Journal of neurosurgery. Pediatrics最新文献

Objective: Although total corpus callosotomy (TCC) is attempted for better seizure control in patients with drug-resistant generalized seizures and intellectual disability, some surgeons may avoid TCC for fear of postoperative neurocognitive sequelae. The present study aimed to identify the predictors of favorable or unfavorable seizure outcomes and evaluate the postoperative changes in cognitive function after TCC.

Methods: The authors retrospectively reviewed the clinical records of consecutive patients who underwent TCC to palliate generalized seizures between 2010 and 2022 in the Department of Neurosurgery, Hiroshima University Hospital and had a minimum follow-up of 2 years. The inclusion criteria were 1) age at surgery ≥ 5 years, 2) preoperative IQ score ≤ 50; and 3) 1- or 2-stage TCC. A multivariable logistic regression analysis was performed to determine the predictors of seizure outcome after TCC. The changes in IQ were compared between the favorable and unfavorable seizure outcome groups.

Results: The authors included 40 patients. Favorable seizure outcomes (> 75% reduction in seizure frequency, severity, or both) were obtained after TCC in 29 (72.5%) and 23 (57.5%) patients at 1 year and 2 years postoperatively, respectively. Multivariable logistic regression analysis revealed that interictal electroencephalography showing multiple independent spike foci (MISF) was an independent predictive factor of an unfavorable seizure outcome at 2 years after TCC (p = 0.0270; OR 6.48, 95% CI 1.24-33.96). ΔIQ from preoperatively to 1 year postoperatively was significantly higher in the favorable seizure outcome group (median 0, range -1 to +14) than in the unfavorable seizure outcome group (median -6, range -14 to -5; p = 0.0042). From 1 to 2 years postoperatively, there was no decline in IQ in either group and no difference in ΔIQ between the two groups.

Conclusions: The authors found that MISF is a predictor of unfavorable seizure outcome after TCC. Unfavorable seizure outcomes are associated with postoperative aggravation in cognitive function. The cognitive deterioration may be due in part to the surgical intervention itself. Thus, appropriate selection of the candidates for TCC is desired.

Objective: The authors provide an updated analysis of inpatient healthcare utilization, associated costs, and mortality trends for pediatric hydrocephalus in the US from 2006 to 2019. The goals were to describe patient, hospital, and hospitalization characteristics and determine factors associated with mortality.

Methods: This cross-sectional study used 2006, 2009, 2012, 2016, and 2019 data from the Healthcare Cost and Utilization Project Kids' Inpatient Database, which collects nationally representative weighted data samples of pediatric hospital discharges. Admissions related to hydrocephalus were categorized as being associated with permanent cerebrospinal fluid (CSF) diversion (including CSF shunt management and endoscopic third ventriculostomy [ETV] with or without choroid plexus cauterization [CPC]) or unrelated to permanent CSF diversion.

Results: Each year, there were approximately 30,000-32,000 hydrocephalus-related admissions, resulting in 331,000-526,000 hospital days and US$3.4-5.0 billion charges, for pediatric patients. In 2019, hydrocephalus accounted for 0.5% of all pediatric hospital admissions, 1.4% of all pediatric hospital days, and 2.4% of all pediatric hospital charges in the US. The median (IQR) length of stay across all hydrocephalus-related admissions decreased from 4 (2-15) days in 2006 to 3 (2-9) days in 2019. CSF shunt-related admissions decreased from 11,111 in 2006 to 7959 in 2016; notably, admissions for CSF shunt malfunctions/revisions decreased over time (12,327 in 2006 to 5960 in 2019). In 2019, hospital stays were shorter (4.99 vs 6.69 days) and charges were lower (US$108 million vs US$128 million) in patients who underwent ETV or ETV+CPC compared to those who had initial shunt placement, respectively. However, these unadjusted differences likely reflect baseline patient selection rather than inherent procedural superiority. Patients admitted for periventricular-intraventricular hemorrhage of prematurity (pIVH) had longer hospital stays (p < 0.001) and higher mean costs than others. Compared with survivors, children who died were younger, had pIVH, had a birth-related admission, were self-paying, and were admitted to a nonchildren's hospital (p < 0.05).

Conclusions: Pediatric hydrocephalus continues to pose a heavy burden in the US. Despite advancements in management, it remains associated with high costs, significant hospital utilization, and substantial morbidity and mortality. ETV admissions were associated with shorter hospital stays and lower costs, and pIVH was associated with particularly high resource utilization and markedly higher in-hospital mortality. Future efforts should focus on reducing mortality and improving care delivery for high-risk subgroups, particularly those with pIVH and birth-related etiologies.

Objective: Rasmussen's encephalitis (RE) affects the structure and function of one cerebral hemisphere, typically during childhood. Hemispherotomy is a surgical treatment that functionally disconnects the affected hemisphere. The cognitive consequences of surgery and the potential for postoperative recovery remain poorly understood, however, due to their complex and multifactorial nature. This study aimed to examine the long-term cognitive outcomes of patients with RE following hemispherotomy and identify the clinical factors influencing recovery.

Methods: Forty-four patients who underwent childhood hemispherotomy for RE (28 girls, 23 with left RE) were included in this retrospective study. Neuropsychological assessments were conducted during postoperative follow-up, and verbal (VIQ) and nonverbal (performance IQ [PIQ]) IQ scores from the most recent evaluation were analyzed. The impact of age at seizure onset (ASO), age at hemispherotomy, preoperative epilepsy duration, side of hemispherotomy, age at neuropsychological evaluation, and postoperative follow-up duration on intellectual efficiency scores was assessed using partial least squares analysis.

Results: The mean ASO was 5.9 years, the mean age at hemispherotomy was 9.6 years, the mean epilepsy duration was 3.7 years, and the mean postoperative follow-up duration was 9.5 years. After hemispherotomy, 91% of patients were seizure free (Engel class I), and 86% were no longer receiving antiepileptic medication. Patients who underwent hemispherotomy of the language-dominant hemisphere (Hdom) had lower VIQ scores but higher PIQ scores compared to those with hemispherotomy of the nondominant hemisphere (Hnondom). Results showed that higher VIQ was significantly associated with several clinical factors, including a shorter epilepsy duration before surgery, a younger age at surgery, and hemispherotomy of the nondominant hemisphere for language. In contrast, no clear link was found between clinical variables and PIQ.

Conclusions: In RE, early hemispherotomy performed soon after disease onset appears to be associated with better long-term intellectual outcomes. Verbal functions can be recovered following hemispherotomy of the dominant hemisphere, highlighting the preferential reorganization of language in postoperative cognitive recovery. These findings underscore the critical importance of early surgical decision-making in optimizing patient care and maximizing postoperative recovery.

Objective: The cognitive outcome of patients with sagittal synostosis (SS) has been studied previously; however, patients with developmental or behavioral problems have often been excluded, resulting in an incomplete picture. In this retrospective study, the authors evaluate the cognitive profile of patients with both SS and developmental or behavioral problems, as well as its relationship with the type and timing of the surgery performed.

Methods: All patients born between 2000 and 2018 at a single institution who had a diagnosis of SS and underwent surgery were included in the study. Patients were treated with one of the following surgical techniques: spring-assisted correction, extended strip craniotomy, or frontobiparietal remodeling. Subsequently, if parents had concerns about their child's developmental or behavioral functioning, the child underwent psychological or psychiatric diagnostic assessment. The Wechsler Preschool and Primary Scale of Intelligence or the Wechsler Intelligence Scale for Children were used to evaluate cognitive profiles. Full Scale IQ (FSIQ), Verbal IQ (VIQ), Performance IQ (PIQ), and index scales were used to measure cognitive profiles.

Results: Among the 534 patients with SS who presented to the outpatient clinic after surgery, 99 (18.5%) had parents who expressed concerns about their child's developmental or behavioral functioning. Seventy-eight patients (14.6%) underwent psychological or psychiatric assessment. The mean FSIQ, VIQ, and PIQ scores were 96.29 ± 13.66, 98.21 ± 14.51, and 96.10 ± 12.98, respectively; both the mean FSIQ and PIQ were lower than the norm (p = 0.02). Patients with SS had lower Visual Spatial Index scores than the norm (91.82 ± 16.80, p = 0.005). The type and timing of surgery were not related to the cognitive profile of patients with SS. Results were adjusted for sex, age at surgery, and parental education level.

Conclusions: Of the 534 patients with SS, 99 had parents who voiced concerns about their child's development or behavior. The intellectual ability of patients with both SS and developmental or behavioral issues was slightly lower than the norm. Surgical technique and timing did not affect the cognitive profile.

Objective: Cerliponase alfa is an enzyme replacement treatment for neuronal ceroid lipofuscinosis type 2 (CLN2), administered via biweekly intracerebroventricular infusions. Typically, infusions are delivered via a head-sited access device; however, subcutaneously tunneled chest-sited devices could offer an alternative for improved ease of access and patient comfort. The aim of this study was to evaluate the safety, efficacy, and user experience of chest-sited versus head-sited intracerebroventricular access devices in patients with CLN2.

Methods: This single-center prospective observational study included pediatric patients with CLN2 who received cerliponase alfa via intraventricular infusions using chest-sited and/or head-sited devices. Incidents and complications, as well as access frequency, were documented over 1 year for both device types. Families and nurses were surveyed regarding sedation, distraction, access, securing, satisfaction, and device preference, and comparisons were made between chest-sited and head-sited devices regarding these factors.

Results: Seventeen children (10 female, mean age 7.5 [SD 3.1] years) with CLN2 were included; 11 received chest-sited and 6 received head-sited devices. Families of 16 patients (10 with chest-sited and 6 with head-sited devices) completed the questionnaire. Sedation was required for 4 patients, 1 with a chest-sited device and 3 with head-sited devices. Distraction techniques, particularly singing and technology, were effective for 75% of patients who completed the family questionnaire. There was no significant difference in ease of access or securing between the two device types (p > 0.9999) according to the families. However, nurses reported significantly easier access (p = 0.0308) and securing (p < 0.0001) with chest-sited devices. Among patients who received both device types, 50% preferred chest-sited devices, 50% had no preference, and no patients preferred head-sited devices. Access success rates were higher for chest-sited devices (p = 0.0128), but device survival was comparable (p = 0.4492). The incident and complication rate was similar between groups, with no device-associated infections recorded.

Conclusions: Chest-sited intracerebroventricular access devices for cerliponase alfa infusions offered benefits that included reduced sedation, easier access, better device securing, higher access success rates, and greater nursing satisfaction. Both devices had similar survival rates and incident and complication rates. Chest-sited devices can be considered a safe and effective alternative in this setting.

Objective: Referral patterns for pediatric epilepsy surgery in publicly funded healthcare systems remain unclear. This study quantified referral intervals and identified predictors of prolonged referrals at Finland's primary pediatric epilepsy surgery center. Additionally, the authors examined time intervals from drug-resistant epilepsy (DRE) diagnosis to surgical referral.

Methods: A retrospective analysis of the charts of 251 pediatric epilepsy surgery patients treated between 2002 and 2024 was conducted (median age at surgery: 9.63 years). Geographical regions were categorized based on university hospitals' catchment areas to southern, western, central, and northern regions. The authors collected data on clinical, diagnostic, and surgical characteristics; referral centers; and time intervals across different stages of the referral process. Predictors of prolonged referral duration (above median) were identified using an adjusted logistic regression model.

Results: The median (IQR) onset-to-referral interval was 3.24 (5.34) years, with no significant regional differences (p = 0.73). Among 155 patients with detailed data on prescription of a third antiseizure medication, the median (IQR) interval from DRE to surgical referral was 2.02 (3.37) years. Referral hospitals with systematic epileptologist consultants had a shorter onset-to-surgery interval (3.89 vs 5.31 years, p = 0.043) than those without. Adjusted predictors of prolonged onset-to-referral interval included seizure remission (OR 8.70, p < 0.001) and MRI negativity (OR 3.73, p = 0.037). Furthermore, active infantile spasms at referral (OR 0.076, p = 0.046), daily seizures (OR 0.27, p < 0.001), and low-grade tumors (OR 0.25, p = 0.011) were associated with shorter onset-to-referral interval.

Conclusions: Prolonged durations of epilepsy before surgical referral are linked to epilepsy severity and pre-referral MRI findings. In Finland, referral intervals are consistent across regions despite long travel distances. Overall, epilepsy treatment is efficient nationwide, with relatively short and equal referral intervals. Epileptologist outreach consultations should be considered to facilitate earlier identification of surgical candidates and reduce referral delays.

Objective: The objective of this study was to investigate the relationship between unilateral premature fusion of the posterior intraoccipital synchondrosis (UPF-PIOS) and head tilt in pediatric patients, as well as to analyze the clinical features of children diagnosed with UPF-PIOS exhibiting torticollis.

Methods: A retrospective observational study was conducted at the Center for Torticollis in the Department of Physical Medicine and Rehabilitation of a tertiary hospital. Among 14,869 pediatric patients who visited the clinic between January 1, 2005, and July 15, 2024, 1680 children with persistent torticollis underwent 3D CT for further evaluation. UPF-PIOS was diagnosed based on three criteria: 1) asymmetrical posterior skull base on 3D volume-rendered images, 2) confirmation of UPF-PIOS on 3D or axial craniofacial CT, and 3) a ≥ 2-mm vertical skull length difference between the right and left sides on coronal CT images. Clinical characteristics such as sex, age at diagnosis, skull asymmetry, head tilt direction, and craniovertebral junction abnormalities were analyzed.

Results: Fifteen cases of UPF-PIOS were identified (0.89% of those who underwent imaging). The median age at diagnosis was 19 months, with most diagnosed before age 3 years. The median vertical length difference was 8.0 mm, with the affected side being shorter. All patients exhibited head tilt toward the UPF-PIOS side, and 53% had craniovertebral junction abnormalities.

Conclusions: UPF-PIOS is an underrecognized cause of torticollis in young children. Its characteristic clinical features include an asymmetrical posterior skull base, shortening of the skull on the affected side, and a head tilt toward the side of the prematurely fused synchondrosis. Recognizing this pattern-a tetrad of unilateral synchondrosis fusion and the three resultant findings-is clinically important for differential diagnosis. Considering UPF-PIOS in children with torticollis (especially when physical therapy fails) can prevent misdiagnosis (e.g., distinguishing it from lambdoid craniosynostosis) and guide appropriate management. Further research with larger cohorts and long-term follow-up is warranted to confirm these findings.

Objective: The Matsushima grading system is widely used to evaluate angiographic revascularization outcomes after pial synangiosis for moyamoya vasculopathy by quantifying collateral ingrowth. The authors hypothesized that the orbital grading system (OGS), a new scale developed for adults to measure collateral ingrowth, would provide greater consistency and interrater reliability. They aimed to compare the performance of these scales for the first time in the pediatric population.

Methods: The authors performed a retrospective analysis of patients with moyamoya vasculopathy with follow-up catheter angiography after indirect revascularization at a single major pediatric center from 2006 to 2023. An interrater reliability analysis was performed using 5 raters with varying levels of training, who provided scores for 30 blinded cases. Fleiss' kappa coefficient (κ) was calculated as a measure of agreement beyond chance.

Results: A total of 101 patients with a median age of 9 years, of predominantly female sex (56.4%) who were mostly affected by moyamoya disease (71.3%) were included. A total of 158 cerebral hemispheres were treated with pial synangiosis (57 patients had bilateral surgeries). Most hemispheres were categorized as Suzuki stage 3 or above (82.9%). At a median of 12.4 months postoperatively, collateral growth was classified as Matsushima grade A in 57.6%, B in 24.1%, C in 14.6%, and as borderline grades in 3.8%. The OGS scores were grade 0 in 7.0%, grade 1 in 8.9%, grade 2 in 43.7%, and grade 3 in 40.5%. Interrater agreement evaluation revealed that the OGS had a superior κ value compared with the Matsushima scale (0.51 vs 0.15, p < 0.001).

Conclusions: The OGS had higher interrater agreement rates than the Matsushima scale for identifying postoperative collateral ingrowth in children after pial synangiosis. This scale provides a more consistent method for evaluating angiographic outcomes after indirect revascularization in the pediatric population.

Objective: Endoscopic third ventriculostomy (ETV), an alternative to ventriculoperitoneal shunt (VPS) placement, is associated with a higher risk of early failure, sometimes necessitating subsequent VPS insertion. The authors evaluated the impact of ETV prior to VPS placement on the risk of VPS failure by using the Hydrocephalus Clinical Research Network (HCRN) Core Data Project.

Methods: The authors retrospectively reviewed prospectively collected data from the HCRN Core Data Project (registry) on all children who underwent VPS surgery between April 2008 and July 2023. Children who had undergone VPS placement as initial treatment for hydrocephalus were compared with those who had undergone ETV prior to VPS insertion. The primary outcome was initial VPS survival, with failure defined as any subsequent hydrocephalus procedure. Kaplan-Meier and Cox proportional hazards analyses were performed to assess the effect of prior ETV, controlling for age at the first permanent procedure (ETV or VPS placement), hydrocephalus etiology, and complex chronic conditions (CCCs).

Results: VPS placement was performed in 6206 children, of whom 642 underwent VPS placement after failed ETV. VPS insertion after ETV improved shunt survival at 1 year (74.3% vs 67.8%, p < 0.001). Unadjusted analysis showed improved VPS survival after ETV (HR 0.78, 95% CI 0.68-0.90, p = 0.001). Corrected age ≥ 30 days at the time of the first permanent procedure, choroid plexus cauterization, etiology of hydrocephalus other than intraventricular hemorrhage of prematurity, and the absence of a CCC were also associated with improved shunt survival on univariable analysis. Multivariable analysis revealed that hydrocephalus etiology, the absence of a CCC, corrected age ≥ 30 days at the time of the first permanent procedure, and ETV prior to VPS insertion (HR 0.85, 95% CI 0.74-0.98) remained associated with shunt survival (all p < 0.05).

Conclusions: This analysis demonstrates an association between ETV prior to VPS placement and improved VPS survival, even when controlling for age, comorbid conditions, and hydrocephalus etiology. Further work is needed to understand the mechanism of this effect.