Journal of neurosurgery. Pediatrics最新文献

Objective: Hemispherotomy and posterior quadrant disconnection (PQD) are standard surgical treatments for medically intractable epilepsy in pediatric patients. However, the current academic literature is limited on their efficacy and safety in very young patients. This study evaluates the outcomes of patients ≤ 12 months of age who underwent hemispherotomy or PQD.

Methods: The authors retrospectively reviewed the medical records of patients ≤ 12 months of age who underwent epilepsy surgery at Washington University and St. Louis Children's Hospital from 2003 to 2023. Data on diagnosis, age at surgery, operative details, hospital length of stay, and complications were reported. The Engel classification and Gross Motor Function Classification System (GMFCS) were used to assess seizure and motor outcomes, respectively.

Results: A total of 14 patients who underwent either hemispherotomy (n = 12) or PQD (n = 2) were included. Overall, 57% had a diagnosis of hemimegaloencephaly, 29% had middle cerebral artery infarcts, and 14% had cortical development malformation. The mean age at surgery was 6 months, with the youngest child being 2 months of age. The mean estimated blood loss was 58% of the total blood volume. The mean duration of follow-up was 4.8 years. At 1 year postoperatively, seizure freedom was 75%. At the last follow-up, 54% of patients were free of disabling seizures (Engel class I), with 57% of this subgroup being completely seizure free (Engel class IA). Of the remaining patients, 23% experienced rare disabling seizures (Engel class II) and 23% experienced worthwhile improvement (Engel class III). At the last follow-up, 67% of patients had a GMFCS level of I or II (walking with no or some limitations) and 33% had a GMFCS level of IV (assisted self-mobility). There were no deaths or long-term postoperative complications.

Conclusions: Hemispherotomy and PQD in children ≤ 12 months of age are associated with meaningful seizure control and improved motor outcomes. These procedures should be considered for the treatment of medically refractory epilepsy, even in very young infants.

Objective: Nonsyndromic sagittal craniosynostosis is the most common form of craniosynostosis, and its management includes open cranial vault reconstruction (CVR) or minimally invasive techniques such as endoscopic suturectomy with helmet orthosis (ESO) and spring-assisted suturectomy (SAS). Delayed surgical referral can limit eligibility for minimally invasive techniques, which are associated with lower morbidity. Disparities in socioeconomic factors may contribute to later presentation, particularly for minority populations. This study explores the impact of sociodemographic variables, Area Deprivation Index (ADI), and Child Opportunity Index (COI) on referral timing, surgical timing, and the type of intervention performed.

Methods: The authors performed a retrospective review of children who underwent surgery for nonsyndromic single-suture sagittal craniosynostosis at a large urban referral center between 2015 and 2023. Patients were categorized into earlier referral (< 2 months) or later referral (≥ 2 months) cohorts and earlier surgery (< 6 months) or later surgery (≥ 6 months) cohorts. Demographics, socioeconomic indices (ADI, COI), and procedure type were compared between groups.

Results: Two hundred four children, 29.4% of whom were female, met the study inclusion criteria. Among these children, 50.0% underwent CVR, 36.3% SAS, and 13.7% ESO. Patients in the later surgery cohort were significantly more likely to be non-White (29.1%, p < 0.01), non-English speaking (13.9%, p < 0.01), and publicly insured or uninsured (59.5%, p < 0.01). Non-White patients were referred later (93 vs 45 days, p < 0.001), disproportionately older at surgery (207 vs 148 days, p < 0.001), and more likely to undergo CVR (69.2%, p < 0.001). Distance to the hospital, median household income, ADI, and COI were not significant predictors of the type of surgery performed.

Conclusions: This study highlights disparities in access to minimally invasive craniosynostosis surgery, particularly among non-White, non-English speaking, and socioeconomically disadvantaged children in the state of Georgia. Targeted efforts in community education are needed to reduce this disparity and strive toward equitable access to care.

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.