Pediatric Neurosurgery最新文献

{"title":"A Molecular Update and Review of Current Trials in Paediatric Low-Grade Gliomas.","authors":"Sarah Al-Jilaihawi, Stephen Lowis","doi":"10.1159/000533703","DOIUrl":"10.1159/000533703","url":null,"abstract":"<p><strong>Background: </strong>Paediatric low-grade gliomas (pLGGs) are the most common primary brain tumour in children. Though considered benign, slow-growing lesions with excellent overall survival, their long-term morbidity can be significant, both from the tumour and secondary to treatment. Vast progress has been made in recent years to better understand the molecular biology underlying pLGGs, with promising implications for new targeted therapeutic strategies.</p><p><strong>Summary: </strong>A multi-layered classification system of biologic subgroups, integrating distinct molecular and histological features has evolved to further our clinical understanding of these heterogeneous tumours. Though surgery and chemotherapy are the mainstays of treatment for pLGGs, many tumours are not amenable to surgery and/or progress after conventional chemotherapy. Therapies targeting common genetic aberrations in the RAS-mitogen-activated protein kinase (RAS/MAPK) pathway have been the focus of many recent studies and offer new therapeutic possibilities. Here, we summarise the updated molecular classification of pLGGs and provide a review of current treatment strategies, novel agents, and open trials.</p><p><strong>Key messages: </strong>(1) There is a need for treatment strategies in pLGG that provide lasting tumour control and better quality of survival through minimising toxicity and protecting against neurological, cognitive, and endocrine deficits. (2) The latest World Health Organisation classification of pLGG incorporates a growing wealth of molecular genetic information by grouping tumours into more biologically and molecularly defined entities that may enable better risk stratification of patients, and consideration for targeted therapies in the future. (3) Novel agents and molecular-targeted therapies offer new therapeutic possibilities in pLGG and have been the subject of many recent and currently open clinical studies. (4) Adequate molecular characterisation of pLGG is therefore imperative in today's clinical trials, and treatment responses should not only be evaluated radiologically but also using neurological, visual, and quality of life outcomes to truly understand treatment benefits.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10038764","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":"Influence of Paraspinal Growth-Friendly Spinal Implants in Children with Spinal Muscular Atrophy on Parasol Deformity, Rib-Vertebral Angles, Thoracic, and Lung Volumes.","authors":"Julia Austein,&nbsp;Friederike Austein,&nbsp;Katja A Lüders,&nbsp;Lena Braunschweig,&nbsp;Konstantinos Tsaknakis,&nbsp;Heiko M Lorenz,&nbsp;Anna K Hell","doi":"10.1159/000531549","DOIUrl":"10.1159/000531549","url":null,"abstract":"<p><strong>Introduction: </strong>Children with spinal muscular atrophy (SMA) and progressive neuromuscular scoliosis often require early growth-friendly spinal implant (GFSI) treatment for deformity correction with implant fixation either through pedicle screws or bilateral to the spine using ribto pelvis fixation. It has been proposed that the latter fixation may change the collapsing parasol deformity via changes in the rib-vertebral angle (RVA) with a positive effect on thoracic and lung volume. The purpose of this study was to analyze the effect of paraspinal GFSI with bilateral rib-to-pelvis fixation on the parasol deformity, RVA, thoracic, and lung volumes.</p><p><strong>Methods: </strong>SMA children with (n = 19) and without (n = 18) GFSI treatment were included. Last follow-up was before definite spinal fusion at puberty. Scoliosis and kyphosis angles, parasol deformity, and index, as well as convex and concave RVA, were measured on radiographs, whereas computed tomography images were used to reconstruct thoracic and lung volumes.</p><p><strong>Results: </strong>In all SMA children (n = 37; with or without GFSI), convex RVA was smaller than concave values at all times. GFSI did not crucially influence the RVA over the 4.6-year follow-up period. Comparing age- and disease-matched adolescents with and without prior GFSI, no effect of GFSI treatment could be detected on either RVA, thoracic, or lung volumes. Parasol deformity progressed over time despite GFSI.</p><p><strong>Conclusion: </strong>Despite different expectations, implantation of GFSI with bilateral rib-to-pelvis fixation did not positively influence parasol deformity, RVA and/or thoracic, and lung volumes in SMA children with spinal deformity directly and over time.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9632288","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":"Patient- and Caregiver-Reported Outcome Measures after Single-Level Selective Dorsal Rhizotomy in Pediatric and Young Adult Patients with Spastic Cerebral Palsy.","authors":"Abeelan Rasadurai,&nbsp;Nicole Alexandra Frank,&nbsp;Ladina Aurea Greuter,&nbsp;Maria Licci,&nbsp;Peter Weber,&nbsp;Stephanie Jünemann,&nbsp;Raphael Guzman,&nbsp;Jehuda Soleman","doi":"10.1159/000530748","DOIUrl":"10.1159/000530748","url":null,"abstract":"<p><strong>Introduction: </strong>The aim of this cohort study was to assess the outcome of single-level selective dorsal rhizotomy (SDR) in children and young adults with spastic cerebral palsy (CP) treated at our institution, focusing on patient-reported outcome measures (PROMs) and quality of life (QoL) of patients and their caregivers.</p><p><strong>Methods: </strong>We included consecutive patients undergoing SDR from 2018 to 2020 at our institution. Subjective outcome was measured through PROMs, while functional outcome was measured through baseline characteristics, operative outcome, as well as short- and long-term follow-up. Furthermore, the effect of age at the time of surgery on patient/caregiver satisfaction was analyzed.</p><p><strong>Results: </strong>Seven patients (3 female, 43%) with a median age at surgery of 11.9 years (IQR 8.7-15.5) were included. All patients had a Gross Motor Function Classification (GMFCS) score of at least IV before surgery. Five surgeries were palliative and two non-palliative. Based on PROMs, SDR showed very good QoL and health-related outcome measures for both palliative and non-palliative patients. Patient/caregiver satisfaction was higher for the early subgroup (age ≤11) than the late subgroup (age &gt;11). Functional outcome showed reduced spasticity in both groups. Blood transfusions were never needed, while no cerebrospinal fluid leak, infection, or permanent morbidity was seen.</p><p><strong>Conclusion: </strong>Based on PROMs, SDR leads to high satisfaction and improved QoL, especially if done at an early age. Further studies with larger cohorts are necessary to underline and confirm our observations.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10614523/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10116370","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Use of Intraoperative Neuronavigation to Identify Transdural Collaterals in Moyamoya Vasculopathy: A Simple Way to Make It Safer","authors":"Matias L Costa, Danil A. Kozyrev, Harishchandra Lalgudi Srinivasan, M. Hausman-Kedem, Tali Jonas Kimchi, J. Roth","doi":"10.1159/000525454","DOIUrl":"https://doi.org/10.1159/000525454","url":null,"abstract":"Introduction: Transdural collaterals (TC) from the external carotid artery must be preserved when operating on patients with moyamoya vasculopathy. Several techniques have been used to identify the superficial temporal artery (STA) and middle meningeal artery (MMA) during surgery and prevent their damage. However, the use of neuronavigation for this specific purpose has never been described in the literature. We describe an operative case in which neuronavigation was used to preserve the TC (originating from the MMA), detailing our technique step by step and reviewing alternative methods previously reported. Case Presentation: A 6-year-old girl with moyamoya disease, who had developed marked bilateral TC from the MMA sparing the middle cerebral artery territory, underwent staged bilateral indirect revascularization surgery. Intraoperative neuronavigation was used to identify the STA and MMA with their main branches during skin incision, craniotomy, and dural opening. The neuronavigation matched the intraoperative findings exactly, and the target structures remained undamaged. The patient was discharged home after both surgeries with no neurological deficits. One year following surgery, the patient has excellent collateralization from both STAs and is asymptomatic and neurologically intact. Conclusion: With the use of intraoperative neuronavigation, the STA, MMA, and their main branches, as well as their relationship to the bone, can be identified and preserved. This approach can help in preventing undesirable injury to TC during surgery and may potentially prevent perioperative stroke in patients with moyamoya vasculopathy undergoing revascularization surgery.","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2022-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48991999","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":"Moyamoya Syndrome in a Patient with Williams Syndrome: A Case Report","authors":"Taisuke Akimoto, J. Suenaga, Tomoko Hayashi, Daisuke Hirokawa, S. Ito, Hironobu Sato, Tetsuya Yamamoto","doi":"10.1159/000525229","DOIUrl":"https://doi.org/10.1159/000525229","url":null,"abstract":"Introduction: Moyamoya syndrome associated with Williams syndrome is very rare but has been reported to have severe outcomes. Here, we reported a case of Williams syndrome with moyamoya syndrome that was confirmed by the presence of an RNF213 mutation. Case Presentation: A 6-year-old boy with Williams syndrome presented with right hemiparesis induced by hyperventilation. Magnetic resonance angiography and cerebral angiography showed severe stenosis of the bilateral internal carotid arteries and development of moyamoya vessels. Genetic analysis identified a heterozygous c.14576G>A (p.R4859K) mutation in RNF213. Moyamoya syndrome was diagnosed, and bilateral indirect revascularization surgery was conducted without complications and with a good postoperative course. In moyamoya syndrome associated with Williams syndrome, adequate perioperative management of both the moyamoya arteries and the cardiovascular abnormalities is important to prevent complications. Conclusion: This was the first report on a case in which moyamoya syndrome associated with Williams syndrome was confirmed by the presence of a heterozygous RNF213 mutation. Similar to the workup of moyamoya disease, confirmation of RNF213 mutation in Williams syndrome may be useful in predicting the development of moyamoya syndrome that can lead to severe complications.","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2022-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43263042","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":"Retrospective Investigation of Cranial Volume and Cephalic Index in Patients with Nonsyndromic Sagittal Synostosis Operated by Total Vault Remodeling","authors":"Maximilian Götzinger, M. Verius, R. Eder, I. Laimer, M. Rasse","doi":"10.1159/000525114","DOIUrl":"https://doi.org/10.1159/000525114","url":null,"abstract":"Background: Premature fusion of cranial sutures affects skull development and leads to head deformity. Intracranial pressure increase and brain growth restriction can occur in untreated craniosynostosis. Operative treatment aims to achieve an immediate and long-lasting correction of skull shape that is close to the average and to prevent or release possible increased intracranial pressure by increasing the intracranial volume (ICV) or normalizing the ICV if it is already below the standards. This study was designed to evaluate the effect of a total calvarial reconstruction on skull development in patients with nonsyndromic sagittal synostosis. Material and Methods: The study population included 19 male and 5 female patients with isolated nonsyndromic sagittal suture synostosis. During the operation, temporarily fixed prebent metal plates provided an intraoperative reference for the desired cranial expansion gain of height and shortening. Preoperative and postoperative ICVs and cephalic indices were measured on computed tomography datasets using the software program ImageJ and were compared with one another and with normative data. Results: The male population presented with a preoperative mean ICV of 863.3 cm³. A postoperative mean ICV increase of 243.5 cm³ (p < 0.001) and a further ICV enlargement (p < 0.001) was measured. The mean CI changed from 71.0% preoperatively to 75.4% postoperatively (p = 0.002) and decreased insignificantly in the follow-up (p = 0.546). The female population had a preoperative mean ICV of 804.9 cm³. Postoperatively, the mean ICV increased by 211.1 cm³ (p = 0.043) and also increased in the follow-up (p = 0.043). Their mean CI values increased from 66.5% preoperatively to 72.8% (p = 0.043) postoperatively and decreased insignificantly in the follow-up (p = 0.345). Conclusion: This method of total vault remodeling provides reliable ICV increase and improvement in length and width of skull proportions beyond the immediate postoperative period together with an ICV increase.","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2022-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48101865","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 Neonatal, Intraoperative Neuromonitoring in the Surgical Correction of a Thoracic Dermal Sinus Tract: Technical Note","authors":"Chidyaonga Shalita, Eric W. Sankey, Stephen M. Bergin, John McManigle, A. Buckley, R. Radtke, C. Torres, G. Dear, E. Thompson","doi":"10.1159/000524924","DOIUrl":"https://doi.org/10.1159/000524924","url":null,"abstract":"Introduction: Intraoperative neuromonitoring (IONM) is commonly used during surgery of the spine and spinal cord for early surveillance of iatrogenic injury to the central and peripheral nervous system. However, for infants and young children under 3 years of age, the use of IONM is challenging due to incomplete central and peripheral myelination. Case Presentation: We report a case of a T4-T6 dermal sinus tract (DST) that was resected on day of life 23, with the successful use of IONM. Conclusion: To our knowledge, this is the youngest reported case of the use of IONM in the surgical correction of a DST in a neonatal patient. This case demonstrates the potential efficacy of IONM in neonatal spine surgery and the techniques used to adapt the technology to an immature nervous system.","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2022-05-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43834725","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":"Novel “T-Dimension” Therapies for Pediatric Optic Pathway Glioma: A Timely, Targeted, and Tailored Treatment Trend","authors":"Alice Giotta Lucifero, S. Elbabaa, Matías Baldoncini, Nunzio Bruno, S. Savasta, G. Marseglia, S. Luzzi","doi":"10.1159/000524873","DOIUrl":"https://doi.org/10.1159/000524873","url":null,"abstract":"Introduction: Novel targeted and tailored therapies can substantially improve the prognosis for optic pathway glioma (OPG), especially when implemented in a timely manner. However, their tremendous potential remains underestimated. Therefore, in this study, we provide an updated overview of the clinical trials, current trends, and future perspectives for OPG’s novel therapeutic strategies. Methods: We completed an extensive literature review using the PubMed, MEDLINE, and ClinicalTrials.gov databases. We analyzed and reported the data following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Results: Thioguanine, procarbazine, lomustine, and vincristine/vinblastine, as well as cisplatin-etoposide, provided excellent results in advanced-phase trials. Selumetinib and trametinib, two oral MEK inhibitors, have been approved for recurrent or refractory OPGs in association with the angiogenetic inhibitor bevacizumab. Among the mTOR inhibitors, everolimus and sirolimus showed the best results. Stereotactic radiosurgery and proton beam radiation therapy have advantages over conventional radiotherapy regimens. Timely treatment is imperative for acute visual symptoms with evidence of tumor progression. This latest evidence can help define a novel “T-Dimension” for pediatric OPG therapies. Conclusion: The novel “T-Dimension” for pediatric OPGs is based on recent evidence-based treatments, including combination chemotherapy regimens, molecular targeted therapies, stereotactic radiosurgery, and proton beam radiation therapy. Additional clinical trials are essential for validating each of these new therapies.","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2022-05-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46284302","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":"Heartbroken Child: A Rare Case Report of Neurogenic Pulmonary Edema and Takotsubo Cardiomyopathy following Recurrent Medulloblastoma Excision with Possible Aetio-Patho-Bio-Physiological Mechanisms","authors":"H. Siroya, A. Uppar, V. Madhugiri, B. Devi","doi":"10.1159/000524896","DOIUrl":"https://doi.org/10.1159/000524896","url":null,"abstract":"Introduction: Takotsubo (Japanese fishing pot for trapping octopus) cardiomyopathy is a rare phenomenon of acute coronary syndrome presenting usually with the presence of transient apical ballooning of the left ventricle in the absence of obstructive coronary artery disease. It is mainly seen in women of older age secondary to emotional, physical, or psychological stress. In age less than 18 years, it is mainly seen in adolescents suffering from psychiatric disorders and substance abuse. In children, it is rarely described. Case Presentation: We present here a peculiar case of a 12-year-old child with neurogenic pulmonary oedema and takotsubo cardiomyopathy syndrome after surgery for recurrent medulloblastoma. Also, management challenges are discussed. Discussion/Conclusion: Takotsubo cardiomyopathy is not just a classical or inverted type but indeed a spectrum. It can be seen in any case, be it a child or adult secondary to handling or injury to the ponto-medullary junction, rostral pons, or dorsolateral medulla. The density and distribution of beta-adrenergic receptors may be different in children and adults which needs further research. Prognosis is usually excellent across all ages.","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2022-05-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47681481","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":"Prevalence of Postoperative Complications of Autologous and Heterologous Cranioplasty in the Pediatric Population: A Systematic Review of the Literature","authors":"I. Zaed, Francesca Faedo, S. Chibbaro, D. Cannizzaro, M. Tomei, F. Servadei, A. Cardia","doi":"10.1159/000524874","DOIUrl":"https://doi.org/10.1159/000524874","url":null,"abstract":"Introduction: Cranial reconstruction (CR) is a neurosurgical procedure performed to restore the cranial vault after a decompressive craniectomy. There are contrasting reports from the literature about the complications related to the use of heterologous materials for CR in the pediatric population. In this study, the authors try to better define such a rate of adverse events for autologous and heterologous materials. Materials and Methods: A systematic review of articles published up to December 2021 was performed. Studies were included if they reported the specific use of cranioplasty materials following craniectomy in patients younger than 18 years of age and had a minimum follow-up of at least 1 year. Results: A total of 20 studies were selected. A total of 544 cases were included, of which 422 (77.6%) were with heterologous materials and 122 (22.4%) with autologous bone. The mean average age was 9.5 years. Polyetheretherketone and polymethylmethacrylate reported 29% and 33.3%, respectively, of complications, but only 3% and 5.6% of surgical revision. PHA reported a rate of 11.9%. Titanium reported 9.2% of complications and 4.1% of surgical revisions. Porous polyethylene had a complication rate of 36.4% and a revision rate of 0%. Conclusion: There is still no perfect material for CR. It seems that heterologous materials are superior to autologous bone for CR in children, and we may consider, whenever economic conditions will allow it, to use alloplastic material as first-line in small children.","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2022-05-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46261328","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}