Child's Nervous System最新文献

Purpose: Gangliogliomas are rare, slow-growing brain tumors frequently associated with seizures in pediatric patients. This study evaluated the utility of high-field intraoperative magnetic resonance imaging (ioMRI) in the surgical management of ganglioglioma-related drug-resistant epilepsy in children. Specifically, it sought to determine whether ioMRI improves outcomes by optimizing the extent of resection, enhancing seizure control, reducing reoperations, and minimizing complications.

Methods: This retrospective single-center study included 14 pediatric patients with cerebral ganglioglioma who underwent epilepsy surgery with ioMRI from 2014 to 2022. The median age was 11.5 years (range 3-16 years). Patient demographics, the rate of continued ioMRI-guided surgery, the extent of resection, histology, complications, reoperations, and seizure outcomes at 1 year postoperatively were assessed.

Results: Residual tumor was detected using ioMRI in 9 of 14 patients (64.3%), leading to further resection. Complete tumor resection was achieved in 12 patients (100%) as intended, while two patients underwent planned tumor debulking. Temporary neurological deficits were observed in two patients, with no permanent deficits documented. One patient required reoperation, and another was scheduled for one. The median follow-up duration was 43 months (range 12-65 months). Seizure outcomes were classified as Engel I in 9 of 10 (90%) and Engel III in 1 of 10 patients (10%). Four patients were lost to follow-up.

Conclusion: The use of ioMRI in pediatric epilepsy surgery for ganglioglioma facilitated more complete resections, contributing to favorable seizure outcomes and a low complication rate. These findings support ioMRI as a valuable tool in optimizing surgical management for this patient population.

Malignant tumours arising at the site of closed neural tube defects are extremely rare and have only been described three times in the literature. We report the case of a child with a congenital lumbosacral lipoma, a dermal sinus tract and low-lying spinal cord who was initially treated non-operatively due in part to non-attendance and who then presented at 2 years of age with recurrent urinary tract infections with intermittent pyrexia, back pain and difficulty walking. A craniospinal MRI was performed with suspicion of infection at the site of the closed neural tube defect (in view of the dermal sinus tract) and leptomeningeal spread to the rest of the spine and brain. An urgent lumbar laminectomy for untethering of the spinal cord and washout under intraoperative neuro-monitoring was performed. A solid mass was encountered in the lumbosacral canal and debulked. It was initially thought to represent solid granulomatous inflammatory tissue. Histopathological examination was, however, consistent with embryonal rhabdomyosarcoma. After multidisciplinary team discussion, chemotherapy was initiated. She developed hydrocephalus which required treatment initially endoscopically and later with a shunt. Unfortunately, she passed away 69 days after diagnosis due to disease progression despite chemotherapy. Rhabdomyosarcomas at the site of a lipomatous malformation are extremely rare and may arise from striated muscle cells within the lipoma. The underlying mechanisms are unclear. Urgent repeat imaging in patients with lipomas and rapid deterioration is essential to rule out this rare but devastating phenomenon.

Introduction: Hydrocephalus is a common condition in childhood, often treated with ventriculoperitoneal shunts (VPS). Endoscopic third ventriculostomy (ETV) has emerged as an effective alternative, and the addition of choroid plexus coagulation (CPC) is believed to enhance its success, particularly in children under 6 months.

Methods: In this observational study, a retrospective analysis was conducted on 167 pediatric patients who underwent ETV, with and without CPC, at the Hospital da Restauração in Pernambuco. The primary outcome was to evaluate the success rates of ETV and ETV + CPC at immediate postoperative or outcome at discharge and 6-month follow-up. Statistical analyses, including chi-square, univariate, and multivariate logistic regression, were employed to assess correlations between dependent and independent variables.

Results: ETV + CPC showed a significantly higher success rate in the immediate postoperative period (91%) compared to ETV alone (75%) (p = 0.007). After 6 months, success rates were similar for both groups (67% for ETV + CPC and 66% for ETV), with no significant difference (p = 0.855). Univariate and multivariate analyses revealed that the type of surgery (ETV + CPC vs. ETV) was a significant predictor of immediate postoperative success (OR 2.81, 95%CI 1.18-6.72, p = 0.020). Age and sex did not correlate significantly with surgical outcomes.

Conclusion: The addition of CPC to ETV significantly enhances immediate postoperative success in young children with hydrocephalus. Although long-term outcomes equalized, these findings suggest that CPC can be a valuable adjunct in optimizing short-term results.

Introduction: Intracranial infections due to sinusitis and otitis, although rare, can progress rapidly and result in significant morbidity, necessitating multifaceted management including extended antibiotic therapy and surgical intervention. Predominantly affecting infants and older children, these infections have seen a perceived increase in incidence following the coronavirus disease 2019 (COVID-19) pandemic.

Objectives: Our study aims to describe the clinical and epidemiological characteristics of intracranial infections secondary to sinusitis or otitis in the pediatric population and assess changes in incidence and clinical presentation post-pandemic. Specific objectives include analyzing neurosurgical management practices, the role of ENT-neurosurgery cooperation, incidence of epileptic seizures, and management of associated venous thrombosis.

Materials and methods: A retrospective multicentric study was conducted in hospitals across the Iberian Peninsula, including data from January 2018 to December 2022. Data were divided into pre-lockdown (January 2018 to March 2020) and post-lockdown (March 2020 to December 2022) periods for analysis.

Results: The study included 60 pediatric cases (38 post-pandemic and 22 pre-pandemic). The average age was 9.8 years, with a male predominance (61.67%). Sinusitis was the most frequent cause (86%), and the frontal region was the most common site of infection (75%). Neurological symptoms were more prevalent post-pandemic (55.26% versus 23.68%). The primary pathogen was S. intermedius (29.6%). Most patients required neurosurgical intervention (81.7%), with a significant portion undergoing combined ENT-neurosurgery procedures (52.9%). The average antibiotic treatment duration was 6.6 weeks. Complications included venous sinus thrombosis (20%) and seizures (39.2%). Mortality was 3.3%.

Conclusion: Although there was a perceived increase in cases post-pandemic, our study observed a normalization of incidence after the lockdown, with a decrease in diagnoses during confinement. The accepted antibiotic regimen lasts 6 weeks, extendable to 8 weeks in non-surgical patients, with at least 2 weeks of intravenous treatment. Sinus surgery combined with antibiotics may suffice to avoid craniotomy in some cases, while combined surgery has a lower reoperation rate in others. Anticoagulation should be individualized and discontinued upon recanalization. Prophylactic antiepileptic drug use remains controversial and should be tailored to patients with specific risk factors. Prolonged antiepileptic drug (AED) therapy may be warranted for those with early seizures and hemorrhagic lesions, whereas others may gradually taper off AEDs after the acute stage.

Purpose: The modified Canadian Preoperative Prediction Rule for Hydrocephalus (mCPPRH) was developed to predict the need for permanent CSF diversion in children with posterior fossa tumors (PFT). This study aimed to externally validate the mCPPRH in a cohort of 113 pediatric patients with PFTs.

Methods: We conducted a retrospective analysis, calculating the mCPPRH score for each patient and performing receiver operating characteristic (ROC) curve analysis to assess the tool's discriminative ability. Sensitivity, specificity, predictive values, and likelihood ratios were calculated using a cutoff score of ≥ 5. Multivariable logistic regression with bidirectional stepwise selection was used to evaluate individual components of the score. The mCPPRH components were modified and the performance of adjusted tools was compared to the original.

Results: Of the 113 patients, 35 (31.0%) required permanent CSF diversion. The mCPPRH demonstrated acceptable discriminative ability (AUC = 0.701, 95% CI 0.608-0.795, p < 0.0003). Sensitivity was 34.1%, specificity 89.7%, positive predictive value 60%, and negative predictive value 75.3%. Initial regression identified no significant predictors. In stepwise regression, moderate-severe hydrocephalus independently predicted permanent CSF diversion (OR 6.37, 95% CI 1.71-41.55, p = 0.02). Increasing the age cutoff to < 5 years, removing tumor diagnosis, and modifying hydrocephalus weighting improved performance (AUC = 0.768, sensitivity 71.4%, specificity 75.6%).

Conclusions: The mCPPRH demonstrates acceptable discriminative ability (AUC 0.701) in our cohort, with particular utility in identifying low-risk patients. However, its poor sensitivity (34.1%) and variable predictor performance suggest that additional clinical factors should be considered for treatment planning, particularly in higher-risk cases. Further modification of mCPPRH components is suggested to improve its utility.

Background: The clinical course and therapeutic outcomes of pediatric and adult gliomas vary. Dabrafenib plus trametinib is a new therapeutic option for the management of gliomas. This study aimed to compare the outcomes of co-administration of dabrafenib and trametinib in pediatric and adult gliomas.

Methods: Systematic search was conducted in four electronic databases encompassing Pubmed, Embase, Scopus, and Web of Science. Publications that assessed the role of dabrafenib plus trametinib in adults or pediatrics were included.

Results: Eight studies with 243 individuals, encompassing 161 pediatrics and 82 adults, were included in our study. We demonstrated that despite a higher objective response rate (ORR) (53% [95% CI, 44-61%] vs. 39% [95% CI, 26-54%], P = 0.11) and clinical benefit rate (CBR) (87% [95% CI, 72-95%] vs. 73% [95% CI, 54-86%], P = 0.16) among pediatrics, the difference was insignificant. We exhibited that younger age, BRAF V600 mutation, and longer therapy periods were accompanied by better radiological outcomes among pediatrics, and the female gender was correlated with better radiological results in adults. Our findings showed that the pooled adverse event (AE) rate was 96% (95% CI, 69-100%) in pediatrics and 83% (95% CI, 42-97%) among adults; however, there was no meaningful difference (P = 0.30).

Conclusion: Co-administration of dabrafenib and trametinib is accompanied by promising results among pediatrics and adults diagnosed with glioma. However, the comparison results should be interpreted meticulously due to limitations that may affect the generalizability of the findings.

Objective: This retrospective study aimed to investigate the epidemiology of pediatric patients with thoracolumbar spinal fractures and the characteristics of associated injuries.

Methods: Sixty-one patients under 18 years old with thoracolumbar spinal fractures were included in the study. Patients were divided into two groups: ≤ 8 years (Group 1) (n = 13) and > 8 years (Group 2) (n = 43). Patients were analyzed for age, gender, fracture type, fracture level, etiology, and associated injuries.

Results: The mean age was 158.7 (± 53.1) months, and the male/female ratio was 1.26. The most common fracture etiology was motor vehicle accidents (39.4%) and falls from height (18%). There was no correlation between the fracture type, the number of fractured vertebrae, and age. The most common associated injury was orthopedic injuries (34.3%). In Group 2, associated injuries developed more frequently in the presence of a major fracture, whereas in Group 1, associated injuries could occur even in isolated minor fractures. In Group 1, intrathoracic injuries associated with fractures (36.4%) were significantly higher than in Group 2 (4.2%). Surgical intervention was required in 9.8% of patients.

Conclusion: Although thoracolumbar spinal fractures are rare in children, they are essential because preventable accidents essentially cause them and can have devastating consequences. There are significant differences in epidemiologic and clinical features between age groups. In this study, we have shown that even minor thoracolumbar vertebral fractures may be accompanied by associated injuries in children under 8 years of age. In contrast, major fractures are more frequently related to other organ injuries in older children. This may be because more severe trauma is required for a fracture of the thoracolumbar spine, which is more flexible and elastic in young children compared to adolescents. Our study has provided current and essential results regarding the etiology of pediatric thoracolumbar spine fractures. More multicenter studies are needed to understand the characteristics of thoracolumbar fractures in pediatric patients.

Objectives: To evaluate the survival of endoscopic procedures performed for complex hydrocephalus, quantify clinical outcomes in standardized scales, and assess correlation with radiological outcomes using ventricular volumetry.

Methods: A retrospective analysis of patients with complex hydrocephalus, managed with neuroendoscopic procedures at a tertiary neurosurgical center over 20 years, was performed. In addition to demographic and clinical details, pre-operative and follow-up clinical status (using the Pediatric Functional Status Score (FSS) and Pediatric Cerebral Performance Category (PCPC) Scales) was assessed. Procedure failure was defined as any subsequent surgical procedure for the management of hydrocephalus and survival as time from the first endoscopic procedure to failure or last available follow-up. Ventricular volume and ventricle:brain volume ratio was calculated using serial imaging.

Results: We analyzed 40 pediatric patients who met the study criteria with a mean age of 19 months, the most common subtype being post-meningitic multiloculated hydrocephalus (70%). The median survival of an endoscopic procedure was 24 months (5.7-33.6 months). Over a median follow-up duration of 15 months, 28 days (2.2-111 months), median FSS improved by 5 points, and median PCPC score improved from 4 (severe disability) to 3 (moderate disability). Over a median radiological follow-up of 5.9 months, the median percentage decrease in ventricle size was 27.14%, and the ventricle:brain volume ratio was 30.57%. A strong positive correlation (r = 0.58-0.75) was noted between the decrease in ventricular volume and ventricle:brain ratio with improvement in FSS and PCPC scores.

Conclusions: Endoscopic procedures, although effective in managing complex hydrocephalus, may not be a one-stop long-term solution, which we have described in terms of procedure survival. Objective scales and ventricular volumetry to quantify clinical and radiological improvement demonstrated a significant correlation, even in complex hydrocephalus. The potential of ventricular volumetry as a prognostic factor in complex hydrocephalus is postulated.

Purpose: Pediatric cervical spine injury (PCSI) can result in devastating neurologic disability. While computed tomography (CT) imaging is both sensitive and specific in detecting clinically significant injuries, indiscriminate utilization can lead to excessive ionizing radiation exposure. A routine institutional audit revealed CTs were inappropriately obtained 54% of the time. This study evaluates the effects of an updated protocol to reduce radiation exposure in pediatric trauma patients.

Methods: Data were retrospectively analyzed from a pediatric level 1 trauma center from 2021 to 2022. The data were divided into two cohorts, pre-implementation (2021) and post-implementation (2022). Inclusion criteria were patients 0-14 years old with a Glasgow Coma Scale (GCS) ranging 9-15. Outside-hospital transfers were excluded. The primary study endpoints were guideline compliance and CT utilization.

Results: A total of 82 subjects were enrolled in this study. In 2021, there were 38 subjects (female/male 15/23, mean age 5.9 years old) with an average GCS of 13.6. In 2022, there were 44 subjects (female/male 19/25, mean age 5.2 years old) with an average GCS of 14.0. In 2021, the overall protocol adherence rate was 81.6%, and post-implementation in 2022, compliance was 93.2% (p = 0.109). Following implementation, the rate of inappropriate (protocol non-adherent CT) use decreased from 58.6 to 6.8% (p < 0.05).

Conclusions: Implementation of a new evidence-based institutional protocol for PCSI was associated with improved adherence and reduction of unnecessary CT orders. Ongoing monitoring will help determine if these improvements are sustained.

Purpose: We aimed to evaluate the efficacy of selective serotonin reuptake inhibitors (SSRIs) in treating cerebellar mutism syndrome (CMS).

Methods: We retrospectively reviewed all pediatric patients who underwent a posterior fossa tumor resection between May 2007 to September 2022 at a single quaternary pediatric hospital. We evaluated clinical presentation and hospital course, including imaging findings, pathology, and surgical approaches. Propensity score matching was used to compare the symptom duration of patients who received SSRIs versus those who did not.

Results: A total of 292 patients met the criteria with 25% (n = 73) being diagnosed with CMS. Several factors were significantly associated with a CMS diagnosis, such as pre-operative hydrocephalus (p = 0.002), a vermis-splitting approach (p = 0.007), tumor in the fourth ventricle (p = 0.010), medulloblastoma diagnosis (p = 0.009), and postoperative complication (p < 0.001). Of the patients diagnosed with CMS, 32.9% (n = 24) received SSRI treatment, specifically fluoxetine (n = 18) and sertraline (n = 6). Overall, treatment did not decrease the duration of CMS symptoms or shorten the inpatient rehab course compared to matched controls. However, within the cohort of fluoxetine-treated patients, earlier initiation of medication was significantly correlated with a shorter duration of mutism (p = 0.007).

Conclusions: We report the largest cohort of CMS patients treated with SSRIs. The lack of overall clinical benefit when compared to untreated patients in our study may be due to the length of delay in starting an SSRI, since early initiation of fluoxetine correlated with shorter CMS symptoms. These results support the importance of early clinical detection of CMS and potentially treating CMS early in the patient's postoperative course.