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Open versus endoscopic surgery with helmet molding therapy in non-syndromic patients with craniosynostosis: an updated systematic review and meta-analysis of clinical outcomes and treatment-related costs. 颅骨发育不良非畸形患者接受开放手术与内窥镜手术配合头盔成型疗法的比较:临床疗效和治疗相关费用的最新系统综述和荟萃分析。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-12-16 DOI: 10.1007/s00381-024-06692-2
Omar R Ortega-Ruiz, Mauricio Torres-Martínez, Mariana Villafranca-Cantú, Rebeca Alejandra Ávila-Cañedo, Emilio Piñeyro-Cantú, Eduardo Menchaca-Welsh, Nir Shimony, George I Jallo, Javier Terrazo-Lluch, J Javier Cuéllar-Hernández

Objective: There is an ongoing debate regarding the optimal treatment for craniosynostosis as diverse factors influence the election between endoscopic and open surgery. Previous evidence favors endoscopic procedures. However, evidence remains unfulfilled by a limited number of patients and clustered in very few centers worldwide making it difficult to define it as a replicable technique in different populations. In recent years, evidence regarding endoscopic-assisted procedures has gone through a considerable spurt showing an increased interest among surgeons globally showing optimal outcomes in different populations and centers. In this systematic review and meta-analysis, we performed an updated analysis of previous reviews, including only non-syndromic patients. We also seek to provide a summary of the tendency of treatment observed in the literature. Similarly, this is the first study to include total costs within its analysis.

Material and methods: Three previous meta-analyses published in 2018 yielded 11 eligible papers. We performed a systematic review and meta-analysis of the literature in MEDLINE and EMBASE databases through PubMed, Scopus, and Ovid to fill the gap of information between 2018 and 2024. Twenty-three total articles were included in the final analysis.

Results: Variables analyzed were baseline characteristics, length of stay, blood loss, transfusion rates and volume, operative time, and costs. The analysis of data concluded a younger age at surgery in patients undergoing endoscopic surgery (p ≤0.00001). Blood loss, transfusion rates, and volumes depicted favored outcomes for endoscopy with less blood loss during surgery (p ≤0.00001), operative time (p ≤0.00001), and transfusion rates (p ≤0.00001) as well as lower transfused volumes (p ≤0.00001).

Conclusion: Endoscopic surgery carries fewer complications than open surgery. Treatment-related costs are highly decreased in endoscopic procedures after including costs related to outpatient care. Open surgery can be considered in older children if no endoscope or experienced surgeons in endoscopic procedures are available.

目的:关于颅缝闭锁的最佳治疗方法一直存在争议,因为多种因素影响着内镜手术和开放手术之间的选择。先前的证据支持内窥镜手术。然而,由于患者数量有限,并且集中在世界范围内的极少数中心,因此很难将其定义为在不同人群中可复制的技术。近年来,关于内窥镜辅助手术的证据已经经历了相当大的井跃性增长,显示出全球外科医生对不同人群和中心的最佳结果的兴趣增加。在本系统综述和荟萃分析中,我们对之前的综述进行了更新分析,仅包括非综合征患者。我们也试图提供在文献中观察到的治疗趋势的总结。同样,这是第一个将总成本纳入分析的研究。材料和方法:2018年发表的三项荟萃分析产生了11篇符合条件的论文。我们通过PubMed、Scopus和Ovid对MEDLINE和EMBASE数据库中的文献进行了系统回顾和meta分析,以填补2018年至2024年之间的信息空白。最终分析共纳入23篇文章。结果:分析的变量包括基线特征、住院时间、出血量、输血率和输血量、手术时间和费用。数据分析表明,内镜手术患者手术年龄较低(p≤0.00001)。术中出血量、输血率和输血量较少(p≤0.00001)、手术时间(p≤0.00001)、输血率(p≤0.00001)以及输血量较低(p≤0.00001)是内镜检查的有利结果。结论:内镜手术并发症比开放手术少。在包括门诊护理费用后,内窥镜手术的治疗相关费用大大降低。如果没有内窥镜或经验丰富的外科医生,可以考虑对年龄较大的儿童进行开放手术。
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引用次数: 0
Spinal intradural malignant peripheral nerve sheath tumor at the foramen magnum in a non-neurofibromatosis child. 一名非神经纤维瘤病患儿位于枕骨大孔的脊髓硬膜内恶性周围神经鞘瘤。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-12-16 DOI: 10.1007/s00381-024-06719-8
Madhivanan Karthigeyan, Goutham Varma, Debajyoti Chatterjee, Sai Shiva Tadakamalla, Pravin Salunke, Rajeev Goel

Primary spinal intradural malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms, especially in children with a non-neurofibromatosis background. Scarce pediatric data exist with regard to such tumors. A 4-year-old child with a history of spastic limb weakness was operated for a foramen magnum spinal lesion (intradural and extradural) with imaging suggestive of schwannoma; the histopathology, however, was that of a MPNST. He underwent redo-surgery for recurrent infiltrating lesion, became ventilator dependent, and expired. Along with this case, we briefly discuss the relevant literature on pediatric primary spinal MPNSTs. The report represents an unusual site for spinal MPNST in a child with dual components. It is important for clinicians to consider such malignant lesions among the differentials of paediatric spinal intradural/ extradural lesions. Compared to that of adults and other regions, the prognosis for spinal intradural MPNSTs in children remains grim.

原发性脊髓硬膜内恶性周围神经鞘肿瘤(MPNSTs)是一种罕见的肿瘤,特别是在非神经纤维瘤背景的儿童中。关于这类肿瘤的儿科数据很少。一例4岁儿童因脊髓枕骨大孔病变(硬膜内和硬膜外)影像学提示神经鞘瘤接受手术治疗。然而,组织病理表现为MPNST。该患者因复发性浸润性病灶再次接受手术治疗,依赖呼吸机,最终死亡。结合本病例,我们简要讨论了小儿原发性脊柱mpnst的相关文献。该报告描述了一个不寻常的部位脊柱MPNST在儿童的双重成分。对于临床医生来说,在小儿脊髓硬膜内/硬膜外病变的鉴别中考虑这种恶性病变是很重要的。与成人和其他地区相比,儿童脊髓硬膜内mpnst的预后仍然严峻。
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引用次数: 0
Traumatic pediatric cervical spine injury-a proposed clearance algorithm incorporating a 24-h time delay. 外伤性小儿颈椎损伤-一种包含24小时时间延迟的拟议清除算法。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-12-16 DOI: 10.1007/s00381-024-06716-x
Victoria E Fischer, Vaidehi M Mahadev, Jacob A Bethel, Jaime A Quirarte, Robert J Hammack, Cristian Gragnaniello, Izabela Tarasiewicz

Purpose: Pediatric cervical spine injury (pCSI) is rare. Physiological differences necessitate alternate management from adults. Yet, no standardized pediatric protocols exist. Previous investigations applying adult-validated clinical decision rules (CDRs)-NEXUS Criteria (NX) and Canadian C-spine Rules (CCR)-to children are mixed. We hypothesized a combined NX + CCR approach applied at a delayed 24-h time point would enhance screening efficacy in select patients.

Methods: We conducted a retrospective review of a prospectively-collected database over 15 months at a pediatric-capable Level-1 trauma center. Age and mechanism determined initial inclusion. NX and CCR criteria were collected and retroactively applied on arrival (T0) and 24 h later (T1). Statistical analyses were performed in SPSS.

Results: A total of 306 patients met inclusion. Current practices compel computed tomography (CT) overuse for craniocervical evaluations: 298 (97.4%) underwent ≥ 1 CT. Of cervical spines imaged (n = 175), 161 (92.0%) underwent CT while 74 (42.3%) underwent magnetic resonance imaging with 14 (18.9%) completed after 72 h. Of collars placed on arrival (n = 181), 136 (75.1%) were cleared before discharge with 86 (63.2%) CTs denoting preferred clearance modality; CT utilization was unchanged when stratified by age < 5 years (p = 0.819). Notably, we found more patients met NX + CCR criteria at T1 versus T0 (p = 0.008) without missed pCSI resulting in imaging overutilization in 15 (8.6%) patients.

Conclusion: We showed incorporating a 24-h time delay before a second CDR reapplication may enhance screening efficacy in pCSI. Our new algorithm combines these findings with other literature-based recommendations and may represent a standardizable option for evaluating pCSI in the acute trauma setting.

目的:小儿颈椎损伤(pCSI)较为少见。生理差异需要成人的替代治疗。然而,没有标准化的儿科协议存在。先前对儿童应用成人验证的临床决策规则(CDRs)-NEXUS标准(NX)和加拿大颈椎规则(CCR)的研究是混合的。我们假设在延迟的24小时时间点应用NX + CCR联合方法可以提高选定患者的筛查效果。方法:我们对一家具有儿科能力的一级创伤中心前瞻性收集的数据库进行了回顾性审查,时间超过15个月。年龄和机制决定了最初的夹杂物。收集NX和CCR标准,并在到达时(T0)和24 h后(T1)追溯应用。采用SPSS软件进行统计学分析。结果:306例患者符合纳入标准。目前的做法迫使过度使用计算机断层扫描(CT)进行颅颈评估:298例(97.4%)接受了≥1次CT检查。在颈椎成像(n = 175)中,161例(92.0%)接受了CT检查,74例(42.3%)接受了磁共振检查,其中14例(18.9%)在72小时后完成。在到达时放置的项圈中(n = 181), 136例(75.1%)在出院前清除,86例(63.2%)CT显示首选清除方式;结论:我们显示在第二次CDR重新应用前纳入24小时的时间延迟可以提高pCSI的筛查效果。我们的新算法将这些发现与其他基于文献的建议结合起来,可能代表急性创伤环境中评估pCSI的标准化选择。
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引用次数: 0
Mechanisms of hydrocephalus after intraventricular haemorrhage: a review. 脑室内出血后脑积水的机理:综述。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-12-15 DOI: 10.1007/s00381-024-06711-2
Wenchao Wu, Qingsong Li

Intraventricular haemorrhage (IVH) is bleeding within the ventricular system, which in adults is usually mainly secondary to cerebral haemorrhage and subarachnoid haemorrhage. Hydrocephalus is one of the most common complications of intraventricular haemorrhage, which is characterised by an increase in intracranial pressure due to an increased accumulation of cerebrospinal fluid within the ventricular system, and is closely related to the patient's prognosis. Surgical methods such as shunt surgery have been used to treat secondary hydrocephalus in recent years and have been effective in improving the survival and prognosis of patients with hydrocephalus. However, complications such as shunt blockage and intracranial infection are often faced after surgery. Moreover, little is known about the mechanism of hydrocephalus secondary to intraventricular haemorrhage. This review discusses the mechanisms regarding the occurrence of secondary hydrocephalus after intraventricular haemorrhage in adults in terms of blood clot obstruction, altered cerebrospinal fluid dynamics, inflammation, and blood composition.

脑室内出血(IVH)是脑室系统出血,成人通常继发于脑出血和蛛网膜下腔出血。脑积水是脑室内出血最常见的并发症之一,其特征是脑脊液在脑室系统内积聚增加导致颅内压升高,与患者预后密切相关。近年来,分流术等手术方法被用于治疗继发性脑积水,有效提高了脑积水患者的生存率和预后。但术后易出现分流管堵塞、颅内感染等并发症。此外,对脑室内出血继发脑积水的机制知之甚少。本文从血凝块阻塞、脑脊液动力学改变、炎症和血液成分等方面讨论成人脑室内出血后继发性脑积水的发生机制。
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引用次数: 0
Subarachnoid puncture via the anterior fontanelle with intrathecal vancomycin for refractory purulent meningitis: a pediatric case report. 经前囟门进行蛛网膜下腔穿刺并注射万古霉素治疗难治性化脓性脑膜炎:一例儿科病例报告。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-12-15 DOI: 10.1007/s00381-024-06668-2
Yan Li, Xinghui Yang, Yongping Xie, Guolan Huang, Xiaohui Fang, Lisu Huang

This case report presents a novel treatment approach for refractory purulent meningitis in a 1-month-old infant caused by penicillin-sensitive group B Streptococcus. Despite initial treatment with intravenous antibiotics, including penicillin and vancomycin, the infant experienced persistent symptoms and bilateral subdural effusions. Conventional therapies failed to resolve these issues, leading to the use of a new technique: subarachnoid puncture via the anterior fontanelle combined with intrathecal vancomycin administration. This minimally invasive procedure allowed for targeted antibiotic delivery, significantly reducing subdural effusions and improving cerebrospinal fluid parameters. The infant showed substantial clinical improvement and was discharged after 68 days with no complications during a 3-month follow-up. This approach offers a promising alternative to more invasive treatments such as craniotomy or subdural external drainage, though further research is needed to confirm its efficacy and safety in broader clinical contexts.

这一病例报告提出了一种新的治疗方法难治性化脓性脑膜炎在1个月的婴儿引起青霉素敏感的B组链球菌。尽管最初给予静脉注射抗生素治疗,包括青霉素和万古霉素,婴儿仍出现持续症状和双侧硬膜下积液。传统的治疗方法不能解决这些问题,导致使用一种新的技术:经前囟门的蛛网膜下腔穿刺联合鞘内万古霉素。这种微创手术允许靶向抗生素递送,显著减少硬膜下积液并改善脑脊液参数。患儿临床表现明显改善,68天后出院,随访3个月无并发症。尽管需要进一步的研究来证实其在更广泛的临床背景下的有效性和安全性,但这种方法为诸如开颅术或硬膜下外引流等更具侵入性的治疗提供了一种有希望的替代方法。
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引用次数: 0
Relationship between the volume of ventricles, brain parenchyma and neurocognition in children after hydrocephalus treatment. 脑积水治疗后儿童脑室容积、脑实质和神经认知之间的关系。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-12-14 DOI: 10.1007/s00381-024-06674-4
Qinzhu Yang, Kun Huang, Gongwei Zhang, Xianjun Li, Yi Gao, Cailei Zhao

Purpose: The treatment of hydrocephalus aims to facilitate optimal brain development and improve the overall condition of patients. To further evaluate the postoperative recovery process in individuals undergoing hydrocephalus treatment, we investigated the interplay between brain parenchymal and ventricular volumes, alongside neurocognitive parameters.

Methods: In this study, 52 children under the age of 10 undergoing hydrocephalus treatment were included. All participants underwent T1w MR images and Gesell developmental schedule assessments. Initially, we investigated the correlation between patients' brain development and motor assessment scores. This analysis explored the association between cognition and both brain parenchymal and ventricular sizes. Furthermore, we investigated these relationships in the contexts of communicating and obstructive hydrocephalus. Finally, to quantitatively evaluate patients' brain development using more detailed texture information from imaging, we employed three different classification models for prediction. To compare their performances, we assessed these classification frameworks using a fourfold cross-validation method.

Results: Leveraging the deep learning framework, both pre- and postoperative T1w MR images have demonstrated a significant predictive value in estimating patients' brain development, with the accuracy of 0.808 for postoperative images. In the statistical analysis, we identified a correlation between developmental assessments in children with communicating hydrocephalus and postoperative brain parenchymal volume.

Conclusion: The findings indicate that postoperative evaluation of brain development is more closely associated with brain parenchymal and ventricular volumes than the Evans index. Additionally, deep learning frameworks exhibit promising potential as effective tools for accurately predicting patients' postoperative recovery.

目的:脑积水的治疗旨在促进大脑的最佳发育,改善患者的整体状况。为了进一步评估接受脑积水治疗的个体的术后恢复过程,我们研究了脑实质和脑室体积以及神经认知参数之间的相互作用。方法:本研究纳入52例10岁以下接受脑积水治疗的儿童。所有参与者都接受了T1w磁共振成像和Gesell发育时间表评估。最初,我们研究了患者大脑发育与运动评估评分之间的相关性。该分析探讨了认知与脑实质和脑室大小之间的关系。此外,我们在交通和梗阻性脑积水的背景下研究了这些关系。最后,为了利用影像中更详细的纹理信息定量评估患者的大脑发育,我们采用了三种不同的分类模型进行预测。为了比较它们的性能,我们使用四重交叉验证方法评估了这些分类框架。结果:利用深度学习框架,术前和术后T1w MR图像在估计患者大脑发育方面都显示出显著的预测价值,术后图像的准确率为0.808。在统计分析中,我们发现交通性脑积水患儿的发育评估与术后脑实质体积之间存在相关性。结论:与Evans指数相比,术后脑发育评价与脑实质和脑室容积的关系更为密切。此外,深度学习框架作为准确预测患者术后恢复的有效工具显示出很大的潜力。
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引用次数: 0
Surgical outcomes of tethered cord syndrome in patients with normal conus medullaris and filum terminale without urologic symptoms. 无泌尿系统症状的脊髓圆锥和终丝正常患者脊髓栓系综合征的手术效果。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-12-13 DOI: 10.1007/s00381-024-06713-0
Alper Tabanli, Emrah Akcay, Hakan Yilmaz, Seymen Ozdemir, Mesut Mete, Mehmet Selcuki

Purpose: Tethered cord syndrome (TCS) typically presents with urologic symptoms and abnormal imaging findings. However, some patients present with normal conus medullaris level and filum terminale appearance on MRI. This research seeks to assess the intended surgical results in this particular group of TCS patients who do not present with urologic complaints, under the premise that the surgical approach goes a long way in preventing the onset of urologic abnormalities.

Methods: This retrospective study included 59 operated patients with tethered cord syndrome who had a normal level terminating conus medullaris and a normal looking filum terminale without urologic symptoms. Of these patients, 38 were female and 21 were male. All patients underwent somatosensory-evoked potentials (SSEPs), and magnetic resonance imaging (MRI). The surgical technique used was flavotomy, which involves cutting the filum terminale without performing a laminectomy.

Results: The study population had a mean age of 22.5 years (SD = 13.2). During the mean postoperative follow-up period of 2.5 years, none of the patients developed urinary incontinence. Preoperative SSEP abnormalities included conduction block in 39 patients (66.1%), low amplitude in 12 patients (20.3%), and delayed N22 wave latency in 8 patients (13.5%). The surgical procedures were completed without morbidity or mortality, and all patients showed significant postoperative improvement in SSEP parameters.

Conclusion: Our results indicate that even though the filum terminale might have a normal looking MRI, TCS can also occur due to some potential microscopic or structural abnormality. The study proves SSEP to be useful in TCS diagnosis and it also proposes that if surgery is done early before any urologic complaints arise, chances of their onset will be minimized. Such findings support the view that surgical measures should be entertained in symptomatic patients with abnormal SSEP but normal MRI.

目的:脊髓栓系综合征(TCS)通常表现为泌尿系统症状和异常的影像学表现。然而,一些患者在MRI上表现为正常的髓圆锥水平和终丝外观。本研究旨在评估这组没有泌尿系统疾病的TCS患者的预期手术结果,前提是手术方法在预防泌尿系统异常发生方面有很长的路要走。方法:本回顾性研究包括59例脊髓栓系综合征手术患者,这些患者具有正常水平的髓圆锥和外观正常的终丝,无泌尿系统症状。其中女性38例,男性21例。所有患者均接受体感诱发电位(ssep)和磁共振成像(MRI)检查。使用的手术技术是黄酮症切开术,包括切断终丝而不进行椎板切除术。结果:研究人群平均年龄为22.5岁(SD = 13.2)。术后平均随访2.5年,无一例患者出现尿失禁。术前SSEP异常包括传导阻滞39例(66.1%),低幅值12例(20.3%),延迟N22波潜伏期8例(13.5%)。手术全部完成,无发病或死亡,所有患者术后SSEP参数均有明显改善。结论:我们的研究结果表明,即使终末丝在MRI上看起来正常,TCS也可能由于一些潜在的显微或结构异常而发生。该研究证明SSEP在TCS诊断中是有用的,并且还建议如果在任何泌尿系统疾病出现之前早期进行手术,其发病的机会将最小化。这些发现支持了这样一种观点,即对SSEP异常但MRI正常的有症状患者应考虑手术措施。
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引用次数: 0
Primary Ewing's Sarcoma affecting the Central Nervous System: A single-center experience and Narrative review. 影响中枢神经系统的原发性尤文氏肉瘤:单中心的经验和叙述回顾。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-12-13 DOI: 10.1007/s00381-024-06707-y
Sivaraman Kumarasamy, Kanwaljeet Garg, Pankaj Kumar Singh, Amandeep Kumar, Rajeev Sharma, Shweta Kedia, Shashwat Mishra, Sachin Borkar, Dattaraj Parmanad Sawarkar, Satish Kumar Verma, Subhash Gupta, Gurudutta Satyarthee, Ajay Garg, Meher C Sharma, Rajinder Kumar, Manmohan Singh, Ashish Suri, Poodipedi Sarat Chandra, Shashank Sharad Kale
<p><strong>Background and objectives: </strong>Ewing's sarcoma (EWS) is a malignant round-cell tumor arising from the bone and soft tissue. It is a disease of children and young adults. EWS affecting the central nervous system (cranial and spinal column) is relatively rare with an annual incidence of approximately one case per million in the Western population. Due to their rarity, very few studies are available in the literature. We present our experience of managing 21 such cases, highlighting their clinical, and radiological findings, treatment strategy, and surgical outcomes in patients with primary EWS affecting the central nervous system.</p><p><strong>Materials and methods: </strong>We retrospectively collected hospital records of patients with primary EWS affecting the CNS (cranial and spinal column), who had been surgically treated in our Neuroscience Center between 2015 and 2023. Patients' demographics, presentation, radiological findings, treatment strategy including surgery and biopsy followed by adjuvant therapy, and outcome at discharge, and the latest follow-up were analyzed from our database.</p><p><strong>Results: </strong>There were sixteen male and five female patients with a mean age of 18.22 ± 12.73 years (ranging from 6 months to 59 years). The commonest presentation was headache and vomiting in cranial lesions (5/13 patients, 38.46%), and back pain in spinal lesions (4/8 patients, 50%). The site of lesions was cranial in thirteen patients (61.9%) and spinal column in eight patients (38.1%). The commonest site was the frontal region in the cranial group (6 patients, 46.15%) and the lumbar region in the spinal group (4 patients, 50%). All patients underwent surgical intervention [tumor resection (18) and biopsy (3)]. Tumor resection was achieved in 18 patients (85.71%). The extent of resection was gross-total excision in 9 patients (42.86%), near-total excision in 5 patients (23.8%), and tumor decompression in 4 patients (19%). Four patients underwent spinal instrumentation. Fifteen patients (71.42%) received multiagent chemo-radiotherapy according to institute protocol. Five patients (23.8%) with poor KPS expired within 6 months of surgery and could not receive adjuvant therapy. Two patients (9.52%) improved symptomatically, and nine patients (42.86%) remained asymptomatic at a mean follow-up of 25.1 ± 29 months. One patient (4.76%) after receiving 2 cycles of adjuvant therapy had initial improvement but expired later due to disease progression. Four patients after adjuvant chemo-radiotherapy (19%) expired due to systemic spread.</p><p><strong>Conclusion: </strong>Primary EWS affecting the central nervous system is a rare variety. A detailed radiological assessment can aid in adequate planning for safe maximal resection. Timely tissue diagnosis is essential for initiating early treatment. Radical excision followed by adjuvant therapy offers a favorable outcome. Postoperative adjuvant chemo-radiotherapy aids in optimal diseas
背景和目的:尤文氏肉瘤(EWS)是一种起源于骨和软组织的恶性圆细胞肿瘤。这是一种儿童和年轻人的疾病。影响中枢神经系统(颅脑和脊柱)的EWS相对罕见,西方人口的年发病率约为百万分之一。由于它们的罕见性,文献中很少有研究。我们介绍了我们处理21例此类病例的经验,重点介绍了影响中枢神经系统的原发性EWS患者的临床、放射学表现、治疗策略和手术结果。材料和方法:回顾性收集2015年至2023年在我院神经科学中心接受手术治疗的原发性EWS累及中枢神经系统(颅脑和脊柱)患者的住院记录。从我们的数据库中分析患者的人口统计学、表现、放射学表现、治疗策略(包括手术和活检后的辅助治疗)、出院时的结果以及最近的随访。结果:男性16例,女性5例,平均年龄18.22±12.73岁(6个月~ 59岁)。颅脑病变最常见的表现为头痛和呕吐(5/13例,38.46%),脊柱病变最常见的表现为背痛(4/8例,50%)。病变部位为颅脑13例(61.9%),脊柱8例(38.1%)。颅脑组最常见的部位为额区(6例,46.15%),脊柱组最常见的部位为腰椎区(4例,50%)。所有患者均行手术干预[肿瘤切除(18)和活检(3)]。成功切除肿瘤18例(85.71%)。切除范围为全切除9例(42.86%),近全切除5例(23.8%),肿瘤减压4例(19%)。4例患者接受脊柱内固定。15例(71.42%)患者接受多药化疗。KPS差的患者5例(23.8%)在手术6个月内死亡,无法接受辅助治疗。2例(9.52%)患者症状改善,9例(42.86%)患者无症状,平均随访25.1±29个月。1例(4.76%)患者在接受2个周期的辅助治疗后出现初步改善,但后来因疾病进展而死亡。辅助放化疗后4例(19%)因全身扩散而死亡。结论:影响中枢神经系统的原发性EWS是一种罕见的疾病。详细的放射学评估有助于充分规划安全的最大切除。及时的组织诊断对于早期治疗至关重要。根治性切除和辅助治疗提供了良好的结果。术后辅助放化疗有助于最佳的疾病控制和手术效果。在目前的治疗策略下,可以获得良好的手术效果。然而,转移并不罕见,应定期评估。
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引用次数: 0
Surgical treatment of neonatal brachial plexus palsy: A cohort study using the Pediatric Health Information System (PHIS) database. 新生儿臂丛神经麻痹的外科治疗:一项使用儿童健康信息系统(PHIS)数据库的队列研究。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-12-12 DOI: 10.1007/s00381-024-06709-w
Molly F MacIsaac, Joshua M Wright, Nicole K Le, Lee G Phillips, Allan J Belzberg, S Alex Rottgers, Jordan N Halsey

Purpose: This study aims to explore demographic disparities, regional and institutional variations, surgical timing, narcotic use, and management trends in neonatal brachial plexus palsy (NBPP) patients due to limited published literature.

Methods: We conducted a retrospective cohort study using the Pediatric Health Information System (PHIS) database of NBPP patients who underwent surgery within the first 2 years of life. Patients were stratified into two groups based on age at surgery: ≤ 8 months and > 8 months.

Results: A total of 788 patients were identified, with a mean surgical age of 8.1 months. Black patients were disproportionately affected (29%), over twice their national birth rate (14%). Narcotic use was more common in younger patients (63% vs. 53%, p = 0.003), as well as in those treated in the West (71%, p = 0.001) and Northeast (73%, p = 0.004), and by plastic (74%, p < 0.0001) or orthopedic surgeons (69%, p = 0.002). Patients prescribed narcotics had longer hospital stays (1.7 vs. 1.2 days, p < 0.0001) and higher complication rates (7.9% vs. 3.1%, p = 0.009). Narcotic use decreased significantly over the study period (p = 0.002). Short-term outcomes, including complication and readmission rates, were similar across the three primary surgical specialties (plastic, orthopedic, and neurosurgery). High-volume centers had lower complication rates (1.5% vs. 5.4%, p = 0.002) and ICU admissions (5.8% vs. 18%, p < 0.0001) compared to medium-volume centers.

Conclusion: While narcotic use was associated with longer stays and complications, short-term outcomes were consistent across specialties. Standardized care protocols may help improve patient outcomes.

目的:由于文献有限,本研究旨在探讨新生儿臂丛神经麻痹(NBPP)患者的人口统计学差异、地区和制度差异、手术时机、麻醉使用和管理趋势。方法:我们利用儿童健康信息系统(PHIS)数据库对出生后2年内接受手术的NBPP患者进行了回顾性队列研究。患者根据手术年龄分为≤8个月和≤8个月两组。结果:共发现788例患者,平均手术年龄8.1个月。黑人患者受到的影响不成比例(29%),是其全国出生率(14%)的两倍多。麻醉药物的使用在年轻患者中更为常见(63%对53%,p = 0.003),在西部(71%,p = 0.001)和东北部(73%,p = 0.004)以及整形手术(74%,p)中也是如此。结论:虽然麻醉药物的使用与更长的住院时间和并发症有关,但短期结果在各专科是一致的。标准化的护理方案可能有助于改善患者的预后。
{"title":"Surgical treatment of neonatal brachial plexus palsy: A cohort study using the Pediatric Health Information System (PHIS) database.","authors":"Molly F MacIsaac, Joshua M Wright, Nicole K Le, Lee G Phillips, Allan J Belzberg, S Alex Rottgers, Jordan N Halsey","doi":"10.1007/s00381-024-06709-w","DOIUrl":"https://doi.org/10.1007/s00381-024-06709-w","url":null,"abstract":"<p><strong>Purpose: </strong>This study aims to explore demographic disparities, regional and institutional variations, surgical timing, narcotic use, and management trends in neonatal brachial plexus palsy (NBPP) patients due to limited published literature.</p><p><strong>Methods: </strong>We conducted a retrospective cohort study using the Pediatric Health Information System (PHIS) database of NBPP patients who underwent surgery within the first 2 years of life. Patients were stratified into two groups based on age at surgery: ≤ 8 months and > 8 months.</p><p><strong>Results: </strong>A total of 788 patients were identified, with a mean surgical age of 8.1 months. Black patients were disproportionately affected (29%), over twice their national birth rate (14%). Narcotic use was more common in younger patients (63% vs. 53%, p = 0.003), as well as in those treated in the West (71%, p = 0.001) and Northeast (73%, p = 0.004), and by plastic (74%, p < 0.0001) or orthopedic surgeons (69%, p = 0.002). Patients prescribed narcotics had longer hospital stays (1.7 vs. 1.2 days, p < 0.0001) and higher complication rates (7.9% vs. 3.1%, p = 0.009). Narcotic use decreased significantly over the study period (p = 0.002). Short-term outcomes, including complication and readmission rates, were similar across the three primary surgical specialties (plastic, orthopedic, and neurosurgery). High-volume centers had lower complication rates (1.5% vs. 5.4%, p = 0.002) and ICU admissions (5.8% vs. 18%, p < 0.0001) compared to medium-volume centers.</p><p><strong>Conclusion: </strong>While narcotic use was associated with longer stays and complications, short-term outcomes were consistent across specialties. Standardized care protocols may help improve patient outcomes.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"45"},"PeriodicalIF":1.3,"publicationDate":"2024-12-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142812074","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Hyperostotic meningiomas in children. A case-based update. 儿童肥厚性脑膜瘤。基于案例的更新。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-12-11 DOI: 10.1007/s00381-024-06708-x
Fulvio Grilli, Hamzah Youssef Smaili, Federico Bianchi, Paolo Frassanito, Gianpiero Tamburrini, Luca Massimi

Background: Meningiomas are rare tumors in children compared with adults. Their main peculiarities are the frequent convexity or intraventricular location, the common association with neurofibromatosis-2 (NF-2) and the relatively high rate of aggressive and/or hyperostotic variants. Hyperostosis may complicate the surgical management. The goal of this paper is to provide an update on the main characteristics and the management of hyperostotic meningiomas.

Case description: A 7-year-old girl was admitted to our department because of a long-lasting history of left frontal skull bulging. Neuroimaging examinations revealed a huge hyperostotic meningioma with bony invasion and infiltration of the superior sagittal sinus. Genetic tests were positive for NF-2. The tumor (atypical meningioma) was gross totally resected. During the same operation, a custom-made cranioplasty was realized with the help of a frame-based craniectomy. The patient is asymptomatic and under oncological follow-up.

Discussion and conclusion: Although generally rare, hyperostotic meningiomas are relatively common in children and clinically demanding because of their large size, the possible aggressive behavior and the need of a cranioplasty as additional surgical step. Therefore, a careful preoperative surgical planning is required, taking into account that the extent of surgical resection is an important prognostic factor. The long-term outcome is good.

背景:儿童脑膜瘤是一种少见的肿瘤。它们的主要特点是常呈凸状或位于脑室内,常与神经纤维瘤病-2 (NF-2)相关,侵袭性和/或增厚变异的发生率相对较高。骨质增生可能使手术治疗复杂化。本文的目的是提供一个更新的主要特点和管理的肥厚脑膜瘤。病例描述:一名7岁女童因左额颅骨长时间突出入住我科。神经影像学检查显示一个巨大的骨质增生脑膜瘤,并伴有骨侵犯和上矢状窦浸润。基因检测NF-2呈阳性。肿瘤(非典型脑膜瘤)全部切除。在同一手术中,在框架颅骨切除术的帮助下实现了定制的颅骨成形术。患者无症状,正在接受肿瘤随访。讨论与结论:虽然通常罕见,但肥大脑膜瘤在儿童中相对常见,由于其体积大,可能的侵袭性行为以及需要颅骨成形术作为额外的手术步骤,临床要求很高。因此,考虑到手术切除的程度是一个重要的预后因素,需要仔细的术前手术计划。长期结果是好的。
{"title":"Hyperostotic meningiomas in children. A case-based update.","authors":"Fulvio Grilli, Hamzah Youssef Smaili, Federico Bianchi, Paolo Frassanito, Gianpiero Tamburrini, Luca Massimi","doi":"10.1007/s00381-024-06708-x","DOIUrl":"https://doi.org/10.1007/s00381-024-06708-x","url":null,"abstract":"<p><strong>Background: </strong>Meningiomas are rare tumors in children compared with adults. Their main peculiarities are the frequent convexity or intraventricular location, the common association with neurofibromatosis-2 (NF-2) and the relatively high rate of aggressive and/or hyperostotic variants. Hyperostosis may complicate the surgical management. The goal of this paper is to provide an update on the main characteristics and the management of hyperostotic meningiomas.</p><p><strong>Case description: </strong>A 7-year-old girl was admitted to our department because of a long-lasting history of left frontal skull bulging. Neuroimaging examinations revealed a huge hyperostotic meningioma with bony invasion and infiltration of the superior sagittal sinus. Genetic tests were positive for NF-2. The tumor (atypical meningioma) was gross totally resected. During the same operation, a custom-made cranioplasty was realized with the help of a frame-based craniectomy. The patient is asymptomatic and under oncological follow-up.</p><p><strong>Discussion and conclusion: </strong>Although generally rare, hyperostotic meningiomas are relatively common in children and clinically demanding because of their large size, the possible aggressive behavior and the need of a cranioplasty as additional surgical step. Therefore, a careful preoperative surgical planning is required, taking into account that the extent of surgical resection is an important prognostic factor. The long-term outcome is good.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"43"},"PeriodicalIF":1.3,"publicationDate":"2024-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142806168","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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Child's Nervous System
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