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Chondroblastoma of the occipital bone with aneurysmal bone cyst: A rare case report. 枕骨软骨母细胞瘤伴动脉瘤样骨囊肿:罕见病例报告。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-11-06 DOI: 10.1007/s00381-024-06632-0
Dexi Bi, Zihao Wang, Zhibo Dai, Shichun Yang, Gao Zeng, Ligang Wang

Background: Chondroblastoma is a rare, benign bone tumor originating from immature chondrocytes, typically found in the epiphyseal plates of long bones. Its occurrence in the skull, particularly the occipital bone, is extremely rare.

Case presentation: We report a 10-year-old girl presenting with paroxysmal dizziness, occipital pain, and a palpable mass. Imaging studies revealed an osteolytic lesion in the occipital bone with a multiloculated appearance, indicating a concomitant aneurysmal bone cyst. The patient underwent craniotomy with complete tumor excision. Postoperative follow-up showed no neurological deficits or complications and MRI confirmed no recurrence.

Conclusion: This case highlights the rare presentation of occipital bone chondroblastoma and emphasizes the importance of early surgical intervention for positive outcomes in pediatric patients.

背景:软骨母细胞瘤是一种罕见的良性骨肿瘤,起源于未成熟的软骨细胞,通常存在于长骨的骺板中。发生在颅骨,尤其是枕骨的情况极为罕见:我们报告了一名 10 岁女孩的病例,她出现阵发性头晕、枕骨疼痛并可触及肿块。影像学检查显示,枕骨中存在溶骨性病变,且呈多灶性外观,表明同时存在动脉瘤性骨囊肿。患者接受了开颅手术,肿瘤被完全切除。术后随访显示无神经功能缺损或并发症,核磁共振成像证实无复发:本病例突显了枕骨软骨母细胞瘤的罕见表现,并强调了早期手术干预对儿童患者取得良好疗效的重要性。
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引用次数: 0
Weekly vindesine as second-line chemotherapy in pediatric low-grade glioma. 小儿低级别胶质瘤的二线化疗--每周一次的文迪新。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-11-01 Epub Date: 2024-07-31 DOI: 10.1007/s00381-024-06557-8
Anan Zhang, Xiaoxia Wang, Godfrey C F Chan, Anthony P Y Liu, Yijin Gao
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引用次数: 0
Brachial plexus peripheral nerve sheath tumors (PNSTs): clinical and surgical management in the pediatric population. 臂丛周围神经鞘瘤(PNST):儿科临床和手术治疗。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-11-01 Epub Date: 2024-06-28 DOI: 10.1007/s00381-024-06509-2
Fernando Guedes, Evelina Llorian, Vinícius M Henriques, Francisco José Lourenço Torrão-Junior

Purpose: Peripheral nerve sheath tumors (PNSTs) are rare in pediatric patients, especially in the brachial plexus. Research on PNSTs is lacking. This article presents a retrospective cohort study of pediatric patients diagnosed and treated with PNSTs, specifically brachial plexus tumors.

Methods: All pediatric patients intervened in a single center between 2007 and 2023 with brachial plexus tumors were systemically analyzed.

Results: Eleven pediatric patients with 14 brachial plexus PNSTs were studied. The gender distribution was 64% female and 36% male, with an average age of 10.7 years. Ninety-one percent had a previous NF-1 diagnosis. Right brachial plexus presented a higher prevalence (64%). Pain, Tinel's sign, and stiffness masses were common during diagnosis. Motor deficits were noted in 43% of the patients. Surgery was indicated for symptoms, particularly pain and rapid growth, increasing malignancy risk. Due to suspected malignancy, an en bloc resection with safety margins was performed. Among the patients, 57% received a histopathological diagnosis of MPNST (malignant peripheral nerve sheath tumor). Treatment included radiotherapy and chemotherapy. Clinical follow-up was conducted for all cases, involving clinical and oncological evaluations for all MPNSTs.

Conclusions: This article present a series of pediatric brachial plexus tumors, especially in NF-1, and emphasizes the importance of thorough evaluation for this group. Swift diagnosis is crucial in pediatrics, enabling successful surgery for small lesions with limited neurological symptoms, improving long-term outcomes. Prompt referral to specialized services is urged for suspected masses, irrespective of neurological symptoms. Benign tumor postsurgical progression shows better outcomes than MPNSTs, with complete resection as the primary goal. Needle-guided biopsy is not recommended.

目的:周围神经鞘瘤(PNST)在儿科患者中很少见,尤其是臂丛神经。有关周围神经鞘瘤的研究十分缺乏。本文对诊断和治疗 PNST(尤其是臂丛神经肿瘤)的儿科患者进行了回顾性队列研究:方法:对2007年至2023年期间在一个中心接受干预的所有臂丛神经肿瘤儿科患者进行系统分析:研究了11名患有14种臂丛神经瘤的儿科患者。性别分布为女性64%,男性36%,平均年龄10.7岁。91%的患者曾被诊断为 NF-1。右臂丛神经的发病率较高(64%)。在诊断过程中,疼痛、Tinel 征和僵硬肿块很常见。43%的患者存在运动障碍。手术指征是症状,尤其是疼痛和快速增长,增加了恶性肿瘤的风险。由于怀疑是恶性肿瘤,患者接受了有安全边缘的全切除手术。其中 57% 的患者经组织病理诊断为 MPNST(恶性周围神经鞘瘤)。治疗包括放疗和化疗。对所有病例进行了临床随访,包括对所有 MPNST 进行临床和肿瘤学评估:本文介绍了一系列小儿臂丛神经肿瘤,尤其是NF-1,并强调了对这类肿瘤进行全面评估的重要性。迅速诊断对儿科至关重要,可使神经症状有限的小病灶成功接受手术,改善长期预后。对于疑似肿块,无论是否有神经系统症状,都应立即转诊至专业机构。手术后良性肿瘤的预后优于多发性骨髓瘤,完全切除是首要目标。不建议进行针导活检。
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引用次数: 0
Recurrent tethered cord: outcome and follow-up of 20 de-thetering for symptomatic spina bifida: choort study. 复发性脐带拴系:20 例症状性脊柱裂去势手术的结果和随访:Choort 研究。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-11-01 Epub Date: 2024-07-17 DOI: 10.1007/s00381-024-06537-y
Vitoria Cristina de Almeida, Mila Torii Correa Leite, Patricia Alessandra Dastoli, Rodrigo Akira Watanabe, Nelci Zanon

Objective: The objective of this study was to evaluate the prevalence of tethered cord among patients in the postoperative period of open and occult spina bifida. To identify warning signs for its early diagnosis, as well as outcomes after the new surgical approach.

Methods: Retrospective study of patients followed at the Pediatric Neurosurgery Department of the Federal University of São Paulo with spinal dysraphism. Signs and symptoms indicating reoperation were collected, and postoperative results were classified as improved, unchanged, or worsened.

Results: 222 medical records of patients diagnosed with spinal dysraphism were evaluated. Symptomatic Tethered Cord Syndrome (STCS) was identified in 30 patients (13.51%), with clinical manifestations related to orthopedic deformities (66.7%), neurological deficits (56.7%), urological dysfunction (50%), and intestinal dysfunction (40%). 20 cases underwent surgery for tethered cord release. The mean age at the time of surgery was 7.7 ± 4.9 years, with 13 female patients (65%). In the postoperative evaluation, improvement in low back pain (90.9%), urological pattern, and urinary tract infection episodes (45.4%) were particularly noteworthy. 3 patients (33.3%) with constipation showed improvement, and one worsened (11.1%). Improvement in ambulation was seen in two cases (16.7%). Low back pain was the first symptom to improve after surgery, with an average time of 1.3 months, followed by changes in the urological pattern at 15.6 months. Improvement in constipation was observed in the first month in 2 cases (66.7%), positive changes in ambulation were observed around 7 months after surgery, and only one case showed improvement in clubfoot correction.

Conclusions: The prevalence of tethered cord recurrence after primary correction surgery for open or occult neural tube closure defects was similar to that found in the literature. The results were encouraging, with good postoperative evolution of patients, especially in the improvement of low back pain and urological symptoms.

研究目的本研究旨在评估开放性和隐匿性脊柱裂患者术后脐带系带的发生率。确定早期诊断的警示信号以及新手术方法后的结果:方法:对圣保罗联邦大学小儿神经外科的脊柱发育不良患者进行回顾性研究。方法:对圣保罗联邦大学小儿神经外科随访的脊柱发育不良患者进行回顾性研究,收集表明需要再次手术的体征和症状,并将术后结果分为好转、不变或恶化。结果:共评估了 222 份确诊为脊柱发育不良患者的病历,其中发现 30 例患者(13.51%)患有症状性系索综合征(STCS),临床表现与骨科畸形(66.7%)、神经功能缺损(56.7%)、泌尿系统功能障碍(50%)和肠道功能障碍(40%)有关。20例患者接受了系带松解手术。手术时的平均年龄为(7.7 ± 4.9)岁,其中女性患者 13 例(65%)。在术后评估中,腰痛(90.9%)、泌尿系统模式和尿路感染发作(45.4%)的改善尤为显著。3 名便秘患者(33.3%)的情况有所改善,1 名恶化(11.1%)。有两名患者(16.7%)的行走能力有所改善。腰痛是术后最先得到改善的症状,平均改善时间为 1.3 个月,其次是泌尿系统模式的改变,平均改善时间为 15.6 个月。2例患者(66.7%)的便秘在术后第一个月就得到了改善,术后7个月左右,患者的行走能力出现了积极的变化,只有1例患者的足外翻矫正情况有所改善:结论:开放性或隐匿性神经管闭合缺损初次矫治术后系带复发率与文献报道相似。结果令人鼓舞,患者术后情况良好,尤其是腰背痛和泌尿系统症状有所改善。
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引用次数: 0
Transcranial motor-evoked potentials disappear with pons transection. 脑桥横断后,经颅运动诱发电位消失。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-11-01 Epub Date: 2024-08-21 DOI: 10.1007/s00381-024-06575-6
Ryota Sasaki, Tae Kyun Kim, Tsunenori Takatani, Young-Soo Park, Ichiro Nakagawa

Motor-evoked potential (MEP) monitoring by transcranial electrical stimulation (TES) is important for intraoperative motor function assessment in neurosurgery; however, false-negative results sometimes occur, and these findings should be interpreted with caution. Herein, we report an interesting MEP change resulting from a pons transection. The patient was a boy aged 5 years and 2 months. He underwent multiple craniotomies for cerebellar anaplastic ependymoma and was already paralyzed in the right upper and lower limbs. Therefore, we decided to remove the recurrent lesion from the left anterior pons. MEPs were recorded on both the right and left sides after the start of surgery but disappeared 1 h 30 min after the start of surgery in the TES on the operative side, even when the stimulation intensity was increased. The contralateral TES consistently recorded stable MEPs throughout the surgery. The tumor was completely resected on imaging. Immediately postoperatively, the patient experienced flaccid paralysis on the right side of the body, which recovered to preoperative levels over time. A transcranial MEP cannot be derived if the corticospinal tract is transected at the pons. Transcranial MEP findings may accurately reflect the corticospinal tract function if the injury is caudal to the pons.

通过经颅电刺激(TES)监测运动诱发电位(MEP)对于神经外科术中运动功能评估非常重要,但有时会出现假阴性结果,因此应谨慎解释这些结果。在此,我们报告了一起因脑桥横断而导致的有趣的 MEP 变化。患者是一名 5 岁零 2 个月的男孩。他因小脑无细胞上皮瘤接受了多次开颅手术,右上下肢已经瘫痪。因此,我们决定切除左侧大脑前部的复发病灶。手术开始后,左右两侧都记录到了MEPs,但手术开始1小时30分钟后,手术侧TES的MEPs消失了,即使增加刺激强度也是如此。对侧 TES 在整个手术过程中始终记录到稳定的 MEPs。成像显示肿瘤已完全切除。术后,患者右侧身体立即出现弛缓性麻痹,随着时间的推移,麻痹程度逐渐恢复到术前水平。如果皮质脊髓束在脑桥处横断,则无法获得经颅 MEP。如果损伤在脑桥尾部,经颅 MEP 结果可准确反映皮质脊髓束的功能。
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引用次数: 0
Stigma, seizure self-efficacy, and quality of life in children with epilepsy. 癫痫患儿的耻辱感、癫痫发作自我效能感和生活质量。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-11-01 Epub Date: 2024-08-30 DOI: 10.1007/s00381-024-06590-7
Seren Aydin, Gökçen Öz Tunçer, Şeyma Genç, Gülbahar Kurt Bayir, Ayşe Aksoy

Introduction: In addition to seizure care, it has been well-established that a holistic approach to the treatment of children with epilepsy that addresses the social, behavioral, and psychological dimensions also benefits their quality of life (QoL). The aim of this study was to investigate the patient and parental perceived stigma, seizure self-efficacy of children with epilepsy, and the relationship with quality of life in terms of sociodemographic and epilepsy-related factors.

Method: The study group consisted of children, aged between 8 and 18 years and with a diagnosis of epilepsy of at least six months duration and their parents. Pediatric Quality of Life Inventory (PedsQL), Child-Parent Stigma Scales, and the Seizure Self-Efficacy Scale for Children (SSES-C) were used for evaluation.

Results: One hundred and forty-four children (mean age 12.6 ± 2.9 years) took part, of whom 48.6% were female. The mean Child Stigma Scale score was 1.77 ± 0.82, Parent Stigma Scale score 2.41 ± 0.75, SSES-C score was 3.37 ± 0.98, and PedsQL score was 72.8 ± 18.6 for children self-reports (CsR) and 73.2 ± 18.8 for parent proxy-reports. The intraclass correlation coefficient for parent-child PedsQL indicated a good level of agreement. There was a significant negative correlation between the Child Stigma Scale and the PedsQL-CsR scores. A significant positive correlation was identified between the SSES-C scores and the PedsQL-CsR scores.

Discussion: Perceptions of stigma in children with epilepsy and their parents were high in this study population. Of note, the elevated stigma perception reported by the patients had a detrimental impact on seizure self-efficacy. This relationship may affect the children's QoL and further complicates epilepsy management in this patient group.

导言:除了癫痫发作护理外,针对癫痫儿童的社会、行为和心理层面的综合治疗方法也有利于提高他们的生活质量(QoL),这一点已得到充分证实。本研究旨在从社会人口学和癫痫相关因素的角度,调查患者和家长感知到的耻辱感、癫痫患儿的癫痫发作自我效能以及与生活质量的关系:研究对象包括年龄在 8 至 18 岁之间、诊断为癫痫且病程至少六个月的儿童及其父母。评估采用儿科生活质量量表(PedsQL)、儿童-家长耻辱感量表和儿童癫痫发作自我效能量表(SSES-C):144名儿童(平均年龄为12.6 ± 2.9岁)参加了评估,其中48.6%为女性。儿童耻辱感量表的平均得分为 1.77 ± 0.82,家长耻辱感量表的平均得分为 2.41 ± 0.75,SSES-C 的平均得分为 3.37 ± 0.98,儿童自我报告(CsR)的 PedsQL 得分为 72.8 ± 18.6,家长代理报告的 PedsQL 得分为 73.2 ± 18.8。亲子 PedsQL 的类内相关系数显示出良好的一致性。儿童耻辱感量表与 PedsQL-CsR 分数之间存在明显的负相关。SSES-C评分与PedsQL-CsR评分之间存在明显的正相关:讨论:在这项研究的人群中,癫痫患儿及其家长的耻辱感很高。值得注意的是,患者报告的成见感升高对癫痫发作自我效能感有不利影响。这种关系可能会影响儿童的生活质量,并使这一患者群体的癫痫管理更加复杂。
{"title":"Stigma, seizure self-efficacy, and quality of life in children with epilepsy.","authors":"Seren Aydin, Gökçen Öz Tunçer, Şeyma Genç, Gülbahar Kurt Bayir, Ayşe Aksoy","doi":"10.1007/s00381-024-06590-7","DOIUrl":"10.1007/s00381-024-06590-7","url":null,"abstract":"<p><strong>Introduction: </strong>In addition to seizure care, it has been well-established that a holistic approach to the treatment of children with epilepsy that addresses the social, behavioral, and psychological dimensions also benefits their quality of life (QoL). The aim of this study was to investigate the patient and parental perceived stigma, seizure self-efficacy of children with epilepsy, and the relationship with quality of life in terms of sociodemographic and epilepsy-related factors.</p><p><strong>Method: </strong>The study group consisted of children, aged between 8 and 18 years and with a diagnosis of epilepsy of at least six months duration and their parents. Pediatric Quality of Life Inventory (PedsQL), Child-Parent Stigma Scales, and the Seizure Self-Efficacy Scale for Children (SSES-C) were used for evaluation.</p><p><strong>Results: </strong>One hundred and forty-four children (mean age 12.6 ± 2.9 years) took part, of whom 48.6% were female. The mean Child Stigma Scale score was 1.77 ± 0.82, Parent Stigma Scale score 2.41 ± 0.75, SSES-C score was 3.37 ± 0.98, and PedsQL score was 72.8 ± 18.6 for children self-reports (CsR) and 73.2 ± 18.8 for parent proxy-reports. The intraclass correlation coefficient for parent-child PedsQL indicated a good level of agreement. There was a significant negative correlation between the Child Stigma Scale and the PedsQL-CsR scores. A significant positive correlation was identified between the SSES-C scores and the PedsQL-CsR scores.</p><p><strong>Discussion: </strong>Perceptions of stigma in children with epilepsy and their parents were high in this study population. Of note, the elevated stigma perception reported by the patients had a detrimental impact on seizure self-efficacy. This relationship may affect the children's QoL and further complicates epilepsy management in this patient group.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142104809","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
Intraoperative cone-beam computed tomography for catheter placement verification in pediatric hydrocephalus: technical note. 用于验证小儿脑积水导管位置的术中锥形束计算机断层扫描:技术说明。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-11-01 Epub Date: 2024-09-04 DOI: 10.1007/s00381-024-06592-5
Matthias Krause, Jasmina Lagumdzija, Simon Enzinger, Jörn Wittig, Alexander Gaggl, Roman P Metzger, Christoph J Griessenauer

Ventriculoperitoneal (VP) shunt placement, essential for managing hydrocephalus, often risks catheter malpositioning, especially in patients with small ventricles. We present a novel technique combining neuronavigation with intraoperative cone-beam computed tomography using the BrainLab system and Loop-X mobile imaging unit. This approach enables real-time verification of catheter placement by integrating preoperative MRI data with intraoperative CT imaging. In a 12-year-old boy with therapy-refractory idiopathic intracranial hypertension, neuronavigation was guided by the BrainLab Skull Fix and Cushing canula, ensuring precise catheter insertion into the right frontal horn. Post-placement, Loop-X facilitated immediate verification of the catheter's trajectory and positioning, corroborated by postoperative MRI. This technique demonstrated high precision and minimized radiation exposure, emphasizing its utility in reducing revision rates due to suboptimal catheter placement.

脑室腹腔(VP)分流术是治疗脑积水的关键,但经常存在导管定位错误的风险,尤其是对于脑室较小的患者。我们利用 BrainLab 系统和 Loop-X 移动成像装置,提出了一种将神经导航与术中锥形束计算机断层扫描相结合的新技术。这种方法通过整合术前核磁共振成像数据和术中 CT 成像,实现了导管放置的实时验证。在一名患有治疗难治性特发性颅内高压的 12 岁男孩身上,BrainLab 颅骨固定器和库欣管引导神经导航,确保导管精确插入右额角。置入后,Loop-X 可立即验证导管的轨迹和定位,术后核磁共振成像也证实了这一点。这项技术显示出高度的精确性,并最大限度地减少了辐射暴露,强调了其在降低因导管置入不理想而导致的翻修率方面的实用性。
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引用次数: 0
Biobank for craniosynostosis and faciocraniosynostosis, rare pediatric congenital craniofacial disorders: a study protocol. 罕见小儿先天性颅面疾病--颅骨畸形症和面颅骨畸形症生物数据库:研究方案。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-11-01 Epub Date: 2024-08-12 DOI: 10.1007/s00381-024-06555-w
Lucia De Martino, Peppino Mirabelli, Lucia Quaglietta, Ursula Pia Ferrara, Stefania Picariello, Domenico Vincenzo De Gennaro, Marco Aiello, Giovanni Smaldone, Ferdinando Aliberti, Pietro Spennato, Daniele De Brasi, Eugenio Covelli, Giuseppe Cinalli

Purpose: Craniosynostosis (CRS) is a rare congenital cranial malformation in which 1 or more cranial or facial sutures are fused in utero or rapidly fused in early infancy. The cranial sutures separate the skull bone plates and enable rapid growth of the skull in the first 2 years of life, in which growth is largely dictated by growth of the brain. CRS is a rare disease that occurs in 1 in 2100 to 1 in 2500 births and may be either nonsyndromic (also referred to as isolated) or syndromic. In syndromic CRS, other birth defects are present next to the CRS. The distinction between nonsyndromic and syndromic manifestations is made on the basis of dysmorphologic evaluation and genetic evaluation. Owing to advances in genetic diagnostics, nonsyndromic patients are increasingly recognized as syndromic patients. CRS treatment is almost entirely surgical and is sometimes paired with postoperative helmet therapy for maintenance. Corrective procedures are complex, long, and associated with the risk of numerous complications, including heavy blood loss and its sequelae. Although surgery may restore a normal appearance, even in nonsyndromic patients, patients may experience persistent deficits in intellectual ability and cognitive function. The European Commission (EC) has prioritized rare diseases in recent horizon European research programs; indeed, collections or even individual samples may be extremely valuable for research.

Methods and results: Here, we present a study protocol in which the combined expertise of clinicians and researchers will be exploited to generate a biobank dedicated to CRS. The generation of the CRS biobank presented in this study will include the collection of different types of biological materials as well as advanced radiological images available to the scientific community.

Conclusion: The activation of a CRS biobank will provide an opportunity to improve translational research on CRS and to share its benefits with the scientific community and patients and their families.

目的:颅骨连接畸形(Craniosynostosis,CRS)是一种罕见的先天性颅骨畸形,患者的一条或多条颅缝或面缝在子宫内融合,或在婴儿早期迅速融合。颅缝将颅骨骨板分开,使头骨在出生后的头 2 年迅速生长,而头骨的生长主要由大脑的生长决定。CRS 是一种罕见的疾病,每 2100 到 2500 个新生儿中就会有 1 例患病,可能是非综合征(也称为孤立性),也可能是综合征。在综合征型 CRS 中,除 CRS 外还存在其他出生缺陷。区分非综合征和综合征表现的依据是畸形评估和遗传评估。由于基因诊断技术的进步,非综合征患者越来越多地被认为是综合征患者。CRS 的治疗几乎全部采用外科手术,有时还搭配术后头盔疗法进行维持。矫正手术复杂、时间长,并伴有大量并发症的风险,包括大量失血及其后遗症。虽然手术可以恢复正常外观,但即使是非综合征患者,也可能出现持续的智力和认知功能障碍。欧盟委员会(European Commission,EC)在最近的欧洲地平线研究计划中将罕见病列为优先研究对象;事实上,采集的样本甚至个体样本对研究都极具价值:在此,我们将介绍一项研究方案,其中将综合利用临床医生和研究人员的专业知识,建立一个专门用于 CRS 的生物库。本研究中介绍的 CRS 生物库的建立将包括收集不同类型的生物材料以及可供科学界使用的先进放射图像:启动 CRS 生物库将为改善 CRS 转化研究提供机会,并与科学界、患者及其家属分享研究成果。
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引用次数: 0
Regression of Chiari malformation type 2 following early postnatal meningomyelocele repair-a retrospective observation from an institutional series of patients. 产后早期脑膜瘢痕修复术后Chiari畸形2型的消退--机构系列患者的回顾性观察。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-11-01 Epub Date: 2024-08-29 DOI: 10.1007/s00381-024-06586-3
Radek Frič, Mona Kristiansen Beyer, Bernt Johan Due-Tønnessen

Purpose: Spontaneous regression of Chiari malformation type 2 (CM2) is observed rarely, as CM2 is associated with meningomyelocele (MMC) that is surgically repaired either pre- or early postnatally. While the radiological regression of CM2 occurs frequently following prenatal repair of MMC, it has been reported in only a few studies after postnatal repair.

Methods: From the consecutive series of children with postnatally repaired MMC, we reviewed the clinical and radiological data regarding CM2, particularly its regression either spontaneously or following CSF diversion.

Results: Eighteen children underwent postnatal repair of MMC between February 2011 and April 2023. CM2 was present in 16 (89%), and hydrocephalus in 15 children (83%), requiring shunting in 14 of them. During the mean clinical observation time (from birth to April 2023) of 59 ± 51 months, three children with CM2 (19%) underwent 1-2 foramen magnum decompressions (FMD), five children (28%) 1-4 surgical untethering procedures and 13 children with shunted hydrocephalus (93%) 1-5 shunt revisions. Out of sixteen children with CM2, we observed regression of CM2 on MRI in only one case (6%) during the mean radiological follow-up (from birth to the last MRI taken) of 49 ± 51 months.

Conclusion: In our experience, spontaneous regression of CM2 in children with postnatally repaired MMC occurs quite rarely. Pathophysiological mechanisms behind the development of CM2 in children with MMC remain unclear, but our observation supports the hypothesis of an association between the downward displacement of the hindbrain and the low intraspinal pressure secondary to CSF leakage in children born with MMC.

目的:Chiari畸形2型(CM2)很少出现自发性消退,因为CM2与脑膜疝(MMC)有关,而MMC在出生前或出生后早期就已通过手术修复。虽然在产前对 MMC 进行修复后,CM2 的放射学退变经常发生,但在产后修复后,只有少数研究报告了这种情况:方法:我们从一系列连续的产后修复 MMC 患儿中回顾了有关 CM2 的临床和放射学数据,尤其是其自发或 CSF 转移后的消退情况:2011年2月至2023年4月期间,18名患儿接受了MMC产后修复手术。16名患儿(89%)出现CM2,15名患儿(83%)出现脑积水,其中14名患儿需要进行分流。在 59 ± 51 个月的平均临床观察时间内(从出生到 2023 年 4 月),3 名 CM2 患儿(19%)接受了 1-2 次枕骨大孔减压术(FMD),5 名患儿(28%)接受了 1-4 次手术松绑,13 名分流性脑积水患儿(93%)接受了 1-5 次分流手术。在 16 名患有 CM2 的儿童中,我们在平均 49 ± 51 个月的放射学随访期间(从出生到最后一次拍摄核磁共振成像),仅在一个病例(6%)的核磁共振成像中观察到 CM2 消退:根据我们的经验,产后修复的 MMC 患儿 CM2 自发消退的情况非常罕见。MMC 患儿出现 CM2 的病理生理学机制尚不清楚,但我们的观察结果支持后脑向下移位与先天性 MMC 患儿脑脊液渗漏导致椎管内压力过低之间存在关联的假设。
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引用次数: 0
Peripheral nerve injuries associated with dislocated supracondylar fractures of distal humerus in children: incidence and need of surgical treatment. 与儿童肱骨远端髁上骨折脱位相关的周围神经损伤:发生率和手术治疗的必要性。
IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Pub Date : 2024-11-01 Epub Date: 2024-06-18 DOI: 10.1007/s00381-024-06497-3
Robert Chrenko, Martin Hanko, Marek Grega, Ľubomír Sýkora, René Jáger

Purpose: The objective of this study was to determine the incidence, necessity for neurosurgical intervention, and overall results of the treatment of pediatric peripheral nerve injuries associated with dislocated supracondylar fractures of the distal humerus.

Method: A retrospective analysis of pediatric patients with supracondylar fractures treated from April 2019 to April 2022 with a minimum follow-up of 3 months was conducted.

Results: Of 453 included patients, there were 51 recorded peripheral nerve injuries. The ulnar nerve was the most frequently injured nerve. Nine patients required neurosurgical intervention, with the most common procedure being the release of entrapped nerves. The combination of a supracondylar fracture and arterial injury was identified as a significant risk factor for peripheral nerve injury (p < 0.001). Only one patient experienced an unsatisfactory outcome.

Conclusion: Although the prognosis for peripheral nerve injuries in children with supracondylar fractures is generally favorable, these injuries must be properly identified. We recommend an active neurosurgical approach in children with persisting neurological deficits to minimize the risk of permanent neurological impairment.

目的:本研究旨在确定与肱骨远端髁上骨折脱位相关的小儿周围神经损伤的发生率、神经外科干预的必要性以及治疗的总体效果:对2019年4月至2022年4月期间接受治疗且随访至少3个月的肱骨髁上骨折儿科患者进行回顾性分析:在纳入的453例患者中,有51例记录到周围神经损伤。尺神经是最常见的损伤神经。九名患者需要接受神经外科干预,最常见的手术是松解卡压的神经。肱骨髁上骨折和动脉损伤被认为是导致周围神经损伤的重要风险因素(P 结论):尽管肱骨髁上骨折患儿周围神经损伤的预后一般较好,但必须正确识别这些损伤。我们建议对持续存在神经功能缺损的儿童采取积极的神经外科治疗方法,以最大限度地降低永久性神经功能损伤的风险。
{"title":"Peripheral nerve injuries associated with dislocated supracondylar fractures of distal humerus in children: incidence and need of surgical treatment.","authors":"Robert Chrenko, Martin Hanko, Marek Grega, Ľubomír Sýkora, René Jáger","doi":"10.1007/s00381-024-06497-3","DOIUrl":"10.1007/s00381-024-06497-3","url":null,"abstract":"<p><strong>Purpose: </strong>The objective of this study was to determine the incidence, necessity for neurosurgical intervention, and overall results of the treatment of pediatric peripheral nerve injuries associated with dislocated supracondylar fractures of the distal humerus.</p><p><strong>Method: </strong>A retrospective analysis of pediatric patients with supracondylar fractures treated from April 2019 to April 2022 with a minimum follow-up of 3 months was conducted.</p><p><strong>Results: </strong>Of 453 included patients, there were 51 recorded peripheral nerve injuries. The ulnar nerve was the most frequently injured nerve. Nine patients required neurosurgical intervention, with the most common procedure being the release of entrapped nerves. The combination of a supracondylar fracture and arterial injury was identified as a significant risk factor for peripheral nerve injury (p < 0.001). Only one patient experienced an unsatisfactory outcome.</p><p><strong>Conclusion: </strong>Although the prognosis for peripheral nerve injuries in children with supracondylar fractures is generally favorable, these injuries must be properly identified. We recommend an active neurosurgical approach in children with persisting neurological deficits to minimize the risk of permanent neurological impairment.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141418085","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}
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Child's Nervous System
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