Craniosynostosis is a common defect in the craniofacial structure of children, yet its pathogenesis remains unclear. Current research focuses mainly on bone-related genetic mutations, with less emphasis on lipid metabolism disorders. Hence, it is imperative to investigate the underlying mechanisms from various perspectives. We investigated the association between craniosynostosis and phospholipid metabolism through a multi-omics approach. This included Mendelian randomization (MR) to assess causal relationships of lipid traits, transcriptomic differential analysis of clinical samples, and single-cell RNA sequencing of murine cranial sutures to identify key cell subtypes. Furthermore, drug-target MR and network pharmacology were conducted to explore the potential therapeutic repurposing of statins. MR analysis revealed a significant protective effect of phospholipids in very low-density lipoprotein remnants against craniosynostosis. Drug-target MR targeting HMGCR indicated a protective trend (OR = 0.79) for statins, consistent with the lipid-metabolic link, although statistical significance was limited by sample size. Transcriptomic analysis identified MORN5 as a key upregulated gene enriched in glycerophospholipid metabolism. Single-cell RNA sequencing identified a specific osteogenic subtype (C4) and lipid-metabolic subtypes (C5/C6) driving the disease. Pharmacological prediction and molecular docking further suggested that Rosuvastatin could target MORN5 and epidermal growth factor receptor, showing high binding affinity (<-5 kcal/mol). Our findings provide robust evidence that phospholipid metabolism plays a critical role in craniosynostosis. While the genetic evidence for statins is exploratory, the consistent protective trend and molecular docking results suggest a potential therapeutic avenue that warrants further investigation in larger cohorts and experimental models.
Background This study aimed to identify the characteristics of Paroxysmal sympathetic hyperactivity (PSH) in pediatric patients after brain tumor resection in the Intensive Care Unit (ICU), screen laboratory markers for early auxiliary diagnosis, and explore effective intravenous treatment regimens. Methods Pediatric patients who underwent brain tumor resection between January 2020 and December 2022 were enrolled. SPSS version 26.0 was used for data processing and analysis. Results The incidence of PSH after pediatric brain tumor resection was 15.6%. Younger age and preoperative hydrocephalus were identified as independent risk factors. Optimal cut-off values were determined for blood lactate (2.55 mmol/L) and blood glucose (7.27 mmol/L). Dexmedetomidine combined with remifentanil resulted in a significantly greater reduction in PSH scores at 1 hour after treatment compared to other treatments ( P < 0.05). The length of ICU stay was significantly longer in the PSH-positive group (3.33 ± 3.89 days) than in the PSH-negative group (1.64 ± 2.42 days) ( P = 0.027), and the 1-year mortality rate (13.3%) was significantly higher in the PSH-positive group than in the PSH-negative group (1.9%) ( P = 0.012). Conclusions PSH is not uncommon after pediatric brain tumor resection. Younger age and preoperative hydrocephalus are independent risk factors. Blood lactate and blood glucose may serve as auxiliary diagnostic indicators. Dexmedetomidine combined with remifentanil may represent an effective therapeutic regimen for PSH. PSH episodes are associated with a prolonged ICU stay and an increased risk of mortality.
RATIONALE AND OBJECTIVES:To compare the image quality and quantitative parameters of point spread function-encoded echo planar imaging (PSF-EPI) and turbo gradient spin echo BLADE (TGSE-BLADE) for distortion-free diffusion-weighted imaging (DWI) of intracranial tumors. MATERIALS AND METHODS:This prospective study included 37 patients (18 males, 19 females, median age: 11 years) with intracranial tumors who underwent 3 T MRI using both PSF-EPI and TGSE-BLADE DWI sequences. Two radiologists independently evaluated qualitative parameters, including tumor depiction, artifacts, geometric distortion, and overall image quality on a 5-point scale. Quantitative metrics, including signal-to-noise ratio (SNR), contrast, contrast-to-noise ratio (CNR), and apparent diffusion coefficient (ADC), were measured. Statistical methods included paired tests, Cohen's Kappa statistics, and Bland-Altman analysis for assessing ADC agreement in normal-appearing white matter and tumors. RESULTS:PSF-EPI demonstrated significantly better tumor depiction (p<0.01) and overall image quality (p<0.01) than TGSE-BLADE, with no significant difference in artifact (p=0.256) or distortion scores (p=0.389). Inter-rater agreement was substantial to almost perfect. Quantitatively, PSF-EPI yielded significantly higher SNR (p<0.001), whereas CNR was comparable between the two sequences (p=0.988). Tumor contrast was slightly higher in TGSE-BLADE than in PSF-EPI (p=0.034). In normal-appearing white matter, ADC measurements showed good agreement with only a small mean difference and no significant inter-sequence difference (p=0.097). Likewise, no significant inter-sequence difference in lesion ADC was observed (p=0.195), although ADC differences in tumors showed wider dispersion than those in normal-appearing white matter on Bland-Altman analysis. CONCLUSION:While both sequences provide distortion-free imaging, PSF-EPI offers superior image quality and significantly higher measured SNR. ADC measurements were comparable between PSF-EPI and TGSE-BLADE. These findings support the use of PSF-EPI as a robust technique for intracranial tumor imaging, particularly when lesion conspicuity and anatomical delineation are clinically prioritized.
Objective To characterize glymphaticu2010 and cerebrospinal fluid (CSF)u2010related imaging alterations in moderateu2010tou2010advanced, deep brain stimulation (DBS)u2010eligible Parkinsonu2019s disease (PD) using a multidimensional magnetic resonance imaging (MRI) framework. Methods We studied 60 moderateu2010tou2010advanced, DBSu2010eligible patients with PD and 30 frequencyu2010matched healthy controls with 3.0 T MRI. The u201CSourceu2010Dynamicsu2010Functionu2010Structureu201D framework integrated choroid plexus volume fraction, C1u2013C2 phaseu2010contrast MRI, diffusion tensor image analysis along the perivascular space index, and perivascular space volumetry. Multivariable analyses were used to examine associations between imaging biomarkers and motor severity, anxiety, and sleep quality. Results Patients with PD showed reduced diffusion tensor image analysis along the perivascular space index, a nominal increase in choroid plexus volume fraction, and a lowu2010netu2010flow CSF pattern with nominal evidence of increased reflux and marked spatial heterogeneity, despite preserved gross perivascular morphology. Ventral CSF dynamics showed significant partial correlations with motor severity, whereas selected dorsal flow metrics remained significantly associated with anxiety severity in covariateu2010adjusted regression analyses. The combined logistic model showed modest diagnostic performance (area under the curve = 0.686), with the highest area under the curve numerically but no statistically significant advantage over individual imaging metrics in paired DeLong comparisons. Conclusion This framework provides an exploratory neuroimaging reference for moderateu2010tou2010advanced, DBSu2010eligible PD and may support future validation studies and DBSu2010related investigations.
Objective: To characterize glymphatic- and cerebrospinal fluid (CSF)-related imaging alterations in moderate-to-advanced, deep brain stimulation (DBS)-eligible Parkinson’s disease (PD) using a multidimensional magnetic resonance imaging (MRI) framework. Methods: We studied 60 moderate-to-advanced, DBS-eligible patients with PD and 30 frequency-matched healthy controls with 3.0 T MRI. The “Source-Dynamics-Function-Structure” framework integrated choroid plexus volume fraction, C1–C2 phase-contrast MRI, diffusion tensor image analysis along the perivascular space index, and perivascular space volumetry. Multivariable analyses were used to examine associations between imaging biomarkers and motor severity, anxiety, and sleep quality. Results: Patients with PD showed reduced diffusion tensor image analysis along the perivascular space index, a nominal increase in choroid plexus volume fraction, and a low-net-flow CSF pattern with nominal evidence of increased reflux and marked spatial heterogeneity, despite preserved gross perivascular morphology. Ventral CSF dynamics showed significant partial correlations with motor severity, whereas selected dorsal flow metrics remained significantly associated with anxiety severity in covariate-adjusted regression analyses. The combined logistic model showed modest diagnostic performance (area under the curve = 0.686), with the highest area under the curve numerically but no statistically significant advantage over individual imaging metrics in paired DeLong comparisons. Conclusion: This framework provides an exploratory neuroimaging reference for moderate-to-advanced, DBS-eligible PD and may support future validation studies and DBS-related investigations.
Central nervous system germ cell tumors (CNS GCTs) are common malignancies of the central nervous system of children and adolescents. The management of CNS GCTs is complex because of varied tumor sites, clinical presentations, treatments and outcomes. Multidisciplinary integrated treatment involving radiotherapy, chemotherapy and surgery is the most commonly used approach for CNS GCTs worldwide and is essential for achieving favorable clinical outcomes. The Pediatric Oncology Committee of the Chinese Anti-Cancer Association (CACA) has formulated the guidelines for the management of CNS GCTs based on domestic and foreign clinical studies and experiences. The guidelines cover the diagnosis and treatment of primary CNS GCT in the pineal region, the sellar region, and the thalamus and basal ganglia region, recurrent, disseminated, and bifocal/multifocal CNS GCT, as well as traditional Chinese medicine. In these guidelines, different treatment regimens are proposed for secretory or non-secretory GCTs. The therapeutic concepts of primary treatment and second-look surgery are also introduced. The hope is to help standardize the diagnosis and treatment, and further improve the outcomes of CNS GCTs patients.
Early-onset Schizophrenia (EOS) is a profoundly progressive psychiatric disorder characterized by both positive and negative symptoms, whose pathogenesis is influenced by genes, environment and brain structure development. In this study, the MIND (Morphometric Inverse Divergence) network was employed to explore the relationship between morphological similarity and specific transcriptional expression patterns in EOS patients. This study involved a cohort of 187 participants aged between 7 and 17 years, consisting of 97 EOS patients and 90 healthy controls (HC). Multiple morphological features were used to construct the MIND network for all participants. Furthermore, we explored the associations between MIND network and brain-wide gene expression in EOS patients through partial least squares (PLS) regression, shared genetic predispositions with other psychiatric disorders, functional enrichment of PLS weighted genes, as well as transcriptional signature assessment of cell types, cortical layers, and developmental stages. The MIND showed similarity differences in the orbitofrontal cortex, pericalcarine cortex, lingual gyrus, and multiple networks in EOS patients compared to HC. Moreover, our exploration revealed a significant overlap of PLS2 weighted genes linking to EOS-related MIND differences and the dysregulated genes reported in other psychiatric diseases. Interestingly, genes correlated with MIND changes (PLS2-) exhibited a significant enrichment not only in metabolism-related pathways, but also in specific astrocytes, cortical layers (specifically layer I and III), and posterior developmental stages (late infancy to young adulthood stages). However, PLS2+ genes were primarily enriched in synapses signaling-related pathways and early developmental stages (from early-mid fetal to neonatal early infancy) but not in special cell types or layers. These findings provide a novel perspective on the intricate relationship between macroscopic morphometric structural abnormalities and microscopic transcriptional patterns during the onset and progression of EOS.
Objective To research new methods and materials for cranioplasty in children with various types of skull defects. Methods and Results A total of 40 childrens with different locations, sizes and reasons of skull defects underwent surgery using 3 classes of homogeneous yet heterogeneous biomimetic hydroxyapatite collagen bone materials developed by Tsinghua University. These include porous mineralized collagen scaffolds (pMC), compact mineralized collagen scaffolds (cMC), and biphasic mineralized collagen composite scaffolds (bMC). Cranioplasty was successful in all cases, with no serious postoperative complications and good recovery outcomes. After 3 months to one year of follow-up, there was no displacement of the implants, the shape of the head was normal, the incisions healed well. Reexamination CT showed that the biomimetic hydroxyapatite collagen bone material implants were not shifted, and imaging fusion was partially achieved. The pMC CT HU-value was 300-400 HU, and the cMC CT HU - value was 700-900 HU, and the CT HU - value was close to normal bone tissue over time. Conclusions The use of different types of biomimetic hydroxyapatite collagen composites allows for personalized cranial reconstruction based on individual needs, and has been proven to possess good bio- compatibility and osteoinductive capacity. This approach is thus promising for research and clinical applications, with significant value.
PurposeThis study aims to summarize the characteristics of children under three years old (≤3 years) with central nervous system (CNS) tumors and to investigate the factors that influence their overall survival (OS) time.MethodsWe treated 171 pediatric patients (≤3 years) with CNS tumors at Yuquan Hospital of Tsinghua University from January 2016 to June 2023. Of these, 162 cases were successfully followed up. Kaplan–Meier survival analysis and Cox regression were utilized to evaluate factors potentially influencing OS of malignancies.ResultsThere was a male predominance among the patients. The three most common tumors were embryonal tumors, gliomas, and craniopharyngiomas. Gross total resection (GTR) was achieved in select cases. Patients with high-grade malignancies were advised to undergo chemotherapy and/or radiotherapy after surgery. Optic gliomas and diffuse midline gliomas were partially resected and treated with adjuvant treatments. The median survival time of low-grade malignant tumors was 41.5 months, while that of high-grade malignant tumors was 15 months. Kaplan–Meier survival analysis identified the factors potentially influencing OS of malignancies: extent of resection, CNS WHO grade, grade of malignancies, and Ki-67 labeling index (Ki-67 LI). Subsequent multivariate analysis highlighted the interactive factor (extent of resection × CNS WHO grade) along with Ki-67 LI, as the most significant variables. Factors such as sex, age, tumor location, and onset-to-treatment time appeared not to affect OS.ConclusionsGTR remains the cornerstone of treatment for children (≤3 years) with CNS tumors, except for optic glioma, diffuse midline glioma, and germinoma. The interactive factor (extent of resection × CNS WHO grade) and Ki-67 LI are the most significant factors affecting OS. The implementation of preoperative neoadjuvant chemotherapy and early postoperative chemotherapy may enhance prognosis.
Background: To create and validate nomograms for the personalized prediction of survival in octogenarians with newly diagnosed nonsmall‐cell lung cancer (NSCLC) with sole brain metastases (BMs). Methods: Random forests (RF) were applied to identify independent prognostic factors for building nomogram models. The predictive accuracy of the model was evaluated based on the receiver operating characteristic (ROC) curve, C‐index, and calibration plots. Results: The area under the curve (AUC) values for overall survival at 6, 12, and 18 months in the validation cohort were 0.837, 0.867, and 0.849, respectively; the AUC values for cancer‐specific survival prediction were 0.819, 0.835, and 0.818, respectively. The calibration curves visualized the accuracy of the model. Conclusion: The new nomograms have good predictive power for survival among octogenarians with sole BMs related to NSCLC.
BACKGROUND:Despite different intracranial tumour subtypes varying largely in their prognoses and recommended treatment regimens, they can have markedly similar appearances on standard radiology, especially in paediatric patients where they tend to occur in the midline. There is a need for a non-invasive, accurate method of determining tumour diagnosis to help expedite treatment planning. Existing studies have found magnetic resonance spectroscopy (MRS) to have value in diagnosing intracranial tumours in adults. The aim of this study was to investigate whether MRS could be accurate in diagnosing and grading paediatric intracranial tumours. METHODS:The hospital database was retrospectively searched for paediatric intracranial tumour patients ≤18 years that had 1.5 T MRS data available. Medical and demographic data were collected from existing records including MRS metabolites N-acetylaspartate (NAA), creatine (Cr), and choline (Cho), and final histopathologic diagnosis. MRS metabolites were then statistically compared against final histopathologic diagnosis. RESULTS:In total, 166 patients were included. In the overall cohort, the tumour to control tissue Cr ratio was significantly higher in grade 1 than grade 4 tumours (P=0.03), and tumour Cho/Cr was significantly higher in grade 4 than grade 1 tumours (P=0.004). When analyzing tumour subtypes, control tissue Cr was significantly higher in embryonal/germ cell tumours than glial tumours (P=0.044). Binary logistic regression models including MRS metabolite ratios and age, sex, and tumour location covariates could diagnose grade 4 tumours [area under the curve (AUC) =0.857], and grade 1 tumours (AUC =0.766) with reasonable accuracy. CONCLUSIONS:This study suggests that MRS has benefits in the non-invasive diagnosis of paediatric intracranial tumours, in particular, identifying low- and high-grade tumours. Future advances in MRS technology, and larger cross-sectional studies will be necessary to improve the clinical integration of MRS for accurate non-invasive paediatric intracranial tumour diagnosis.
Since the first Chinese pediatric neurosurgery meeting was convenced in 2002, the pediatric neurosurgery discipline in China has undergone remarkable transformation and growth. Throughout this period, both general and pediatric hospitals have progressively established dedicated Pediatric Neurosurgery Departments. Additionally, the methodology for training medical professionals in this field has undergone a substantial evolution, shifting from the conventional pediatric surgery training paradigm to a comprehensive system tailored for neurosurgical specialists and postgraduate students. Consequently, there has been a consistent increase in the number of physicians specializing in pediatric neurosurgery nationwide. In relation to surgical instruments and equipment, significant advancements have been achieved in pediatric neurosurgery. Notably, the neurosurgical operating microscope has emerged as a vital tool, significantly enhancing the therapeutic outcomes for a wide range of pediatric neurosurgical conditions. Furthermore, both the Chinese Medical Doctor Association and the Chinese Medical Association have established specialized pediatric neurosurgery subgroups, providing robust support for the continuous development and advancement of this field in China.
Background: Cognitive impairment mechanisms in children with preoperative brain tumors are not well understood. This study aimed to determine the correlation between the changes of resting-state functional magnetic resonance imaging (rs-fMRI) and the fourth edition of the Wechsler Intelligence Scale for Children (WISC-IV) in patients with brain tumors before surgery and in healthy controls (HCs). Methods: rs-fMRI data were acquired using 3-T magnetic resonance imaging (MRI) scanners for 21 patients with pediatric brain tumor and 19 age- and gender-matched HCs. The data of WISC-IV were collected by psychiatrists. We used chi-square tests and two-sample t-tests to identify clinical features with significant associations before surgery. A two-sample t-tests was used to identify brain regions with significant changes in regional homogeneity (ReHo) before surgery in patients. Pearson correlation coefficients were used to assess the relationship between changes in ReHo and the five measures in the WISC-IV. Results: The ReHo values were significantly decreased in the left anterior cingulate (T=-4.391) and right middle frontal gyrus (MFG) (T=-5.130) in patients compared to controls. Notably, ReHo values in the right MFG showed a positive correlation with the Perceptual Reasoning Index (R=0.471; P=0.031) and Working Conclusions: The study identified significant ReHo alterations in patients with pediatric brain tumor, primarily in brain regions associated with cognitive processing, and revealed a positive correlation between these alterations and specific cognitive functions. These findings contribute to understanding cognitive impairments in this patient group and suggest potential areas for targeted intervention.
Background This study aimed to investigate the neuroanatomical subtypes among early-onset schizophrenia (EOS) patients by exploring the association between structural alterations and molecular mechanisms using a combined analysis of morphometric similarity network (MSN) changes and specific transcriptional expression patterns. Methods We recruited 206 subjects aged 7 to 17 years, including 100 EOS patients and 106 healthy controls (HC). Heterogeneity through discriminant analysis (HYDRA) was used to identify the EOS subtypes within the MSN strength. The differences in morphometric similarity between each EOS subtype and HC were compared. Furthermore, we examined the link between morphometric changes and brain-wide gene expression in different EOS subtypes using partial least squares regression (PLS) weight mapping, evaluated genetic commonalities with psychiatric disorders, identified functional enrichments of PLS-weighted genes, and assessed cellular transcriptional signatures. Results Two distinct MSN-based EOS subtypes were identified, each exhibiting different abnormal MSN strength and cognitive functions compared to HC. The PLS1 score mapping demonstrated anterior–posterior gradients of gene expression in EOS1, whereas inverse distributions were observed in EOS2 cohorts. Genetic commonalities were identified in autistic disorder and adult schizophrenia with EOS1 and inflammatory bowel diseases with EOS2 cohorts. The EOS1 PLS1- genes (Z < -5) were significantly enriched in synaptic signaling-related functions, whereas EOS2 demonstrated enrichments in virtual infection-related pathways. Furthermore, the majority of observed associations with EOS1-specific MSN strength differences contributed to specific transcriptional changes in astrocytes and neurons. Conclusions The findings of this study provide a comprehensive analysis of neuroanatomical subtypes in EOS, shedding light on the intricate relationships between macrostructural and molecular aspects of the EOS disease.
The coordination between neurogenesis and angiogenesis plays an important role in nerve tissue development and regeneration. Recently, using bioactive materials to drive neurogenic and angiogenic responses has gained increasing attention. Understanding the neurovascular link between regulatory cues offers valuable insight into the mechanisms underlying nerve regeneration and the design of new bioactive materials. In this study, we utilized a dual-functionalized peptide nanofiber hydrogel presenting the brain-derived neurotrophic factor and vascular endothelial growth factor mimetic peptides RGIDKRHWNSQ (RGI) and KLTWQELYQLKYKGI (KLT) to construct an artificial neurovascular microenvironment. The dual-functionalized peptide nanofiber hydrogel enhanced the neurite outgrowth of pheochromocytoma (PC12) cells and tube-like structures formation of human umbilical vein endothelial cells (HUVECs) in vitro , and promoted rapid lesion infiltration of neural and vascular cells in a rat brain injury model. Using indirect co-culture models, we found that the dual-functionalized peptide hydrogel effectively mediated neurovascular crosstalk by regulating secretion of paracrine factors from PC12 cells and HUVECs. When the two cells types were directly co-cultured on the dual-functionalized peptide hydrogel, the efficiency of cell-cell communication was enhanced, which further accelerated the differentiation and maturation of PC12 cells with an increased number of pseudopodia and spread morphology, and HUVECs tube-like structure formation. In summary, the dual-functionalized peptide nanofiber hydrogel successfully formed an artificial neurovascular niche to directly regulate the behaviors of neural and vascular cells and promote their neurovascular crosstalk through paracrine signaling and direct cell-cell contact.
Purpose: To predict the prognosis of craniopharyngioma in children by optical coherence tomography angiography (OCTA).Methods: We evaluated if the relationship between preoperative OCTA of the choroidal capillary density (CCD) and visual outcome continued over long-term visual recovery in 38 patients undergoing craniopharyngioma resection. Patients were evaluated 3 times: 1 week before surgery (Visit1), followed-up at 6–10 weeks (Visit2), and 9–15 months (Visit3) after surgery.Results: In total 38 patients (70 eyes) with craniopharyngiomas, which included 20 (52.6%) boys and 18 (47.4%)girls, the mean age was 11.8 ± 2.7 years (range: 6–18 years). The age (p = 0.71), gender (p = 1.00), mean refractive error (p = 0.55), and axial length (p = 0.23) of 38 normal volunteers (76 eyes) were matched. After surgery, the cross-compression of patients was relieved. The average visual acuity change in the normal CCD group was 0.07 ± 0.02; the average visual acuity change in the low CCD group was 0.01 ± 0.01, p < 0.001. Preoperative CCD value is related to the preoperative BCVA (p < 0.001), and the visual function after the long-term follow-up (9–15 months) (p < 0.001). The prognosis of CCD has the same trend as the BCVA. Further correlation analysis shows that CCD and BCVA are significantly correlated (r = 0.878; p < 0.001). CCD has a weak but significant correlation both with MD (r = 0.19; p < 0.001) and PSD (r = −0.21; p <0.001). A natural cutoff of CCD is approximately 38%. With the normal CCD group the maximum improvement of BCVA exceeds 0.3 post-operatively, compared to eyes in the low CCD group that improve by <0.03, and worse after surgery.Conclusions: Long-term vision recovery after surgical decompression of craniopharyngiomas in children can be predicted by preoperative by OCTA. Patients with normal CCD before surgery showed a tendency to improve vision; this trend of improvement persisted in subsequent follow-ups. The CCD baseline natural cutoff value for predicting visual prognosis before and after surgery is about 38%.
Radial glia (RG) cells that align in parallel in the embryonic brain are found to be able to guide the directed migration of neurons in response to brain injury. Therefore, biomaterials with aligned architectures are supposed to have positive effects on neural migration and neurogenic differentiation for brain injury repair that are rarely addressed, although they have been widely demonstrated in spinal cord and peripheral nerve system. Here, we present a highly biomimetic scaffold of aligned fibrin hydrogel (AFG) that mimics the oriented structure of RG fibers. Through a combination of histological, behavioral, imaging, and transcriptomic analyses, we demonstrated that transplanting the AFG scaffold into injured cortical brains promotes effective migration, differentiation, and maturation of endogenous neural stem cells, resulting in neurological functional recovery. Therefore, this study will light up a new perspective on applying an aligned scaffold to promote cortical regeneration after injury by inducing endogenous neurogenesis.
目的 探讨儿童脑膜瘤的特性和诊疗进展,为治疗提供新的视点.方法 回顾性分析清华大学玉泉医院神经外科2014年1月—2022年4月收治的9例经手术病理证实为脑膜瘤患儿的临床资料.结果 患儿最常见的临床症状是偏瘫、呕吐.所有患儿术前均行头颅CT、MRI检查,脑组织标本均经病理诊断,其中包括2例内皮型脑膜瘤,2例纤维型脑膜瘤,1例砂粒型脑膜瘤,1例血管瘤型脑膜瘤,1例横纹肌样脑膜瘤,1例间变型脑膜瘤,1例非典型脑膜瘤.9例患者中7例全切除,2例次全切除(其中Simpson Ⅰ级2例,Ⅱ级3例,Ⅲ级2例,Ⅳ级2例),术后无手术相关偏瘫或其他肢体功能障碍,无术后颅内血肿.到目前为止,其中2例复发,且均为WHO Ⅲ级脑膜瘤.结论 相比于成人,儿童脑膜瘤发病率低,可能与放射治疗和神经纤维瘤病2型关系密切,病理恶性度高,手术全切除是治疗儿童脑膜瘤的首选方法,对于3岁以上未能全切患儿应当考虑术后放疗,可延长生存期、降低肿瘤复发率.
Objective To investigate the indication and significance of preoperative chemotherapy for pediatric patients with brain malignant tumors. Methods and Results The clinical data of 9 pediatric patients with brain malignant tumors who were admitted to Department of Neurosurgery, Tsinghua University Yuquan Hospital from January 2017 to August 2019 were retrospectively analyzed. The patients were 6 males and 3 females, aged from 1.50 months to 6 years. Among the 9 cases, 5 cases were located in the pineal region, one case in the third ventricle, one case in the frontotemporal and the basal ganglia region and 2 cases in the fourth ventricle. The preoperative clinical diagnosis of the 9 patients included 5 cases of germ cell tumor, 2 cases of medulloblastoma, one case of pineal blastoma and one case of embryonal tumor. Before the chemotherapy, 6 patients were treated with ventriculo⁃peritoneat shunt, 2 patients with ventricular puncture and external drainage, and one patient with endoscopic third ventriculostomy. Before surgery, 2 cases were given carboplatin+etoposide regimen, 4 cases were given cisplatin+etoposide regimen, one case was given carboplatin+etoposide+isophoramide regimen, and 2 cases were given cisplatin+etoposide+isophoramide regimen. 6 patients received one cycle of chemotherapy before tumor resection, 2 patients received 2 cycles and one patient received 3 cycles. All of the patients received tumor resection after 1-3 cycles of chemotherapy. The following treatments were depended on the pathological results. After preoperative chemotherapy, tumors' volume decreased in 7 cases while increased in one case. Additionally, there was one case of tumor apoplexy. The blood supply of tumors of all the 9 patients was not abundant. Among the 9 cases, total removal was achieved in 8 cases and subtotal in one case. The pathological results revealed 3 germ cell tumors, 2 pineoblastomas, 2 medulloblastomas, one diffuse midline glioma, and one atypical teratomoid/rhabdomyoid tumor (AT/RT). The 9 patients were followed up from 8 to 38 months (mean 18 months). Three patients died of tumor recurrence and there were no tumor recurrences in the other 6 patients. Conclusions Preoperative chemotherapy may be considered for children with brain malignant tumors which are clinically sensitive to chemotherapy at high risk of surgery. Preoperative chemotherapy for brain tumors in infants and young children was effective in reduction of tumor volume and vascularity, which significantly facilitated maximal tumor resection.
选取2015年5月至2016年4月清华大学玉泉医院神经外科收治的5例脊髓病变患者为研究对象,进行MRI相关数据的采集,分析弥散张量成像(DTI)的各向异性分数(FA)值在脊髓髓内病变和髓外病变的改变情况,以及脊髓纤维束受压情况。与传统的单次激发DTI相比,多次激发DTI成像清晰,无变形扭曲。病变周边FA值的改变可用于辅助评估脊髓受损的严重程度。FA值在脊髓髓外病变和髓内病变中存在较大差异,有助于两者的鉴别诊断。脊髓弥散张量纤维素成像可用于辅助脊髓髓内病变手术入路的选择以及预判病变的切除程度。