Journal of neurosurgery. Pediatrics最新文献

Objective: Despite improvements in rates of hydrocephalus with prenatal repair of myelomeningocele (MMC), a significant number of children still require CSF diversion. While previous studies have focused on uncovering predictors of future hydrocephalus, differences in timing and presentation of MMC-associated hydrocephalus following pre- and postnatal surgery are not well-characterized. This study aimed to determine how age, head size, and ventricle size differ at hydrocephalus presentation in patients treated with pre- and postnatal surgery for MMC.

Methods: The Hydrocephalus Clinical Research Network Core Data Project was queried to identify patients with MMC who underwent pre- or postnatal repair and required permanent CSF diversion-by shunting or endoscopic third ventriculostomy, with or without choroid plexus cauterization-between April 2008 and June 2024. The primary variable of interest was chronological age at the primary CSF diversion procedure. Secondary variables of interest included absolute head circumference (HC), HC percentile/z-score, and frontal-occipital horn ratio (FOHR).

Results: One thousand forty-four patients from 14 centers were included: 125 (12%) underwent prenatal MMC repair and 919 (88%) underwent postnatal MMC closure. The median patient age at primary CSF diversion procedure was 4.1 months in the prenatal MMC repair cohort versus 0.6 months in the postnatal closure cohort (p < 0.001). Absolute HC (45.0 vs 38.0 cm, p < 0.001), HC percentile (> 99.9 vs 97.0, p < 0.001), HC z-score (3.4 vs 1.9, p < 0.001), and FOHR (0.60 vs 0.56, p < 0.001) were greater in the pre- versus postnatal surgery cohort. Linear mixed models adjusting for treatment center and time period in which CSF diversion was performed revealed that patients with MMC treated prenatally underwent CSF diversion 3.02 (95% CI 0.39-5.64) months later (p = 0.024) and had greater HC z-scores (+1.27, 95% CI 0.81-1.73; p < 0.001), but similar FOHRs (-1.08, 95% CI -5.07 to 2.90; p = 0.593) compared to those treated postnatally.

Conclusions: Patients developing hydrocephalus following prenatal MMC repair undergo CSF diversion later and have larger heads than those developing hydrocephalus following postnatal MMC closure; however, FOHRs are similar between groups. The disparity between HC and FOHR may suggest the presence of increased overall brain parenchymal and/or extra-axial volume in children receiving prenatal MMC repair, although further study is required. These findings may inform prenatal counseling and follow-up timing for patients with MMC.

Objective: Mechanisms and patterns of severe traumatic brain injury (TBI) in infants and toddlers are different from adults and even from older children, but there is no taxonomy of injuries that can provide a basis for individualized care. The current project was undertaken as a proof of concept to demonstrate that a taxonomy can be constructed with clinical correlations.

Methods: Data were taken from the American College of Surgeons Trauma Quality Improvement Program for the years 2014 through 2022. Inclusion criteria were an age < 2 years and Abbreviated Injury Scale head score of 4, 5, or 6. Latent class analysis (LCA) was performed based on covariates reflecting socioeconomic status, access to definitive care, mechanism of injury, severity of injury, and pathology.

Results: There were 3735 cases meeting study inclusion criteria. Overall mortality among cases with a known outcome was 19%, and 70% of discharges were routine. Intracranial monitoring was coded for 28% of cases. An LCA model with 4 classes was studied. The mortality rates of the 4 classes were highly distinct at 2%, 13%, 37%, and 65%, respectively. The routine discharge rates were similarly distinct at 92%, 66%, 41%, and 12%, respectively. The rates of intracranial monitoring varied much more narrowly between 26% and 39%.

Conclusions: A scheme for classifying injuries based on LCA had powerful associations with outcomes and proves that a clinically meaningful taxonomy of severe TBI in the very young is possible. The fact that 25% of cases with the best prognosis underwent intracranial monitoring and only 37% of cases with the worst prognosis did so demonstrates the need for an evidence-based taxonomy to guide individualized care. Large-scale prospective study may define categories that are conceptually accessible rather than latent and that guide therapies as well as predict outcomes.

Craniopagus twins, particularly the total vertical type II variant, represent one of the rarest and most complex congenital malformations, with significant surgical challenges. This technical note presents the successful staged separation of craniopagus twins at 3 years 9 months of age, the oldest known to be separated to date. The twins shared a prominent circumferential venous sinus, requiring a novel surgical strategy. Advanced imaging, 3D modeling, and virtual reality planning in the metaverse-between Brazil and the United Kingdom-guided the 7 staged surgeries. Initially, partial separations focused on the more vulnerable twin. However, arteriographic evidence of venous dependency led to a paradigm shift, in which the authors performed the final stages on the other twin to preserve critical venous outflow. This innovative approach allowed division of the shared venous sinus without neurological compromise. The final separation, completed in 2 stages over 31 hours, resulted in successful independent circulations and recovery. This case highlights the importance of individualized surgical planning, collaborative international simulation, and the potential for adapting separation protocols based on venous architecture.

Objective: Electrode placement using robot-assisted stereoelectroencephalography (SEEG) has been proven a safe and accurate technique in children. As its use increases, understanding the impact of registration methods and patient-specific factors on placement accuracy is crucial. The aim of this study was to compare 4 registration methods and to evaluate factors associated with lead placement error.

Methods: This retrospective case series included pediatric patients who underwent robot-assisted SEEG from January 2019 to April 2022 at a single institution. Placement accuracy was assessed at both the inner skull table and the prespecified target using 4 registration techniques: 1) laser-based registration with a Mayfield skull clamp (laser), 2) a Leksell frame with bone fiducials (bone fiducials), 3) a Leksell frame with pins plus one bone fiducial (pins+fiducial), and 4) a frame-based registration with etched frame (frame-based). Accuracy differences were analyzed using Kruskal-Wallis and Wilcoxon tests. A stepwise multivariate linear regression model was used to evaluate predictors of error.

Results: Overall, 231 electrodes were placed in 22 patients (median age 15 years). The median error at the inner skull table was lowest with the pins+fiducial (0.6 mm) technique and highest with the laser (1.7 mm) technique. The target error was also lowest with pins+fiducial (1.1 mm) technique and highest with the laser (2.04 mm) technique. All group differences were statistically significant (p < 0.0001). Younger age (p = 0.0161) and increased bone thickness (p = 0.0304) were independently associated with error at the target and inner skull table, respectively. No clinical complications occurred, including hemorrhage, infection, or electrode malposition.

Conclusions: The registration technique used significantly affects robot-assisted SEEG accuracy in children. The use of frame-based approaches, especially using pins and a single fiducial, yielded the highest accuracy. Additional caution should be exercised in younger patients and with trajectories through thicker bone.

Objective: Posterior fossa decompression is the main surgical treatment for Chiari malformation type I (CM-I). The authors present findings on patient presentation, surgical methods, and postoperative complications from a large cohort of pediatric patients with the aim of utilizing this institutional experience to guide evidence-based management of pediatric CM-I.

Methods: A departmental operative database was queried for patients who underwent a posterior fossa decompression for CM-I between 1992 and 2021. The charts of identified patients were reviewed. Data regarding demographics, presentation, operative details, and complications were collected.

Results: A total of 510 patients were identified. The mean patient age was 10.2 ± 5.2 years, and 57% of patients were female. The most common presenting clinical symptoms or associated signs were exertional suboccipital headaches (65%), syringomyelia (55%), and scoliosis (18%). The mean cerebellar tonsillar descent was 14.2 ± 6.4 mm below the foramen magnum. At surgery, 20% of the patients with a preoperative syrinx had documented arachnoid veils obstructing the outflow tract of the fourth ventricle. Operatively, 99% of patients underwent cervical laminectomy; of these laminectomies, 89% were C1 only. Ninety-seven percent of all patients underwent suboccipital craniectomy with expansile duraplasty (PFDD), while 3% underwent bone-only decompression (PFD). Eighty-seven percent of the patients with PFDD received tonsillopexy and/or tonsillar resection. The median postoperative hospital stay was 2 days for PFD patients and 3 days for PFDD patients (p < 0.01). Postoperative complications developed in 12% of cases, with CSF leakage (5%) and aseptic meningitis (4%) being most common. CSF leakage and aseptic meningitis were also the most common reasons for early readmission after surgery. There were no intraoperative or postoperative deaths.

Conclusions: The authors demonstrate an institutional preference for PFDD and tonsillopexy. Exertional suboccipital pain and syringomyelia were the most common indications for surgery. Complications tended to be more common with PFDD. There was a significant difference in postoperative length of stay between patients who underwent PFD (median 2 days) versus PFDD (median 3 days). CSF leakage and aseptic meningitis were the most common reasons for readmission.

Objective: Patients with ventriculoperitoneal shunts have a lifelong risk of requiring revision procedures that may be time critical. Many of these patients reside distantly from neurosurgical services; however, the influence of this key sociodemographic factor on the outcomes of patients with ventriculoperitoneal shunts has not been previously studied. The Australasian Shunt Registry provides a rare opportunity to study the association of rurality with ventriculoperitoneal shunt outcomes across a large landmass with a broadly distributed population.

Methods: Data were extracted from the Australasian Shunt Registry for all patients aged ≤ 25 years who underwent first-time ventriculoperitoneal shunt insertion from December 2016 to October 2024. The patient's postcode of residence was matched to the corresponding Modified Monash Model (MM) rurality index for that region. The metropolitan cohort was defined as all patients with MM 1, and the rural/regional cohort included all patients with MM ≥ 2. The primary outcome of interest was time from ventriculoperitoneal shunt insertion to first shunt revision (shunt survival).

Results: At the conclusion of the study period, the Australasian Shunt Registry contained records for 5023 unique patients. After application of exclusion criteria, the resulting sample included 930 patients. The majority of these patients reside in a metropolitan MM1 area (617/930 [66%]). The cohorts were similar for demographic and clinical variables. The estimated mean shunt survival was 5.04 (95% CI 4.64-5.43) years for rural patients and 5.69 (95% CI 5.40-5.98) years for metropolitan patients (Wilcoxon test, p = 0.01). That effect remained statistically significant in the Cox proportional hazards model that included all variables that were significant on univariate analysis: a metropolitan address exerted a protective effect on overall shunt survival (HR 0.75, 95% CI 0.59-0.95, p = 0.02).

Conclusions: Rural/regional patients with ventriculoperitoneal shunts experienced reduced overall shunt survival when compared to metropolitan patients.

Objective: Chiari malformation (CM) involves a broad disease spectrum, where rare complex CM cases can be associated with craniocervical junction (CVJ) instability and require occipitocervical fusion. However, the natural progression of CVJ alignment in the general CM type I and 1.5 populations treated with posterior fossa decompression (PFD) remains insufficiently characterized. The authors aimed to compare CVJ alignment changes in patients who underwent PFD versus patients with CM who did not undergo surgery.

Methods: The authors conducted a retrospective cohort study at their institution of all patients diagnosed with CM I and 1.5 from 2000 to early 2023. Demographic, clinical, and surgical data were collected, along with preoperative and postoperative MRI measurements, including tonsillar herniation, brainstem descent, clivoaxial angle (CXA), and condylar-C2 sagittal vertical alignment (C-C2SVA).

Results: A total of 241 patients were included, with 201 undergoing PFD and 40 managed conservatively (controls). No significant differences were observed between groups in age at diagnosis, sex, or genetic diagnoses. In the PFD group, 55% underwent duraplasty and 45% underwent bone-only decompression. Baseline craniocervical alignment measurements showed a lower CXA in the PFD group (144.4° ± 13.4°) compared to controls (148.5° ± 14.2°) (p = 0.04) but no difference in C-C2SVA. Changes over time showed a small but significant decrease in CXA at < 1 year after surgery in the PFD group (-2.7°) compared to controls (-2.0°) (p = 0.008), but no differences were noted at 1-2 years. No differences in C-C2SVA were observed over time in either group.

Conclusions: PFD does not significantly impact craniocervical alignment or increase the risk of occipitocervical fusion in CM I and 1.5 patients. These findings support PFD as a safe first-line treatment, even in complex CM cases, and provide important information for patient education regarding the risks of surgery.

Objective: Use of an irrigating external intracranial drainage system has been an active and promising area of investigation in adult patients with intraventricular hemorrhage, ventriculitis, and chronic subdural hematoma. The objective of this study was to report on the safety and feasibility of an irrigating external intracranial drainage system for use in children.

Methods: Retrospective chart review was undertaken of the medical records of children who required an irrigating external intracranial drain (EID) at two children's hospitals for clearance of infection or blood from the ventricular system or extra-axial space. Irrigation parameters, CSF study results, adverse events during and after the course of irrigation, and ventricular peritoneal shunt outcomes up to 3 months postoperatively were assessed.

Results: Twelve patients younger than 18 years of age were treated between September 2023 and May 2025. Four patients were younger than 1 year of age (mean ± SD 4.72 ± 3.81 months), and 8 patients were between 4 and 18 years (mean 11.52 ± 4.33 years). Four patients required intracranial drainage for ventriculitis, 1 for intraventricular hemorrhage, 4 for postoperative clearance of blood following hemispherectomy or tumor resection, and 3 for subdural hematomas. The mean duration of irrigation was 6.45 ± 5.25 days. One patient had an adverse event during irrigation, which was a clinical seizure. Irrigation was stopped and the patient was treated with levetiracetam with no further seizures. This was the only patient who received irrigation using normal saline with vancomycin. All other patients received irrigation with lactated Ringer's solution without antibiotics. Seven patients had a ventriculoperitoneal shunt after treatment-5 of whom had a shunt on presentation-of which 2 required revisions for valve replacement within 3 months. All patients with ventricular pathology had stable ventricular configuration on follow-up imaging.

Conclusions: The use of an irrigating EID has potential utility for clearance of intracranial purulence and blood products in children. This is the first published series to delineate the safety and feasibility of this system in a group of children, 4 of whom were infants younger than 1 year of age.