic and standardizes sterile technique, preparation, and antibiotic use.

Methods: Prospective quality initiative data obtained for surgeries completed in the checklist era (March 2019-May 2023, follow-up through November 2023) were compared with retrospective chart review data of the prechecklist era cases (March 2016-February 2019). Checklist compliance was monitored by individual checklist elements. Infections of shunts or implanted devices within 6 months of the surgical date were identified prospectively and by routine audits. Infection rates in the prechecklist era were compared to infection rates in the checklist era using Fisher's exact test.

Results: There were 4 infections in the 356 prechecklist era surgeries (1.1% infection rate, 95% CI 0.03%-2.21%) and 9 infections in the 630 postchecklist era surgeries (1.4% infection rate, 95% CI 0.50%-2.34%), resulting in an overall implant infection rate of 1.3% (95% CI 0.60%-2.02%). Infection rates did not significantly change during a period of significant case volume growth, with an absolute risk difference of 0.3% (95% CI -1.13% to 1.73%) and relative risk of 1.3 (95% CI 0.39-4.12, p > 0.999). Shunt infections were the most common infections observed. One baclofen pump infection was observed and no vagus nerve stimulators, deep brain stimulators, generators, Ommaya reservoirs, neonatal reservoirs, or cranioplasties were infected in the study period. The majority of shunt infections occurred in infants younger than 6 months of age.

Conclusions: Adherence to a strict implant protocol can ensure very low rates of infections for pediatric shunts and neurosurgical implants. This study found a lower shunt infection rate than the national pediatric benchmark-the 5.1% infection rate observed within the Hydrocephalus Clinical Research Network (HCRN) and the 3.9% infection rate observed within the HCRN quality network, to which the authors' institution contributes data. Despite surgeon and staff turnover, high overall institutional checklist compliance and consistently low infection rates were observed over time.

Objective: The authors aimed to evaluate the neuropsychological outcome of pediatric victims of traumatic brain injury (TBI) and its association with trauma severity and MRI findings, and to investigate health-related quality of life (HRQOL) of patients and their families after trauma.

Methods: This was a prospective cohort study of pediatric patients (≤ 16 years) who sustained TBI and were admitted to the emergency department of a tertiary care university hospital in Brazil from June 2018 to December 2019. Trauma severity was determined by Glasgow Coma Scale (GCS), neurological outcome by King's Outcome Scale for Childhood Head Injury (KOSCHI), neuropsychological outcome by the Wechsler Intelligence Scale for Children-Fourth Edition, and HRQOL by the Pediatric Quality of Life Inventory 4.0. Patients also underwent MRI examinations. Neurological outcome was assessed twice at a median of 6 months and 13 months after trauma. Neuropsychological and HRQOL assessment and MRI were performed at a median of 13 months after trauma.

Results: Thirty-seven patients were included. According to the neurological outcome categorized by KOSCHI, 25 (67.6%) patients made a good recovery and 12 (32.4%) had a disability. The neurological status did not change between the two assessments. Patients in the disability group had lower GCS scores (median 11 vs 15, p = 0.0006) and lower median values of full-scale intelligence quotient (67 vs 86, p = 0.0002), perceptual reasoning index (75 vs 92, p = 0.03), verbal comprehension index (72 vs 84, p = 0.02), working memory index (74 vs 88, p = 0.003), and processing speed index (68 vs 86, p = 0.01). The presence of MRI alterations was associated with TBI severity (median GCS score 7 vs 15, p < 0.0001). Mean, axial, and radial diffusivity were higher, and fractional anisotropy was lower in patients with TBI compared with controls. HRQOL was worse in the disability group.

Conclusions: Pediatric patients sustaining TBI with a KOSCHI outcome classified as having a disability had poorer neuropsychological testing performance and worse HRQOL compared with patients with a good recovery. MRI metrics abnormalities suggest diffuse white matter disruption associated with pediatric TBI.

Objective: Surgical fixation for occipitocervical instability can be challenging due to limitations in occipital instrumentation that include prominent hardware, limited fixation points on the occiput, and the risk of intracranial injury. Occipital instrumentation is particularly difficult in pediatric patients with thinner skull osteology and smaller bony surface area. Transarticular atlantooccipital and occipital condyle screw placement are newer techniques that have been described as alternative strategies for occipitocervical fixation. Cadaveric studies have demonstrated the feasibility and biomechanical equivalence to traditional plating systems for both techniques, however their clinical application has been limited. The authors present the largest case series of pediatric patients who underwent either transarticular atlantooccipital or direct occipital condyle screw fixation for the treatment of occipital cervical instability. The authors report their early postoperative outcomes, fusion rates, and feasibility of the surgical procedure in pediatric patients.

Methods: Three patients underwent transarticular atlantooccipital screw fixation, and 3 patients underwent direct occipital condyle screw fixation. Clinical presentation, complications, fusion rates, and postoperative outcomes were reviewed.

Results: The age range was 2 to 20 years old. Occipitocervical instability was secondary to congenital skeletal dysplasia and neuromuscular scoliosis. Presenting symptoms included dysphagia, dysphonia, headaches, and neck pain. All patients underwent instrumentation guided by neuronavigation. There were no intra- or postoperative complications, and all patients demonstrated evidence of fusion with an average (range) follow-up of 24.1 (15-36) months. The authors observed an excellent fusion rate with low morbidity.

Conclusion: Transarticular atlantooccipital and direct occipital condyle screw fixation are alternative techniques to occipital plate fixation. These novel techniques can be performed safely in pediatric patients and provide adequate fixation for successful arthrodesis.