Child's Nervous System最新文献

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: A predictive index for surgical complications in pediatric patients is lacking in the current scientific literature. We have developed a simple index to accurately predict the likelihood of complications after surgery. The Surgical Complication Index for Pediatric Patients (SCIPP) is reliable and accurate for both heterogenous and specific groups of pediatric patients.

Methods: A retrospective analysis of pediatric neurosurgical procedures from 2005 to 2023 was conducted. Logistic and linear regression analyses were performed to analyze the correlation with their respective SCIPP scores and post-operative complications and length of stay (LOS).

Results: Our cohort included 90 tethered cord patients and 43 surgeries consisting of craniotomies/craniectomies, laminectomies/laminotomies, and others. The mean SCIPP score across all 133 patients was 2.41 ± 1.76, with an average age of 5.37 ± 5.53 years. From the logistic regression, each 1-point increase in SCIPP score was associated with increased odds of experiencing a complication after surgery in all patients (odds ratio: 1.57; p < 0.001) and tethered cord patients (odds ratio: 1.59; p = 0.007). The reduced 4-point SCIPP was associated with increased odds in all patients (odds ratio: 2.67; p < 0.001) and tethered cord patients (odds ratio: 2.89; p = 0.001) as well. Upon linear regression analysis, each 1-point increase in SCIPP was associated with a 0.49 day increase in LOS (p = 0.002). Each 1-point increase in reduced SCIPP was associated with a 0.42 day increase in LOS but was not statistically significant (p = 0.120).

Conclusion: The SCIPP is a simple and accurate tool that predicts surgical complications and LOS in a variety of pediatric neurosurgical procedures and can be used to counsel patients and families on the risks of surgery.

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.

Purpose: Spinal epidural lipomatosis (SEL) is a relatively rare condition characterized by overgrowth of adipose tissue in the extradural space of the spinal canal. Compression of neural structures can lead to radiculopathy, loss of sensation and motor function, and urinary retention. Known causes of SEL include exogenous steroid use and obesity.

Methods: We describe the unique case of idiopathic SEL in a 1-year-old male, the youngest reported to date, diagnosed with medulloblastoma and treated with chemotherapy and autologous stem cell rescue (AuSCR). We then perform a systemic review of the literature of this rare diagnosis, explore potential causes, and review management.

Results: The patient was non-obese and received a cumulative dose of only 60 mg dexamethasone (150 mg/m²), far below pediatric cancer patients with SEL documented in the literature. The acute presentation of lipomatosis following chemotherapy and AuSCR, associated with transient elevated triglycerides (234 mg/dL), suggests this as a possible cause.

Conclusion: This case illustrates the development and resolution of SEL in a non-obese pediatric cancer patient with limited steroid use. While further study is needed to identify the etiology of SEL, this diagnosis should be evaluated for when reviewing spine imaging following chemotherapy or autologous stem cell rescue.

Purpose: This study was aimed at evaluating cognitive functioning in children with brain tumors before and after surgery and at the end of all adjunctive treatments.

Methods: All newly diagnosed children with primary brain tumor were prospectively assessed. Neurocognitive evaluations were performed before surgery (T0), within 7-10 days from surgery (T1) and 18-24 months after the end of all treatments (T2). Language, memory, attention, executive functions, visual-constructional, and sensorimotor skills were evaluated at T0, T1, and T2, whereas intelligent quotient (IQ) was explored at T0 and T2.

Results: Twenty-five patients (M:F = 15:10, mean age 10.9 ± 3.4 years) were enrolled between January 2019 and December 2022. At baseline, patients showed major deficits in narrative memory (6.6 ± 3.7, p < 0.001) and visuo-spatial memory (copy design tasks: general 6.8 ± 3.9, p < 0.001; specific 6.2 ± 3.3, p < 0.001; and motor 5.2 ± 3.2, p < 0.001). In the post-surgery phase (T1), significant deficits remained in narrative memory (6.2 ± 3.3, p < 0.001) and visual-motor coordination (copy design tasks: specific 5.9 ± 3.0, p < 0.001; motor 4.8 ± 4.3 p < 0.001), while attention and visual-constructional abilities significantly improved (p = 0.04 and p = 0.001 respectively). Nine out of 25 patients (36%) reached the T2 evaluation: persistency of deficits in the area of visuo-spatial processing and a possible decline in median IQ values in comparison to T0 evaluation (93 vs 100, p = 0.05) were shown.

Conclusions: Children with brain tumors may present several neuropsychological impairments since diagnosis. Surgery may have a positive impact in relation to the recovery of some cognitive functions. However, cognitive decline may worsen over time. Baseline and periodic neurocognitive evaluations should be encouraged to indicate targets for cognitive monitoring, to early detect functional difficulties.

Objectives: Posterior fossa decompression is currently an operative treatment of choice for Chiari Malformation I (CM1). However, there is controversy surrounding the possible benefits of employing intraoperative neuromonitoring (INM) for this type of procedure. In addition to presenting our single-center experience on the use of INM, we analyze the cost associated with INM in Chiari Malformation (CM) decompression surgery using the Healthcare Cost and Utilization Project (HCUP) database and discuss the legal implications of somatosensory evoked potentials (SSEP) monitoring during decompression for CM1.

Methods: We conducted a retrospective review of all patients undergoing CM1 decompression with SSEP neuromonitoring from 2011 to 2018. We collected patient characteristics, hospital charges, and surgical cost data from the HCUP database for patients undergoing CM decompression. Finally, we performed a review within the Thompson Reuters Westlaw Edge database for reported litigation involving INM for CM decompression.

Results: None of the 110 patients submitted to surgery for CM1 at our institution had any significant SSEP changes intraoperatively or developed post-operative neurological deterioration. There were higher mean total hospital charges and surgical costs associated with INM ($31,272) for patients who received INM compared to patients who did not receive INM ($24,112). A careful review of the Westlaw database with multiple-word search strategies revealed no reported medical malpractice claims regarding the absence of SSEP neuromonitoring in a CM decompression procedure.

Conclusion: Using data collected at our institution and the HCUP national database, we showed that intraoperative neuromonitoring did not affect surgical planning and decision-making or post operative care, while adding unnecessary costs to CM decompression procedures. The absence of reported malpractice claims targeting the lack of neuromonitoring in CM cases suggests that SSEP neuromonitoring during CM may be unnecessary. We propose that neuromonitoring should not be used for routine CM decompression.