OBJECTIVE:Fatty filum terminale (FFT) is a common cause of tethered cord syndrome (TCS) in children. Traditional detethering often requires laminectomy, which increases surgical trauma and complications. This study aimed to evaluate the safety and efficacy of a minimally invasive keyhole interlaminar approach for FFT. METHODS:The study is a retrospective study. We analyzed 17 pediatric patients with symptomatic FFT who underwent keyhole interlaminar detethering between January 2020 and February 2021. A 1-2 cm midline incision was made at the L3-L4 interlaminar space to establish a working channel without bony removal. Microsurgical detethering was performed under neurophysiological monitoring. Preoperative and postoperative magnetic resonance imaging was used to assess detethering, and neurological function was evaluated using the Spina Bifida Neurological Scale. RESULTS:All 17 patients underwent successful surgery with no intraoperative complications or conversions to open procedures. Postoperative magnetic resonance imaging confirmed complete filum transection. The mean follow-up duration was 33 ± 5 months. The median Spina Bifida Neurological Scale score was 14 preoperatively and 15 at the final follow-up. After surgery, complete recovery was achieved in 14 patients (82%), while 3 patients (18%) experienced recovery with mild residual deficits. No late complications were observed in the follow up visit. CONCLUSIONS:The keyhole interlaminar approach is a safe and effective technique for FFT detethering in children. It offers reduced surgical trauma, faster recovery, and satisfactory neurological improvement, representing a promising alternative to conventional surgery.
To investigate the clinical characteristics, treatment strategies, and prognosis of Brainstem Lesion (BSL) in children with neurofibromatosis type 1 (NF1). A retrospective analysis was conducted on the clinical data of 269 children with NF1 admitted to Children's Hospital of Soochow University from July 2016 to March 2025. All children underwent cranial magnetic resonance imaging (MRI), and BSL was diagnosed based on imaging findings. The incidence, clinical characteristics, disease progression, and prognosis of NF1-associated BSL were analyzed. Family trio whole exome sequencing was performed in 7 of 11 children with NF1-associated BSL to explore the correlation between NF1 genotype and clinical phenotype. Among 269 children with NF1, 11 (4.1
BackgroundTraumatic brain injury is a major cause of death and disability in children, and early identification of high-risk cases is critical for improving clinical outcomes.ObjectiveThis study aimed to develop and validate a clinical prediction model to estimate the 30-day in-hospital mortality in pediatric patients with moderate-to-severe traumatic brain injury (msTBI).MethodsA retrospective analysis was conducted on 289 pediatric patients admitted with msTBI. Independent risk factors were identified using the least absolute shrinkage and selection operator regression and multivariable logistic regression analysis to construct a clinical nomogram. Model performance was assessed using ROC curves, bootstrap validation, and decision curve analysis.ResultsThe median age of the cohort was 5.17 (IQR, 2.75–9.33) years. There were 101 females and 188 males. Four independent predictors were identified: Glasgow Coma Scale score, lactic acid, albumin, and trauma-induced coagulopathy. The model showed AUC of 0.898 (95% CI: 0.896, 0.899) and good agreement between predicted and observed outcomes. Hosmer-Lemeshow test yielded a non-significant P-value (P = 0.475), supporting good model calibration. Clinical decision analysis demonstrated that the threshold probability ranged from 0 to 0.95.ConclusionThis study developed a reliable clinical tool to predict 30-day in-hospital mortality in children with msTBI. It may support early risk stratification and assist clinicians in making informed treatment decisions.
To delineate the incidence, independent determinants, and prognostic impact of trauma-induced coagulopathy (TIC) following pediatric moderate-to-severe traumatic brain injury (msTBI). Children with msTBI admitted to the Children’s Hospital of Soochow University during 2016–2024 were retrospectively enrolled. Logistic regression was first used to identify predictors of TIC. Kaplan–Meier (KM) curves were then constructed to visualize the influence of TIC on in-hospital and 28-day mortality. Univariable and multivariable models were employed to estimate the association between TIC and survival, with adjustment for demographics, clinical presentation, and imaging characteristics. Among 319 msTBI children, TIC is diagnosed in 173 (54.2
The relationship between lipid profiles and intracranial hemorrhage (ICH) has garnered increasing attention. The ratio of low-density lipoprotein to high-density lipoprotein (LHR) is one of the key lipid profile indices. However, studies investigating the association between LHR and the prognosis of critically ill ICH patients remain limited. Data for this study were obtained from the MIMIC-IV 3.1 database. Initially, the association between LHR and short-term outcomes in ICH patients, including ICU mortality, in-hospital mortality, and 28-day mortality, was analyzed using Cox regression in both continuous and categorical models. Additionally, restricted cubic spline (RCS), subgroup, and sensitivity analyses were conducted to further validate our findings. The study included 873 critically ill ICH patients, among whom 20.3
The mitogen-activated protein kinase (MAPK) signaling pathway plays roles in cell proliferation, differentiation, and apoptosis, all crucial for cellular transformation. It’s no surprise that MAPK alterations are prevalent in numerous tumors. Several critical genes in the MAPK signaling pathway, including BRAF, FGFR, and NF1, are mutated in brain tumors. For example, FGFR1 mutation or rearrangement has been described in pilocytic astrocytoma, diffuse astrocytoma, and dysembryoplastic neuroepithelial tumor (DNT). These MAPK-activated brain tumors are benign and seldom progress to malignancies, with the mechanisms driving this rare transformation not yet fully understood. In this study, we present two cases of high-grade glioma characterized by a single activating mutation of FGFR1 and massive chromosome loss (near-haploid genome). Similar haploidy is found in 3 additional high-grade astrocytoma by literature review, all harbor a single gene mutation in the MAPK pathway. We propose that the massive chromosome loss might serve as a significant mechanism contributing to the unusual malignant transformation of benign brain tumors activated by the MAPK pathway.
Infants and toddlers with mild traumatic brain injury (mTBI) and minor subdural hematoma (SDH) were found to have a higher risk of requiring neurosurgical intervention (NI). However, the ability to identify patients with mTBI and minor SDH who require NI remains limited. This study aims to develop a nomogram to predict NI in these patients. A nomogram predicting NI was established using demographic, clinical, radiographic, and laboratory data from patients with mTBI and minor SDH. The least absolute shrinkage and selection operator (LASSO) regression and best subsets regression (BSR) methods were employed to identify variables and select predictive factors. A nomogram was constructed using multivariable logistic regression. The model's performance was evaluated using the area under the receiver operating characteristic curve, calibration curves, the Hosmer-Lemeshow test, and decision curve analysis. Immediate seizures, anemia, and subarachnoid space depth were identified as significant predictive factors by the BSR, leading to the development of a nomogram. The AUC for this nomogram, obtained through bootstrap validation (resampling = 500), was 0.893 (95% CI, 0.844-0.942). The model demonstrated good calibration, and decision curve analysis showed that when the threshold probability ranged from 7 to 83%, using the nomogram to predict NI provided a net benefit. A novel nomogram has been developed to accurately assess the risk of NI in children under 3 years of age with mTBI and minor SDH, potentially aiding in clinical decision-making.
BackgroundElevated glycemic variability (GV) is commonly observed in intensive care unit (ICU) patients and has been associated with clinical outcomes. However, the relationship between GV and prognosis in ICU patients with hemorrhagic stroke (HS) remains unclear. This study aims to investigate the association between GV and short- and long-term all-cause mortality.MethodsClinical data for hemorrhagic stroke (HS) patients were obtained from the MIMIC-IV 3.1 database. GV was quantified using the coefficient of variation (CV), calculated as the ratio of the standard deviation to the mean blood glucose level. The association between GV and clinical outcomes was analyzed using Cox proportional hazards regression models. Additionally, restricted cubic spline (RCS) curves were employed to examine the nonlinear relationship between GV and short- and long-term all-cause mortality.ResultsA total of 2,240 ICU patients with HS were included in this study. In fully adjusted models, RCS analyses revealed a U-shaped association between the CV and both short- and long-term all-cause mortality (P for nonlinearity < 0.001 for all outcomes). Two-piecewise Cox regression models were subsequently applied to identify CV thresholds. The thresholds for all-cause mortality in ICU, during hospitalization, and at 30, 90, and 180 days were determined to be 0.14, 0.16, 0.155, 0.14, and 0.14, respectively. These findings were consistent in sensitivity and subgroup analyses.ConclusionsIn HS patients, higher GV is associated with an increased risk of both short- and long-term all-cause mortality. Our findings suggest that stabilizing GV may improve the prognosis of HS patients.
The existing literature on subdural hematomas (SDH) primarily focused on surgically treated cases. However, minor SDH remain unexplored. This study aims to investigate the association between subarachnoid space depth and neurosurgical intervention in children with minor SDH. Patients (age < 3 years) with minor SDH between June 2015 and June 2024 at the Children’s Hospital of Soochow University were included. Patients with subarachnoid space depth ≥ 3 mm were classified into the ESS group. In total, 277 patients with minor SDH were included, of whom 100 (36.1
[This corrects the article DOI: 10.3389/fonc.2025.1694881.].
OBJECTIVE: Acute rupture and hemorrhage of pediatric brain arteriovenous malformations (AVMs) may lead to cerebral herniation or intractable intracranial hypertension, necessitating emerging surgical interventions to alleviate intracranial pressure. However, there is still controversy regarding the timing of treatment for ruptured AVMs. This study aimed to assess the feasibility of utilizing three-pillar expansive craniotomy (3PEC) at different times during the treatment of pediatric ruptured supratentorial AVMs. METHODS: A retrospective analysis was conducted on all consecutive cases of acute rupture in supratentorial AVM children who underwent 3PEC at a single institution from 2020 to 2022. General information, clinical characteristics, radiological data, and prognosis were reviewed and analyzed. RESULTS: Thirteen children were included in the analysis. The intracranial pressure of all patients decreased to below 15 mmHg within 10 days. The expansion volume of the cranial cavity of the patients increased by 18.3 cm(3)(95% confidence interval, 10.2-26.3;P<0.001) compared to the hematoma volume. None of the patients required decompressive craniectomy due to intractable intracranial hypertension caused by cerebral swelling. The median waiting period for patients with delayed AVMs treatment was 8 days, during which no rebleeding occurred. CONCLUSIONS: Emergency intervention with 3PEC in children experiencing acutely ruptured supratentorial AVMs appears to be feasible. For children requiring delayed management of the AVMs, 3PEC may diminish the risk of rebleeding during the waiting period and shorten the waiting period.
An increased incidence of brain abscesses was observed post-COVID-19 pandemic. However, it remains unclear how the COVID-19 pandemic influenced the epidemiology of brain abscesses. This study aimed to investigate changes in the epidemiology of brain abscesses pre- and post-COVID-19 pandemic. A retrospective study of demographic, clinical, radiological, and laboratory characteristics of patients with brain abscesses in Children's Hospital of Soochow University from 2015–2023 was performed. A total of 34 patients were admitted to the hospital during the study. The post-COVID-19 cohort had an average of 5.5 cases/year, which is a 129.2
OBJECTIVE: The link between tethered cord syndrome (TCS) and neurofibromatosis type 1 (NF1) remains unclear. To date, only 2 studies have indicated a high occurrence of TCS in the pediatric NF1 patient population. Our study aims to ascertain the incidence of TCS among Chinese pediatric patients with NF1. METHODS: A single-institution, 8-year retrospective analysis at a tertiary-level children's hospital was conducted for patients with NF1 who underwent surgery for TCS. The clinical features, imaging characteristics, genetic testing outcomes, and histopathological findings of pediatric NF1 patients with TCS were analyzed. RESULTS: During the study period, a total of 115 pediatric patients were diagnosed with NF1. Among these 115 NF1 patients, 11 individuals, accounting for 9.6% of the sample, were confirmed to have TCS. This rate of occurrence is significantly higher than the incidence rate of TCS in the general population, which stands at 0.17%. All 11 of the pediatric NF1 patients underwent surgery for TCS. Out of the 11 patients who were advised to have tethered cord release surgery, 4 (36.4%) were girls, and the average age of these 11 patients was 8.6 years, with ages ranging from 2 to 17 years. The conus medullaris position of the 11 pediatric NF1 patients ranged from L1 to L2, and 10 of them (91%) had a filum terminale lipoma, characterized by high signal intensity on T1-weighted magnetic resonance images. Another patient was diagnosed with occult TCS. All 11 pediatric NF1 patients exhibited symptoms associated with TCS, such as neuromotor dysfunction, abnormalities in urination, defecation, skeletal system abnormalities, or pain. Eight of the 11 pediatric NF1 patients had undergone genetic testing, which is a crucial part of the diagnostic process for NF1. The histopathological examination of the 11 pediatric NF1 patients who underwent surgery revealed that 10 patients had fatty infiltration of the filum terminale, while the remaining patient exhibited fibrosis of the filum terminale. CONCLUSIONS: In the group of pediatric patients with NF1, the incidence of TCS, presenting to the pediatric neurosurgery clinic for any reason, is significantly higher than that of the general population. However, the pathophysiological relationship between NF1 and TCS is still not clear. It is essential to provide counseling to NF1 patients and their families about the symptoms of TCS to make sure they are aware and can seek the necessary care when needed. This underscores the importance of not only diagnosing NF1 but also staying alert for related conditions like TCS, which may have a higher occurrence in this demographic.
OBJECTIVE: Areas of increased signal intensity, known as T2 hyperintensities (T2Hs), observed on T2-weighted magnetic resonance imaging (MRI) scans, are linked to a spectrum of brain abnormalities in children with neurofibromatosis type 1 (NF1). Defining the radiological characteristics that distinguish non-neoplastic from neoplastic T2Hs in children with NF1 is crucial. Then, we could identify lesions that were most likely to require oncologic surveillance. METHODS: We conducted a single-center retrospective review of all available brain MRIs from 98 children with NF1 and 50 healthy pediatric controls. All T2Hs identified on MRI were characterized based on location, imaging features, and the presence of lesion-related symptoms. Subsequently, all T2Hs were classified using newly established criteria and categorized into 3 distinct groups: low-risk tumor lesions, medium-risk tumor lesions, and high-risk tumor lesions. Lesions deemed to be high-risk will be recommended for surgical treatment. RESULTS: T2Hs were present in 61 (62.2%) individuals of the NF1 cohort. T2Hs were a highly sensitive (100%; 95% confidence interval 92.9%-100.0%) and specific (62.2%; 95% confidence interval 51.9%-71.8%) marker for the diagnosis of NF1. In children aged 4-10, the detection rate of T2Hs is significantly higher than in children under 4 years old and those aged between 10 and 18 (P < 0.05). T2Hs were most frequently located in basal ganglia, cerebellar hemispheres, and brainstem. During the follow-up process, none of the lesions categorized as low-risk or medium-risk tumor lesions progressed to high-risk tumor lesions. Seven patients had high-risk tumor lesions and underwent surgical treatment. The pathological assessment identified 5 cases of glioma among the 7 patients, along with 1 case of gliosis and 1 case of vascular dysplasia. CONCLUSIONS: Low-risk and medium-risk tumor lesions can both be classified as unidentified bright objects . Unidentified bright objects constituted the majority of T2Hs in children with NF1. High-risk tumor lesions should be considered as probable tumors. With the application of standardized radiologic criteria, a high prevalence of probable brain tumors will be identified in this at-risk population of children, which underscores the importance of vigilant and appropriate oncological surveillance to ensure timely detection and intervention for these tumors.
Objective:To explore the staged surgery for children with supratentorial arteriovenous malformation rupture and hemorrhage and evaluate the prognosis of staged treatment of arteriovenous malformation(AVM)in children.Methods:From January 2015 to December 2018, retrospective analysis was conducted for clinical data of 37 children with supratentorial cerebral arteriovenous malformation hemorrhage undergoing staged surgery.Clinical manifestations, surgical approaches and follow-up outcomes were recorded.Results:Emergency operation in the first stage was performed.There were intracranial hematoma evacuation( n=27)and extraventricular drainage( n=10). There was no mortality.After stabling, computed tomographic angiography(CTA)or digital subtraction angiography(DSA)was examined and the results were graded according to the Spetzler-Martin scheme.The grades were Ⅰ( n=12), Ⅱ( n=16)and Ⅲ( n=9). AVM resection was performed at Week 2 after an initial operation.DSA indicated that AVMs were completely removed at Month 6.Glasgow outcome scale(GOS)score at the end of follow-up was 5( n=32, 86.5%)and 4( n=5, 13.5%). No hemorrhage occurred during a follow-up period of(6-48)months. Conclusion:When preoperative cerebrovascular imaging cannot be completed due to certain conditions, staged treatment of supratentorial cerebral arteriovenous malformation rupture during acute phase is a feasible option for achieving a better prognosis with less neurological dysfunction in children.
目的 结合文献探讨胚胎发育不良性神经上皮肿瘤(dysembryoplastic neuroepithelial tumor,DNET)的临床特点及治疗策略.方法 回顾性分析苏州大学附属儿童医院神经外科2017年12月至2018年5月收治的5例胚胎发育不良性神经上皮肿瘤患儿临床资料,结合文献复习,总结胚胎发育不良性神经上皮肿瘤的临床表现、影像学特点、分类、治疗方法和预后.结果 5例患儿中4例表现为癫痫发作,1例表现为头痛、嗜睡等脑积水症状.术前CT和磁共振检查诊断为胚胎发育不良性神经上皮肿瘤,按影像学特点分类并经手术及病理检查确诊.根据影像学特点,5例中DNET Ⅰ型1例,Ⅱ型3例,Ⅲ型1例.1例Ⅰ型患儿行单纯额叶病灶切除术;2例Ⅱ型患儿行颞叶病灶+前内侧颞叶切除术;1例Ⅱ型患儿病灶侵犯功能区及基底节区,予病灶大部切除术;1例Ⅲ型患儿病灶位于脑室内,行脑室镜下肿瘤切除术.术后随访8~14个月,5例患儿Engel分级均为Ⅰ级,1例脑积水患儿术后症状改善.结论 DNET患儿常表现为癫痫,也可表现为脑积水等症状,临床表现与影像学分型相关,大部分患儿经单纯肿瘤切除或包含肿瘤的脑叶切除可获得良好治疗效果.
Abstract Background: We sought to define the radiologic features which could differentiate non-neoplastic from neoplastic T2 hyperintensities (T2Hs) identified on MRI in children with neurofibromatosis type 1(NF1) and identify lesions that most likely to require oncologic surveillance. Methods: We conducted a single-center retrospective review of all available brain MRIs from 49 children with NF1 and 50 healthy pediatric controls. All T2Hs identified on MRI were characterized based on location, presence of T1 hypointensity, mass effect, contrast enhancement, edma, cystic lesion, or lesion related symptoms. Subsequently, all T2Hs were classified using newly established criteria as either unidentified bright objects (UBOs) or probable tumors. Lesion classification was pathologically confirmed in 6 NF1 cases. Results: T2Hs were present in 35 (71.4%) individuals of the NF1 cohort. UBOs constituted the majority of T2Hs and were most frequently located in basal ganglia, cerebellar hemispheres, and brainstem. T2Hs of 7 NF1 patients were classified as probable tumors, and 6 children with probable tumors received surgical treatment. Five lesions of the six surgical children proved to be glioma. Conclusion: T2Hs has been found to be a highly sensitive and specific marker for the diagnosis of NF1. With the application of standardized radiologic criteria, a high prevalence of probable brain tumors will be identified in this at-risk population of children, of which nearly 70% require treatment, emphasizing the need for appropriate oncologic surveillance for NF1 patients with probable brain tumors.
Abstract Purpose Acute traumatic subdural effusion with hemorrhage (ATSEH) is a common type of head trauma in children. However, there is considerable variation in the previous literature regarding the clinical presentation, pathogenesis, and treatment strategies of ATSEH in children. The purpose of this study was to summarize the clinical features of ATSEH in children as well as to explore their treatment strategies. Methods There were 79 cases of ATSEH studied retrospectively at a pediatric teaching hospital over an 7-year period. The children ranged in age from 1 month to 3 years, with an average age of 8.51(4.33,11.00) months. We analyzed the relationship between the outcome of ATSEH treatment and factors such as age, sex, symptoms, signs, coagulation function, internal environmental homeostasis, and imaging materials. The mean follow-up time was 57.56(34,82) months. The mRS score was used for patient prognosis. Results 27 out of 79 (34.2%) children showed subtle findings. Impaired consciousness was observed in 26.4% of patients. 52 out of 79 (65.8%) children presented with overt neurological symptoms and signs, with vomiting was t the most common symptom. 51 of 79 children (64.6%) presented with increased fontanelle tone. 32 out of 79 (40.51%) patients required operative management. 73 of the 79 patients (92.4%) had a good prognosis. The patients in the surgical group showed reduced fibrinogen (<1.5g/L) in 53.1%, low hemoglobin (88.56±17.21) and hyponatremia (133.50±3.76). Conclusions Patients with an age of 9.07(4.31,8.30) presenting with increased impairment of consciousness and increased anterior fontanelle tone are the basis for surgical treatment. Through the treatment of patients with ATSEH, we have found that the essence of ATSEH is an impairment of cerebrospinal fluid circulation triggered by subdural hemorrhage, which leads to acute hydrocephalus. In patients with ATSEH with malignant cranial hypertension, the timely subdural drainage is the key to treating and reducing serious complications. Prevention and correction of hypofibrinogen and hyponatremia are important aspects of the treatment of ATSEH in children that cannot be ignored.
神经管连接部发育缺陷(JNTD)是一种较为罕见的中枢神经系统先天畸形,主要表现为脊髓形态和功能不连续。检索国内外文献仅见个案报道。本文报道了1例JNTD伴皮毛窦的患儿,行皮毛窦切除术+脊髓拴系松解术,术中证实为JNTD,术后随访3个月,术后无手术相关并发症及新发神经系统症状。