Child's Nervous System最新文献

{"title":"The EMT status in the primary tumor of adamantinomatous craniopharyngioma predict postoperative recurrence in children.","authors":"K Bajdak-Rusinek, N Diak, E Gutmajster, A Fus-Kujawa, K L Stępień, W Wójtowicz, M Kalina, M Mandera","doi":"10.1007/s00381-024-06731-y","DOIUrl":"https://doi.org/10.1007/s00381-024-06731-y","url":null,"abstract":"<p><strong>Introduction: </strong>Adamantinomatous craniopharyngiomas (ACP) are rare epithelial tumors, which by the WHO are classified as non-malignant tumors. Despite radical tumor regression, almost 57% of patients develop a craniopharyngioma recurrence. The pathogenesis of epithelial cancers involves a process called epithelial-mesenchymal transition (EMT), which is involved in tumor progression and its invasion, and the loss of E-cadherin is crucial for this process. Undoubtedly, EMT also plays a role in the progression of ACP, but there are no studies that would examine its role in predicting postoperative tumor recurrence. Therefore, in our study, we aimed to compare the expression of EMT inducers and their markers, namely E-cadherin and vimentin, in material from two groups of pediatric patients, first with postoperative ACP relapse and second without relapse.</p><p><strong>Methods: </strong>A total of 35 formalin-fixed, paraffin-embedded tissue blocks of pediatric patients (19 girls and 16 boys, from 2 to 17 years old) were included. The material was collected during craniopharyngioma resection in the years 2000-2019 and after then examined by the Department of Pathomorphology. Gene expression analysis was done using qRT-PCR.</p><p><strong>Results: </strong>In the studied group of 35 patients, high levels of E-cad and low levels of vimentin expression were found in patients who did not experience relapse (n = 25, p < 0.0001). The opposite was observed in patients who experienced a recurrence (n = 10, p < 0.0001). In contrast, analysis of the recurrent tissue itself showed low levels of vimentin and re-expression of E-cadherin (n = 10, p < 0.0001). Furthermore, our study shows that Snail is a key inducer of EMT in ACP.</p><p><strong>Conclusion: </strong>We believe that the evaluation of the EMT profile in ACP could be a prognostic marker for predicting tumor recurrence in children, which would certainly contribute to a better prognosis for these patients.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"68"},"PeriodicalIF":1.3,"publicationDate":"2024-12-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142881318","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}

{"title":"Cervical spine considerations in Goldenhar syndrome: a clinical perspective.","authors":"Sumana Gadiraju, Gregg A Jacob, Catherine A Mazzola","doi":"10.1007/s00381-024-06727-8","DOIUrl":"https://doi.org/10.1007/s00381-024-06727-8","url":null,"abstract":"<p><strong>Background: </strong>Goldenhar syndrome is a clinically heterogeneous disorder defined by a rare combination of congenital anomalies-an eye abnormality, in addition to two of the following three: ear anomalies, mandibular malformations, and vertebral defects. Notably, children with Goldenhar syndrome present with a high incidence of cervical spine malformations.</p><p><strong>Clinical case: </strong>In this report, we present an unusual case of a 15-year-old child with Goldenhar syndrome, who additionally presents with some clinical features of VACTERL syndrome. The patient was scheduled for mandibular distraction surgery with oral maxillofacial surgery. It was noted on pre-operative three-dimensional computed tomography (3D CT) that there was mild basilar invagination and stenosis of the foramen magnum. A magnetic resonance imaging (MRI) study was recommended and showed cervicomedullary compression, for which a suboccipital craniectomy and C1 laminectomy were performed.</p><p><strong>Conclusion: </strong>Considering the cervical spine anomalies in Goldenhar syndrome, we describe precautions to manage the risk of cervical spinal cord injury during general anesthesia, including pre-operative evaluation for cervical spine compression and/or instability and minimizing neck movement during intubation. For the latter, we recommend a controlled, neutral neck position during intubation, operation, and recovery.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"65"},"PeriodicalIF":1.3,"publicationDate":"2024-12-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142876291","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}

{"title":"Methodology for pediatric head computed tomography image segmentation and volumetric calculation using a tablet computer and stylus pen.","authors":"H Hashimoto, M Shimada, O Takemoto, Y Chiba","doi":"10.1007/s00381-024-06723-y","DOIUrl":"https://doi.org/10.1007/s00381-024-06723-y","url":null,"abstract":"<p><strong>Purpose: </strong>This study presents a MATrix LABoratory (MATLAB)-based methodology for calculating intracranial volumes from head computed tomography (CT) data and compares it with established methods.</p><p><strong>Methods: </strong>Regions of interest (ROI) were manually segmented on CT images using a stylus pen, facilitated by mirroring a computer desktop onto a tablet. The volumetric process involved three main steps: (1) calculating the volume of a single voxel, (2) counting the total number of voxels within the segmented ROI, and (3) multiplying this voxel count by the single-voxel volume. This method was applied to 83 pediatric head CT scans from patients with minor head trauma, and the volumetric results were compared with those obtained from OsiriX.</p><p><strong>Results: </strong>A paired t-test revealed a statistically significant difference (p < 0.001) between volumes obtained with our MATLAB-based method and those from OsiriX, with our method measuring 0.32% higher. However, an unpaired t-test found no statistically significant differences between the volumetric population groups (p = 0.84).</p><p><strong>Conclusion: </strong>The significant difference identified by the paired t-test likely reflects statistical distinctions arising from differences in the calculation methods of the two approaches. Conversely, the unpaired t-test suggests no statistically detectable differences between the volumetric populations. Although this does not imply that the two methods produce identical results, the volumetric populations derived from our method may originate from the same underlying population as those obtained using OsiriX. By taking these points into account, our method has the potential to serve as a valuable tool for volumetric measurements.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"66"},"PeriodicalIF":1.3,"publicationDate":"2024-12-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142876295","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}

{"title":"Illustrations of the pineal gland in Descartes' oeuvre.","authors":"Douglas J Lanska","doi":"10.1007/s00381-024-06737-6","DOIUrl":"https://doi.org/10.1007/s00381-024-06737-6","url":null,"abstract":"","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"67"},"PeriodicalIF":1.3,"publicationDate":"2024-12-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142881305","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}

{"title":"Multinodular and vacuolating posterior fossa lesions of unknown significance: a long-term observational study and literature review.","authors":"Yuki Yasuoka, Fumiyuki Yamasaki, Iori Ozono, Shumpei Onishi, Akira Taguchi, Ushio Yonezawa, Nobutaka Horie","doi":"10.1007/s00381-024-06721-0","DOIUrl":"https://doi.org/10.1007/s00381-024-06721-0","url":null,"abstract":"<p><p>Multinodular and vacuolating neuronal tumor of the cerebrum (MVNT) is a rare tumefactive lesion requiring differentiation from glial tumors. Multinodular and vacuolating posterior fossa lesions of unknown significance (MV-PLUS) were initially reported in 2019 as posterior fossa lesions with characteristics similar to MVNT. MV-PLUS is often considered as a \"leave-me-alone lesion\" not necessitating surgical intervention. However, no studies have reported on MV-PLUS with long-term follow-up. In this report, we presented six new cases of MV-PLUS and reviewed existing literature on the condition. The median observation period for our cases was 93 months (range 79-124 months), significantly longer than previously reported. None of the patients underwent surgical treatment or showed any imaging changes during the observation period. We emphasize the importance of understanding the imaging characteristics of MV-PLUS to accurately differentiate these infratentorial lesions and avoid unnecessary surgical interventions.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"63"},"PeriodicalIF":1.3,"publicationDate":"2024-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142871619","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}

{"title":"Congenital extraventricular neurocytoma with atypical features in a 3-day-old neonate.","authors":"Sho Matsunaga, Yusuke Kimura, Yukinori Akiyama, Masaki Yamamoto, Nobuhiro Mikuni","doi":"10.1007/s00381-024-06725-w","DOIUrl":"https://doi.org/10.1007/s00381-024-06725-w","url":null,"abstract":"<p><p>Extraventricular neurocytoma (EVN) is a rare neuronal tumor with a marked tendency towards ganglionic or glial differentiation. Although EVN commonly occurs in young adults, congenital cases are extremely rare, and standardized therapeutic strategies remain undetermined. The presence of atypical features such as increased mitotic activity on histological analysis is correlated with a higher rate of recurrence and poor prognosis. A 3-d-old infant with no abnormalities at birth presented with recurrent apnea and paroxysmal eye deviation on the upper right side. Magnetic resonance imaging revealed a large intracranial tumor located anterior to the brainstem. The patient underwent biopsy via craniotomy, leading to a histological diagnosis of EVN with atypical features. Despite the absence of adjuvant radiation therapy or chemotherapy, the lesion remained stable over 18 months, and the patient's growth was within normal limits. EVN is an important differential diagnosis of congenital brain tumors. Therapeutic strategies for congenital cases should be distinguished from those used for older children, and treatment decisions should be based on individual patient risks.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"64"},"PeriodicalIF":1.3,"publicationDate":"2024-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142871616","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}

{"title":"Endoscopic third ventriculostomy with or without choroid plexus cauterization for preventing shunt dependence in pediatric hydrocephalus: preliminary results of a prospective clinical case series in a lower-middle-income country (Egypt).","authors":"Osama Aglan, Assem M Abdel-Latif, Abdelrahman El Gayar, Mohamed M Aziz, Ashraf G Al-Abyad, Benjamin C Warf","doi":"10.1007/s00381-024-06702-3","DOIUrl":"https://doi.org/10.1007/s00381-024-06702-3","url":null,"abstract":"<p><strong>Purpose: </strong>Pediatric hydrocephalus imposes a significant clinical and financial burden in developing countries. Traditional treatment by ventricular shunting in this setting suffers a high rate of shunt infection and malfunction. This study aims at assessing the efficacy and safety of endoscopic third ventriculostomy (ETV), either alone or combined with choroid plexus cauterization (CPC), for preventing shunt dependence in pediatric hydrocephalus within a healthcare setting of a tertiary hospital in Egypt.</p><p><strong>Methods: </strong>A prospective clinical case series included patients aged 16 years or younger with clinical manifestations of hydrocephalus requiring CSF diversion. Cases with active infection or brain imaging showing prohibitive anatomical distortion or multiloculated hydrocephalus were excluded. Failed ETV + / - CPC cases were categorized into early (< 3 months) and late (> 3 months) failure groups, each with specific management strategies.</p><p><strong>Results: </strong>A total of 40 patients were admitted to Ain Shams University Hospital between March 2022 and August 2023 and underwent ETV/CPC (n = 32) for those younger than 2 years and ETV alone (n = 8) for patients > 2 years old, using a flexible neuroendoscope. Aqueductal stenosis was the most common etiology, observed in 13 patients (32.5%), followed by Chiari type 2 in 11 patients (27.5%), and post-infectious causes in 6 patients (15%). The median age of the patients was 5.05 months (mean 22 months; range 0.23-169.27 months). The median preoperative ETV success score (ETVSS) was 50. Overall, 25 patients (62.5%) achieved successful outcomes following either ETV alone or ETV/CPC procedures, with no procedure-related morbidity or mortality. Fifteen patients (37.5%) experienced ETV failure, of whom 6 underwent ETV redo procedures. Across the entire cohort, 28 patients (70%) remained shunt-free, with a mean follow-up of 12 months.</p><p><strong>Conclusion: </strong>The addition of CPC to ETV (for patients < 2 years) using the flexible scope demonstrates effectiveness in decreasing the necessity for shunt placement in younger patients with a likelihood of ETV failure. Moreover, being a safe procedure, it supports a sustainable and cost-effective approach to hydrocephalus treatment.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"62"},"PeriodicalIF":1.3,"publicationDate":"2024-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142863491","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}

{"title":"Selective dorsal rhizotomy outcomes in mixed spasticity and dystonia cases.","authors":"Ahmed Aly, Mostafa Elmaghraby, Andrew Dapaah, Benjamin Hall, Christine Sneade, Benedetta Pettorini","doi":"10.1007/s00381-024-06720-1","DOIUrl":"https://doi.org/10.1007/s00381-024-06720-1","url":null,"abstract":"<p><strong>Introduction: </strong>This study investigated the effects of selective dorsal rhizotomy (SDR) on dystonia and dystonic posture in patients with cerebral palsy (CP) presenting with mixed spasticity and dystonia.</p><p><strong>Method: </strong>A prospective, single-centre study was conducted at a UK supra-regional centre from May 2013 to September 2022. All children with spasticity, dystonia and/or dystonic posture undergoing SDR were included. The primary outcome measure was pre- and postoperative assessment of dystonic posture. Dystonia severity was measured using the Barry-Albright dystonia (BAD) scale.</p><p><strong>Results: </strong>Two hundred and fifty-seven patients (aged 3-18 years) underwent SDR. Forty-three patients had dystonia and 52 exhibited dystonic posture without dystonia. At 3-month follow-up (n = 29), GMFCS levels tended to decrease, returning to baseline at 6 months (n = 25). Two patients required medication adjustments post-surgery. Dystonia levels remained unchanged in the dystonia group. In the dystonic posture group, 33 patients showed no change, while 21 improved.</p><p><strong>Conclusion: </strong>SDR may be beneficial for carefully selected patients with dystonia or dystonic posture, without worsening these conditions. Optimal patient selection, clear communication of surgical goals and multidisciplinary involvement are crucial.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"61"},"PeriodicalIF":1.3,"publicationDate":"2024-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142845786","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}

{"title":"Successful endoscopic third ventriculostomy in a patient with 40 years of ventriculoperitoneal shunt dependency since infancy: a case report.","authors":"Daisuke Kita, Yasuhiro Aida, Fumihide Enkaku","doi":"10.1007/s00381-024-06724-x","DOIUrl":"https://doi.org/10.1007/s00381-024-06724-x","url":null,"abstract":"<p><p>Ventriculoperitoneal shunt malfunction is a common complication in patients with lifelong hydrocephalus. We present a case of a 40-year-old man who successfully underwent endoscopic third ventriculostomy (ETV) for shunt malfunction following neonatal meningitis. Despite long-term shunt dependency and an infectious etiology, ETV effectively managed his hydrocephalus, probably due to aqueductal stenosis and preserved cerebrospinal fluid absorption. This suggests that ETV is a viable option for adult hydrocephalus patients experiencing shunt malfunction, even those with lifelong shunt dependency and a history of infectious etiology.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"60"},"PeriodicalIF":1.3,"publicationDate":"2024-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142845789","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}

{"title":"Endoscopic-assisted versus open fronto-orbital distraction for unicoronal craniosynostosis: morphometric and technique considerations.","authors":"Meagan Wu, Connor S Wagner, Dillan F Villavisanis, Jinggang J Ng, Benjamin B Massenburg, Dominic J Romeo, Gregory G Heuer, Scott P Bartlett, Jordan W Swanson, Jesse A Taylor","doi":"10.1007/s00381-024-06662-8","DOIUrl":"https://doi.org/10.1007/s00381-024-06662-8","url":null,"abstract":"<p><strong>Introduction: </strong>In an effort to maximize benefit and minimize morbidity when performing fronto-orbital distraction osteogenesis (FODO) for unilateral coronal synostosis (UCS), we have transitioned to an endoscopic-assisted approach (\"endo-FODO\"). This study compares photogrammetric outcomes of patients who underwent FODO via an endoscopic-assisted versus open approach.</p><p><strong>Methods: </strong>We retrospectively reviewed patients treated for UCS from 2013 to 2023. Photogrammetric outcomes at one to three years postoperatively were compared between patients who underwent endo-FODO and age- and sex-matched controls who underwent open FODO. Differences between pre- and postoperative periorbital symmetry ratios, canthal tilt symmetry, and orbital dystopia angle (ODA) were calculated.</p><p><strong>Results: </strong>Twenty patients (ten per group) underwent surgery at a mean age of 6.1 ± 1.8 and 5.4 ± 1.1 months (p = 0.426) and were photographed at 1.6 ± 0.9 and 1.8 ± 0.9 years (p = 0.597) postoperatively in the endo-FODO and open FODO groups, respectively. Patients who underwent endo-FODO demonstrated significant improvements in margin-reflex distance 1 (MRD1) symmetry ratio (p = 0.004), palpebral height symmetry ratio (p = 0.004), canthal tilt symmetry (p = 0.020), and ODA (p = 0.009). Patients who underwent open FODO likewise demonstrated significant improvements in MRD1 symmetry ratio (p = 0.004), palpebral height symmetry ratio (p = 0.033), and ODA (p = 0.004). All postoperative measurements as well as degrees of improvement were similar between groups (p > 0.05).</p><p><strong>Conclusions: </strong>Endo- and open FODO were associated with significant and comparable improvements in soft tissue periorbital symmetry and orbital dystopia at nearly two years postoperatively. While continued follow-up until cranial maturity is needed to assess the durability of aesthetic results, these data support a minimally invasive, endoscopic alternative to fronto-orbital distraction.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"59"},"PeriodicalIF":1.3,"publicationDate":"2024-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142845783","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}