Journal of neurosurgery. Pediatrics最新文献

Objective: Chiari decompression is the definitive treatment for symptomatic Chiari malformation type I (CM-I). In part 1 of this two-part series, operative management and perioperative complications were discussed. Presented here are the symptomatic outcomes from a large cohort of pediatric patients who underwent Chiari decompression.

Methods: A departmental operative database was queried for patients who underwent Chiari decompression between 1992 and 2021; 510 patients were identified. Data regarding patient outcomes were collected.

Results: Seventy-three percent of patients with exertional suboccipital headaches and 67% of patients with central sleep apnea reported improvement, while 77% of patients with syringomyelia and postoperative imaging exhibited a reduction in the length or diameter of the syrinx after decompressive surgery. Among the 32 patients with syringomyelia as an indication for repeat surgery, 18 (56%) had structural causes of syrinx formation or persistence identified on preoperative imaging and all had structural causes identified intraoperatively. Postoperative complications requiring readmission after revision surgery occurred in 18% of patients, slightly higher than with primary Chiari decompression (11%), although the most common complications remained CSF leakage and aseptic meningitis. Nine percent of patients required repeat decompression due to worsening or new Chiari symptoms or persistent syringomyelia, with a median time to repeat surgery of 17 months.

Conclusions: The authors present an institutional experience with Chiari decompression. Both initial and revision decompressive surgeries were predominantly performed as posterior fossa decompression with duraplasty and tonsillopexy and demonstrated high rates of postoperative symptomatic and radiographic improvement. Revision surgery was necessary in < 10% of patients, most commonly due to syrinx persistence or formation secondary to intradural scarring from the initial surgery.

Objective: Vagus nerve stimulation (VNS) is an established adjunctive therapy for drug-resistant epilepsy (DRE). However, evidence regarding its efficacy in children with structural and nonstructural etiologies of epilepsy remains limited. Herein, the authors aimed to explore the effectiveness of VNS in patients with either etiology.

Methods: In this retrospective single-center study, authors evaluated children (ages 2-18 years) with DRE due to nonlesional monogenic epilepsies (MEs) or malformations of cortical development (MCDs) who had undergone VNS device implantation between 2008 and 2024 and had ≥ 6 months of follow-up. Patients with tuberous sclerosis complex were excluded. Clinical, genetic, neuroimaging, VNS programming, and outcome data were extracted from medical records. The primary outcome was the responder rate, defined as > 50% seizure reduction from baseline at 6 months, 12 months, and the last follow-up, in the two groups.

Results: Of 336 VNS device recipients, 64 children with ME (n = 44) or MCD (n = 20) met the study inclusion criteria. The median follow-up was 3.5-4.0 years. The responder rate in the ME versus MCD group at 6 months, 1 year, and the last follow-up was 38.6% versus 42.1% (p = 0.77), 45.7% versus 44.4% (p = 0.46), and 47.6% versus 63.2% (p = 0.64), respectively. The SCN1A-related Dravet syndrome (SCN1A-DS) subgroup (n = 12) had a responder rate (50.0%) comparable to that of the non-SCN1A-DS ME group (43.7%) at the last follow-up. The frequency of status epilepticus decreased significantly in both groups (p = 0.03). VNS was well tolerated, with mild to moderate side effects reported in < 5% patients. No clinical variable, including age at seizure onset, epilepsy duration, or age at VNS device implantation predicted seizure outcomes.

Conclusions: VNS therapy was noted to have a similar responder rate in children with DRE due to MEs or MCDs. Both groups experienced meaningful benefits, with a small proportion of patients experiencing mild side effects.

Objective: Socioeconomic factors such as income and community type have been shown to correlate with outcomes in adult neuro-oncological care. In pediatric populations, fewer conclusions have been drawn on the effects of factors such as race, sex, and insurance status on survival due to the relative rarity of such cases. This analysis used the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) program database to evaluate differences in outcomes for factors influencing survival in both pediatric and adult CNS malignancies.

Methods: From the SEER research November 2020 (1975-2018) dataset, 29,865 patients with malignant CNS tumors (identified by International Classification of Diseases for Oncology, 3rd ed., code) diagnosed between 1990 and 2018 and directly causing patient deaths were selected. Data were collected on age at diagnosis, sex, race, median household income (in 2019 US dollars), community type (rural-urban continuum), and survival (in months). This cohort was separated into pediatric (ages 0-19 years, n = 1509) and adult (ages ≥ 20 years, n = 28,356) subgroups, and independent survival analysis was conducted by income, community type, sex, and race. Kaplan-Meier curves were visualized for each comparison.

Results: Log-rank analysis of survival in the pediatric cohort showed greater mortality rates in females (p = 0.0224). The same analysis in the adult cohort showed greater mortality rates in lower income groups, more rural community settings, females, and patients categorized as White (p < 0.0001 for all comparisons).

Conclusions: Unlike in the adult population, income, community setting, and race were not associated with significant differences in survival in the pediatric population, prompting further investigation into healthcare delivery differences between pediatric and adult populations. Further study should focus on the cause of these discrepancies to improve equity in neurosurgical care.

Objective: Cranial fixation is essential to many neurosurgical procedures for ensuring patient head immobilization and optimal surgical outcomes. However, complications such as skull fractures, scalp lacerations, and clamp slippage remain underreported and understudied in the pediatric patient population. This study aimed to identify pediatric neurosurgeon perspectives on skull clamping risks and protocols.

Methods: An anonymous 19-question survey was distributed to 295 pediatric neurosurgeons with an email address registered with the American Board of Pediatric Neurological Surgery and/or the American Society of Pediatric Neurosurgeons. Questions addressed clamping practices, injury experiences, perceptions of current safeguards, and attitude toward injury detection and prevention systems.

Results: Of the 295 pediatric neurosurgeons contacted, 64 (21.7%) responded, and 48 (16.3%) completed the full survey. The median age at which respondents reported clamping pediatric skulls without additional support was 2 years, and the median minimum clamping pressure was reported to be 40 pounds. Surgeons reported substantial variability in clamping practices, particularly in minimum and maximum pressure thresholds, often citing patient-specific skull thickness as a key determinant. Injury reporting revealed that 31.5% of respondents experienced at least one skull fracture annually, 48.1% reported at least one scalp laceration annually, and annual slippage or clamp loosening was reported by > 70% of respondents. Additionally, surgeons who provided preoperative counseling regarding clamping risks were more likely to experience related risks. Overall, these surgeons expressed dissatisfaction with existing clamping technologies, citing inadequate safeguards for both injury prevention and detection. Seventy-three percent of surgeons expressed eagerness to adopt technologies capable of mitigating risks, and 65% expressed eagerness to adopt technologies capable of injury detection. These findings highlight the burden of clamp-related complications in pediatric neurosurgery and an interest in the development of safer, more reliable cranial fixation systems.

Conclusions: There are substantial challenges associated with the utilization of cranial fixation in pediatric patients. Exploring real-time intraoperative monitoring technologies may offer a promising avenue for improving patient safety and surgical outcomes.

Objective: Drug-resistant epilepsy (DRE) can arise early in life due to multiple structural abnormalities. In patients with the disorder, seizures can be difficult to control and can significantly impair neurodevelopment. Hemispherectomy is well described as a treatment for refractory hemispheric-onset epilepsy, but its use in infants has been limited due to concerns over surgical risk and physiological tolerance. The aim of this review was to summarize an institutional experience with hemispherectomy in infants to demonstrate the safety and efficacy of the technique.

Methods: A retrospective chart review was conducted to identify children younger than 1 year of age who had undergone a modified functional hemispherectomy combining elements of both hemispherotomy and anatomical resection to treat DRE at Seattle Children's Hospital from 2000 to 2024. The primary outcome was favorable seizure outcome, defined as Engel class I-II at the 1-year follow-up. Secondary outcomes included perioperative complications, development of postoperative hydrocephalus, need for long-term nutritional supplementation, and changes in the anti-seizure medication burden.

Results: Twenty-one children who had undergone functional hemispherectomy were identified, revealing a mean age of 6.2 months (range 0.93-12.3 months) at surgery. The most common etiology was hemimegalencephaly (n = 11, 52%). No serious cardiac, pulmonary, or coagulopathic adverse events occurred, although complications included asymptomatic cerebral venous sinus thrombosis (n = 1, 5%), postoperative hydrocephalus (n = 3, 14%), and electrolyte derangements (n = 1, 5%). The mean hospital stay was 12.5 days (range 6-34 days). Hydrocephalus developed > 12 months postoperatively in 2 (67%) of the 3 patients with this complication. The mean estimated blood loss was 345.8 mL (150-700 mL), and all patients received a transfusion during their surgery with a mean transfusion volume of 450.2 mL (60.4 mL/kg). Seizure outcomes were uniformly assessed at the last clinical follow-up, with a mean follow-up duration of 91.9 months (range 6.1-261.6 months). A favorable seizure outcome (Engel class I-II) was attained in 20 patients (95%), with an Engel class I outcome in 19 patients (90%).

Conclusions: An institutional experience demonstrated that functional hemispherectomy is a safe and well-tolerated procedure in infants and offers excellent seizure control outcomes in hemispheric-onset epilepsies. The surgical technique, a focus on minimizing operative blood loss, and multidisciplinary care of these patients are critical elements in ensuring the safety and success of this procedure. Future studies are needed to better characterize long-term functional and neurodevelopmental outcomes in this age group.

Objective: The choice of surgical technique for decompression of the cervicomedullary junction remains controversial. The authors report their experience with using subpial tonsillectomy for Chiari malformation with and without associated syringohydromyelia.

Methods: Children from two institutions who underwent the subpial tonsillar resection technique for decompression of the craniocervical junction from 2014 to 2024 were included. Demographic information, relevant presurgical symptoms, surgical history, perioperative radiographic characteristics, postoperative outcomes, complications, reoperations, and 90-day readmissions were collected. Two outcomes were assessed at last follow-up: 1) status of presenting symptom(s); and 2) radiographic status of syringohydromyelia or isolated presyrinx edema, if present preoperatively.

Results: A total of 109 patients were identified with a mean age of 10.8 years. Seventy-eight (71.6%) of these patients presented with headaches, and 23 (21.1%) had medullary or lower cranial nerve dysfunction. Radiographically, 86 (78.9%) patients had Chiari type 1 and 23 (21.1%) had Chiari type 1.5. The mean (range) preoperative cerebellar tonsillar herniation was 14.0 (4.5-40) mm, and 49 (45%) patients had syringohydromyelia or presyrinx edema. The mean operative time was 152 minutes, and the mean length of stay was 3.49 days. Fourteen patients were readmitted within 90 days of surgery, 9 within the first 30 days, and 5 after 30 days. Of these 14 readmissions, 8 (57%) were for reasons attributable to the index operation. Over a mean follow-up of 3.70 years, headaches resolved or improved in 68 of 78 (87.2%) patients, and 18 of 23 (78.3%) patients with brainstem or lower cranial nerve dysfunction demonstrated improvement. Of the patients with syringohydromyelia who had follow-up (n = 45), 39 (86.7%) had complete resolution of their syringohydromyelia (n = 16) or > 50% reduction in diameter of the syrinx (n = 23). One patient was deemed a treatment failure, eventually needing a syringopleural shunt.

Conclusions: Subpial tonsillar resection for Chiari malformations is a safe and effective method to maximally decompress the craniocervical junction and immediately reestablish pulsatile cerebrospinal fluid flow out of the fourth ventricle. Most patients will experience improvement or resolution of their associated symptoms and significant reduction (> 50% diameter reduction) or resolution of their syringohydromyelia.