Child's Nervous System最新文献

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.

Background: Myelomeningocele (MMC) is the most common type of congenital spinal malformation, typically requiring surgical intervention. While prenatal repair is increasingly favored, postnatal repair remains the standard in many settings. This study aims to evaluate the antibiotics prescribed to neonates with MMC and their correlation with central nervous system (CNS) infection rates following postnatal surgical repair.

Methods: A retrospective cohort study was conducted, including newborns who underwent postnatal MMC repair between August 2017 and June 2021 at the Children's Hospital of Goiânia. The study examined variables related to the pregnancy and birth periods, as well as anatomical and neurosurgical factors. Neonates were categorized into four groups based on the time interval between birth and surgical repair (within 24, 48, 72 h, and beyond 72 h) and were evaluated for CNS infection rates.

Results: A total of 38 neonates were included, with 24.32% diagnosed with CNS infections post-surgery. Notably, 22.86% of mothers did not receive prenatal care. Cesarean section was the predominant mode of delivery, accounting for 77.78% of cases. The average time from birth to surgery was 67 h. At admission, 73.68% of MMC cases presented with a rupture of the MMC membrane, and 78.94% of newborns received intravenous antibiotics. The mean surgical time was 60 min, with 78.94% of surgeries performed by experienced surgeons.

Conclusion: Timely surgical intervention and the use of prophylactic antibiotics are critical in reducing CNS infection rates in neonates undergoing postnatal MMC repair. Delays in surgery and the lack of antibiotic prophylaxis were associated with higher infection rates, whereas factors such as delivery mode and surgeon experience had less impact on infection outcomes. These findings highlight the need for standardized care protocols and improved prenatal care to optimize neonatal outcomes.

Congenital infantile brainstem high-grade gliomas (HGGs) are extremely rare. Given the limited literature characterizing this disease, management of these tumors remains challenging. Brainstem HGGs are generally associated with extremely poor prognosis. Limited reports of spontaneous regression of radiologically diagnosed infantile brainstem tumors exist in published literature. In this case report, we document the first histologically proven congenital brainstem HGG with molecular characteristics that did not fall under any previously well-defined pediatric brain tumor classifications. The tumor underwent regression after biopsy, documented on neuroimaging up to 2 years of age. A review of the literature was also performed to identify previously reported infantile brainstem HGGs and the management for such tumors. Our case highlights the value of performing histopathological confirmation to guide management and the possible existence of a subcategory of a congenital brainstem HGG with better prognosis.

Background: Atypical teratoid/rhabdoid tumours (ATRTs) are malignant central nervous system tumours, typically presenting in the posterior fossa of very young children. Prognosis remains poor despite current therapy, while tumorigenesis implicates both genomic and epigenetic dysregulation. Primary diffuse leptomeningeal (PDL) ATRT, characterised by the absence of an intraparenchymal mass lesion, is seldom reported but appears associated with a dismal outcome.

Case presentation: We describe a 7-year-old male presenting with a PDL MYC-subgroup ATRT. The patient received multimodal upfront therapy, including high-dose craniospinal radiotherapy, embedded within a chemotherapy backbone. An unexpected clinical and radiological improvement was also observed upon cessation of all therapy for presumed disease progression. Although the patient eventually succumbed to the disease at 30 months, he demonstrated the longest survival for any PDL ATRT patient reported (median 8 months).

Conclusion: Exhaustive literature review identified seven preceding published cases of PDL ATRT. Ours is the only one to have molecular subgrouping assigned. Perfusion imaging, within a multi-parametric diagnostic package, may be a sensitive marker for malignancy against other aetiologies in challenging presentations. Acknowledging the scarcity of the entity, we cautiously suggest a combination of chemotherapy and upfront high-dose craniospinal radiotherapy, if appropriate, may prolong survival for older children with PDL ATRT compared to exclusive chemotherapy or focal irradiation-based strategies. Our patient's recovery during palliation following a radiological diagnosis of disseminated relapse highlights the importance of confirming disease recurrence by tissue extraction where feasible.

Purpose: We hypothesize that distal shunt catheters fully impregnated with barium are more prone to failure compared to distal catheters with only a barium stripe. We sought to evaluate this distinction using a matched case-control study.

Methods: Patient records over an 8-year period were queried for distal shunt revisions for fracture or disconnection (cases). A control group of patients with confirmed functioning distal catheters was queried from the same period and matched based on patient age at exploration/revision. Data were collected via chart review, including demographics, hydrocephalus etiology, distal catheter type, and patient age at revision. Independent T-test, chi-squared, and binomial logistic regression analyses were performed.

Results: There were 194 patients included in the study: 97 patients with distal shunt revision and 97 controls with a functional distal shunt system. The mean patient age at distal catheter revision was 12.87 ± 4.59 years, and the mean patient age of the control group was 12.81 ± 4.59 years. The most common etiology was premature intraventricular hemorrhage (32%). Of the distal failures, 41.2% had fully impregnated barium catheters, while 58.8% had striped barium catheters. Of the control group, 76/97 (78%) patients had barium-striped distal shunt catheters and 21/97 (22%) had fully impregnated catheters. Logistic regression analysis showed that fully impregnated catheters were more likely than striped barium to fail, p = 0.004 (OR = 2.54, 95% CI 1.35-4.77).

Conclusion: In a matched case-control format, odds of failure of fully impregnated distal catheters were 2.54 greater than striped barium catheters.

Introduction: Glioblastomas (GBM) are aggressive tumors that make up about 7% of central nervous system tumors in children. Spinal GBMs (sGBMs) are extremely rare, accounting for less than 1% of pediatric spinal tumors. sGBMs are difficult to treat due to their infiltrative nature and cause significant morbidity. While there is extensive literature on treatment outcomes for cranial GBMs, there is limited research on pediatric sGBMs. This meta-analysis aims to assess the impact of available treatments on overall survival (OS) and progression-free survival (PFS) in pediatric sGBM patients and to identify prognostic factors.

Methodology: A comprehensive review of pediatric sGBM cases up to June 2024 was conducted using PubMed and Mendeley. Inclusion criteria were case series and case reports of pediatric sGBM, excluding those with metastatic sGBM or aggregated patient data. A total of 2202 articles were identified, with 46 meeting the inclusion criteria. Data on demographics, tumor characteristics, extent of resection, and treatments were collected. Kaplan-Meier and Cox proportional hazards models were used for statistical analysis.

Results: The data was collected from 81 patients, 43 females and 38 males, with an average age of 10.7 years. The majority of tumors were found in the cervical region (32%). Subtotal resection (STR) was performed in 53% of cases, and 59% of patients received both chemotherapy (QT) and radiotherapy (RT). The average progression-free survival (PFS) was 10.95 months, with RT significantly improving PFS (15.2 months vs. 2.1 months, p = 0.001). The average OS was 13.4 months, with RT and QT being significant protective factors (p < 0.05). Age over seven years and cervical tumor location were associated with worse OS.

Conclusion: This study highlights the significance of radiation therapy and chemotherapy in enhancing overall survival and progression-free survival in pediatric patients with spinal cord glioblastoma. Specifically, RT significantly improves PFS, while advanced age and tumor location in the cervical region are associated with worse outcomes. These findings can help shape treatment approaches and ultimately enhance the quality of life for pediatric sGBM patients.