Journal of neurosurgery. Pediatrics最新文献

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.

Objective: The Hydrocephalus Clinical Research Network-Quality (HCRNq) was established to encourage adoption of evidence-based best practices for the care of children with hydrocephalus. A shunt infection prevention initiative is ongoing within the network, and an analysis of baseline data suggested important practice variation with respect to shunt infection prevention practices. In a first attempt to standardize care, the authors recommended adoption of an endorsed shunt infection prevention protocol within the network, and now present information related to protocol adoption, compliance, and its effect on shunt infection rates.

Methods: A memorandum was circulated to HCRNq sites endorsing and recommending adoption of a 7-step shunt infection prevention protocol. Patient and procedural data relevant to shunt surgery and infection prevention were then prospectively collected from 31 network sites. The relationship between the infection outcome and patient and procedural variables, including protocol adoption, was modeled using uni- and multivariable statistics.

Results: An unadjusted infection rate of 3.6% was observed over nearly 3500 shunt procedures, similar to what was observed at baseline, but with a narrower range, suggesting that some outlying sites were brought closer to the network average. An increased infection risk was associated with shunt placement for preterm posthemorrhagic hydrocephalus, the occurrence of prior shunt surgery within 6 months of the index procedure, and the use of antibiotic ointment. A reduced infection risk with the use of antibiotic-impregnated catheters was suggested. Adoption of the recommended protocol was modest, and as a result, significant practice variation continued to be observed. In this analysis, no relationship was observed between infection risk and the use of an infection prevention protocol.

Conclusions: This first attempt to encourage standardization of perioperative practices for shunt infection prevention, by endorsing an evidence-based shunt infection prevention protocol for use at network sites, resulted in incremental protocol adoption. Many shunt procedures continue to occur without the cover of a protocol. Although adherence to a protocol did not appear to influence infection risk in the present analysis, an opportunity to improve remains, with a potential beneficial effect on infection rates to follow. The authors continue to work toward further improvement in this regard.

Objective: Children with cerebral arteriovenous malformation (AVM) have a high risk of spontaneous intracerebral hemorrhage. The identification of patients at risk for AVM rupture and accurate prediction of outcomes are critical to guide management strategies. The authors examined the angioarchitectural features of pediatric AVMs associated with hemorrhagic presentation and reviewed the utility of the Ruptured AVM Grading Scale (RAGS) for outcome prognostication in pediatric patients treated surgically at a single institution.

Methods: The authors retrospectively reviewed the records of pediatric (age ≤ 18 years) patients who underwent resection of AVMs at their institution between 1998 and 2023. Demographic factors, clinical presentation, and angioarchitectural details including nidus size, location, venous drainage, presence of flow-related aneurysm, presence of an en passage vessel, and perforator vessel supply were collected. Clinical presentation and angioarchitecture were compared in patients with ruptured and unruptured AVMs. In patients with ruptured AVMs, the area under the receiver operating characteristic curve (AUROC) was calculated based on their RAGS scores at 3 follow-up time points using dichotomous analysis of the modified Rankin Scale score as the response variable.

Results: Of the 62 patients included in this study, 59.7% presented after rupture. Patients with ruptured AVMs had, on average, a smaller nidus (24.84 vs 38.24 mm, p < 0.001) and a higher percentage of deep venous drainage (64.9% vs 32.0%, p = 0.009). Multivariable logistic regression analysis confirmed smaller nidus diameter (OR 0.93, 95% CI 0.87-0.98, p = 0.016) and presence of deep venous drainage (OR 10.78, 95% CI 1.73-42.94, p = 0.011) as independent predictors of hemorrhagic presentation. Good clinical outcomes (modified Rankin Scale [mRS] score ≤ 2) at last follow-up were similar for the unruptured and ruptured cohorts (85.5% vs 88.0%, p = 0.230). For patients with ruptured AVMs, the AUROC values for the RAGS scoring system were 0.48 at 3 months, 0.67 at 1 year, and 0.70 at the last known follow-up.

Conclusions: Smaller AVM nidus size and deep venous drainage were associated with hemorrhagic presentation in children with AVMs. Although the AUROC for RAGS improved with longer follow-up periods, it did not reach the 0.8 threshold needed for clinical utility.

Objective: The treatment of sagittal craniosynostosis typically involves endoscopic suturectomy (ES) to allow skull expansion, followed by postoperative helmet orthosis, resulting in an improvement in cranial deformity as assessed using the cephalic index (CI). The impact of variations in surgical technique on long-term CI outcomes is not well understood, and there is controversy regarding whether adding barrel stave osteotomy (BSO) to standard ES leads to greater improvement in the CI postoperatively. This combined approach is thought to improve cranial shape and overall clinical outcomes but may increase operative burden. The aim of this study was to investigate the impact of BSO during ES on operative outcomes and postoperative cranial deformity in patients who underwent surgical correction of sagittal craniosynostosis.

Methods: The authors conducted a retrospective chart review of children who had been treated with ES for sagittal craniosynostosis between 2010 and 2021 at British Columbia Children's Hospital. Demographics, operative outcomes, and postoperative longitudinal CI measurements were collected and compared between patients who had undergone ES with BSO (ES+BSO) and those who had undergone ES alone. Operative outcomes related to anesthesia and surgical time, blood loss, and hospital length of stay were analyzed, as were changes in the CI at various follow-up times. A mixed-effects model was used to compare longitudinal CI measurements between treatment groups, controlling for patient age, preoperative CI, and duration of helmet therapy.

Results: Eighty-five patients were included in the analysis, 67 treated with ES+BSO and 18 treated with ES. Operative outcomes, including length of hospital stay, operative time, time under anesthesia, blood loss, and need for transfusion did not differ significantly between treatment groups (p > 0.05). The mean follow-up for the last CI measurements was 56.0 months. While preoperative CI was similar for the two groups (mean 67.4 for ES+BSO vs 66.8 for ES, p = 0.61), CI was significantly higher in the ES+BSO group immediately postoperatively (p = 0.004) and at the 6-month (p = 0.01), 2-year (p = 0.02), and final (p = 0.002) follow-ups. A mixed-effects model revealed that the addition of BSO led to significantly greater CI measurements independent of age, preoperative CI, and helmeting duration (estimated effect size 2.21, p = 0.001).

Conclusions: In this series, the addition of BSO to ES significantly improved immediate and long-term cranial deformity in patients with sagittal craniosynostosis, without increasing the operative burden.